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Use of state-of-the art imaging techniques, including high resolution endoscopy with narrow band imaging, confocal laser endomicroscopy and other advanced systems.

A comprehensive Barrett’s program providing all FDA-approved treatments — photodynamic therapy, endoscopic resection, radiofrequency ablation, cryotherapy and surgical resection.

Esophagectomy using conventional and laparoscopic minimally invasive techniques.

NCI-designated comprehensive cancer center with numerous clinical trials evaluating novel tests and treatments for esophageal cancer and other gastrointestinal malignancies. www . DCMS online . org


VOLUME 64, NUMBER 3 Cardiac Disease, Autumn 2013

Autumn 2013

EDITOR IN CHIEF Raed Assar, MD (Chair)

Contents

MANAGING EDITOR Laura Townsend ASSOCIATE EDITORS Laura Armas-Kolostroubis, MD Abubakr Bajwa, MD Kim Barbel-Johnson, MD Ruple Galani, MD Kathy Harris (Alliance) Sunil Joshi, MD (Vice Chair) James Joyce, MD Daniel Kantor, MD Neel Karnani, MD Mobeen Rathore, MD James St. George, MD

EXECUTIVE DIRECTOR Bryan Campbell DCMS FOUNDATION BOARD OF DIRECTORS Ashley B. Norse, MD, President Eli N. Lerner, MD, President-Elect Neel G. Karnani, MD, Vice President Mobeen Rathore, MD, Vice President Daniel Kantor, MD, Vice President Raed Assar, MD, Secretary Sunil Joshi, MD, Treasurer Malcolm Foster, Jr., MD, Im. Past President Cynthia Anderson, MD Elizabeth Burns, MD Paul Chappano, MD Rui Fernandes, MD Ruple J. Galani, MD E. Rawson Griffin, MD Mark L. Hudak, MD TraChella Johnson Foy, MD James J. Joyce, MD Harry M. Koslowski, MD Stephen E. Mandia, MD Jesse P. McRae, MD Jason D. Meier, MD Nathan P. Newman, MD Alexander Pogrebniak, MD James St. George, MD Nathan P. Newman, MD Sanjay Swami, MD David L. Wood, MD Bouali Amoli, DMD, MD, Resident LT George Salgado, MC, USN, Resident Amit Grover, MD, Resident Monique Gray, MD, Resident Northeast Florida Medicine is published by the DCMS Foundation, Jacksonville, Florida, on behalf of the County Medical Societies of Duval, Clay, Nassau, Putnam, and St. Johns. Except for official announcements from the County Medical Societies, no material or advertisements published in NEFM are to be seen as representing the policy or views of the DCMS Foundation or its colleague Medical Societies. All advertising is subject to acceptance by the Editor in Chief. Address correspondence and advertising to: 555 Bishopgate Lane, Jacksonville, FL 32204 (904-355-6561), or email: ltownsend@dcmsonline.org.

www . DCMS online . org

Autumn CME

Congenital 11 AdultCardiac Care Brandon Kuebler, MD; Diego Moguillansky, MD and Arwa Saidi, MD

Successes within congenital cardiology are yielding an exponentially increasing adult population with varying degrees of complexity. Currently, 85 percent of patients with complex cardiac defects are expected to reach adulthood. Adequate preparation during childhood and adolescence increases the likelihood for patients to maintain adequate medical follow-up and successful decision-making in their personal life as adults.

Features Shared Successes and New Challenges

9

Randall M. Bryant, MD, Guest Editor

Role of Cardiac CTA and Cardiac MRI 19 in the Diagnosis of Congenital Heart Disease Arun Chandran, MD and Thomas Moon, MD

Departments

5

From the Editor’s Desk

7

Residents’ Corner

8

Patient Page

57 Trends in Public Health 58 From the President’s Desk

Interventional Catheterization in the 27 Care of the Congential Heart Disease Patient James C. Fudge, Jr., MD, MHS and Robert English, MD

The Staged Surgical Approach to Hypoplastic Left Heart Syndrome

35

Michael Shillingford, MD; Eric Ceithaml, MD and Mark Bleiweis, MD

Arrhythmias in Congenital Heart Disease

41

Randall M. Bryant, MD; Jason Ho, MD and Sharon P. Redfearn, ARNP

Heart Failure Management in End-Stage Congenital Heart Disease

49

Gonzalo Wallis, MD

Special Articles 2013 DCMS Nominating Committee Report

56

Northeast Florida Medicine Vol. 64, No. 3 2013 3




  

                   

4 Vol. 64, No. 3 2013 Northeast Florida Medicine

www . DCMS online . org


From the Editor’s Desk

DCMS Physicians’ Community Contributions Since its founding in 1853, the purpose of the Duval County Medical Society (DCMS) has always been to serve its community; DCMS has led an enormous number of community initiatives. Today, the list of community activities on DCMS’ website makes it easy to become aware of these great contributions. This editorial will highlight how tracking and reporting these activities can help DCMS and its physicians achieve their mission: Helping Physicians Care for the Health of our Community. As this medical society celebrates its 160th year birthday, it is important to review its overall contributions to society. DCMS is currently leading great initiatives to increase access to health care and improve health outcomes Raed Assar, MD, MBA of the population. DCMS Editor-in-Chief Northeast Florida Medicine encourages all its members to be active in the community and has developed this website to inform the community about its physicians’ philanthropic activities. Each year, many physicians in this community contribute to a variety of local and international organizations. Some raise money for charitable organizations or personally donate to charities and religious organizations, in addition to participating in health care walks, fairs and other events. Others are involved in outreach and direct mission type interventions in medically needy areas in the nation or in third world countries. Unfortunately, many of these contributions frequently go unnoticed. This lack of awareness results in the loss of public credit for all of the good will provided in the community and less encouragement for other physicians to take leadership roles in philanthropic initiatives. While the purpose of tracking and reporting of such community initiatives is never for self-promotion, it is important for DCMS and its foundation to highlight these valuable contributions. These activities are essential to maintain a leading role in shaping public health policy and in managing the health of our community. It allows DCMS to demonstrate how it delivers valuable services to the community and beyond. Through its website, DCMS highlights initiatives and organizations that have designated

DCMS representatives or members in leadership roles. DCMS urges its members to provide information about additional initiatives not included on this list to improve awareness of the charitable services that its members provide. Making DCMS members aware of such initiatives will help in many ways. It is the embodiment of leading by example. DCMS recognizes and appreciates the leaders of the current initiatives on the list such as Drs. Foster, Coble, Evans, Bowers, Mandia, Willis, Lucie, Montgomery, Harmon, Burt, Sack, and many others who are active with these initiatives. Additionally, DCMS is grateful for its President, Dr. Eli Lerner, and its Executive Vice President, Bryan Campbell, for representing DCMS on several community initiatives. Reviewing the list and information on the various purposes of these initiatives will garnish more support from physicians and the community. It provides support to physicians who have been thinking about joining such efforts but didn’t know which would work best in their work-life balance and provide them with the most fulfilling experience in community service. Others might have creative ideas that have gone unrealized, but when they review the list, might think of ways to make their dreams of community service possible. This list also creates excitement and new synergies. Many physicians report that hands-on volunteer activities help keep their skills sharp and bring them satisfaction, equaling or even exceeding that of sightseeing or vacationing at the beach. Finding more information about how to get involved and what kind of malpractice protection they receive is paramount. Additionally, the leaders of existing efforts can serve as great mentors and provide excellent guidance on what would work best for the community and how to get started. This repository will help provide a big picture for DCMS on how these initiatives fill the long list of health care needs in its community and beyond. This will lead DCMS to better focus its resources on efforts that work best and eliminate duplicative, costly, or non-effective initiatives while making more important ones even more effective. One final purpose of this list is that it brings DCMS’ members a sense of pride in this great medical society and our profession. Please take a peek at this list today. If you are involved in a community organization representing the medical profession and contributing to the wellbeing of others, please contact Laura Townsend at laura@ dcmsonline.org with the details. The entire society will be grateful that you did. The list of major initiatives is available for review at: http://dcmsonline.org/content. php?page=DCMSintheCommunity.

*Dr. Assar is Aetna’s Medical Director for North Florida. Articles or opinions provided by Dr. Assar do not necessarily reflect the views of Aetna. www . DCMS online . org

Northeast Florida Medicine Vol. 64, No. 3 2013 5


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Rehabilitation Hospital • Outpatient Clinics • Home Health • Skilled Nursing • Medical Group Practice • Research • Community Programs

6 Vol. 64, No. 3 2013 Northeast Florida Medicine

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Residents’ Corner: Naval Hospital Jacksonville’s Family Residency Program Editor’s Note: In an effort to connect more Duval County Medical Society members with residents, in each 2013 issue there will be a “Residents’ Corner” with information about a residency program in the area, details about research being done and/or a list of achievements/accomplishments of the program’s residents. This “Residents’ Corner” features the Naval Hospital (NH) Jacksonville.

By: Dr. Trevor Alexander Hray Naval Hospital (NH) Jacksonville, comprised of the Navy’s fourth largest hospital and five branch health clinics across Florida and Georgia, cares for 163,000 active duty military, retirees and their families. NH Jacksonville offers a broad spectrum of outpatient, inpatient, surgical, maternity and specialty care. Primary care includes the Navy’s largest and award-winning Family Medicine Residency Program, where residents and interns learn to provide quality, comprehensive, compassionate care. Winner of the 2013 Excellence in Teaching Award and the 2011 Family Medicine Clinical Site of the Year from the Uniformed Services University of the Health Sciences—a top-tier medical school, NH Jacksonville’s Family Residency Program has been recognized for its superior clinical training of physicians. In July, NH Jacksonville welcomed 17 new residents and interns from osteopathic and allopathic programs across the country. The Residents on the annual retreat. new residents include physicians who are returning to residency training after serving the Navy and the Marine Corps as a general medical officer (GMO) and in flight medicine. The program is excited to have been selected to participate in the ACGME’s national pilot program evaluating a 4 year training program. Beginning this academic term, one intern per year group will be selected to participate in this pilot program to expand the traditional three-year residency curriculum to four, with the objective of exploring the need for increased training and attaining certification in more outpatient procedures. Drs. Sprosty and Sanchack with the Excellence in Drs. Onos and Lafferty at the Upon completion of the first year, inTeaching Award. annual holiday party. terns will have the opportunity to go into the fleet, where they can serve in a variety of positions, such as general medical officers, flight surgeons or undersea medical officers. Residents in all stages of their training are privileged to provide the primary care needs to an assigned panel of patients, from infants to World War II veterans. Residents are required to successfully complete an Accreditation Council for Graduate Medical Education (ACGME) Family Medicine Program—such as ours at NH Jacksonville—to become board certified by the American Board of Family Medicine (ABFM). NH Jacksonville’s Family Medicine Residency Program graduated 12 interns and nine residents during a ceremony at Naval Air Station Jacksonville’s Officers’ Club on June 28. They then move on to their new assignments in the fleet as Navy medical officers. This year’s graduates were assigned to a variety of operational, outpatient and inpatient settings at facilities in Japan, Cherry Point, N.C., Meridian, M.S. and Kings Bay, G.A.

Drs. Harvey and McMullan during a casting workshop. www . DCMS online . org

Northeast Florida Medicine Vol. 64, No. 3 2013 7


Patient Page

Congenital Heart Disease Defects in the heart structure or its blood vessels which are present at birth: If your child has congenital heart disease...

If you have adult congenital heart disease...

• Your child is expected to live to be an adult.

• As you leave high school your care should be transitioned to an adult congenital heart specialist.

• There may be limitations on physical activity, but most children can be normally active. • Some children require medications and others may require surgery. Some children have other defects outside the heart that will need appropriate specialty care. • Most of your child’s healthcare can be provided by a pediatrician or family physician and will be unaltered by your child’s heart problem, such as immunizations.

• Many adults require no medications, require no additional surgery and are not limited in their jobs or physical activities. • For simple congenital heart defects, you need at least one evaluation by an adult congenital heart disease specialist. • For moderate and complex congenital heart defects you need evaluation by an adult congenital heart disease specialist at least once every two years.

• The pediatric cardiology follow-up will be determined by your child’s specific heart defect and its severity.

• You should have an evaluation by an adult congenital heart disease specialist if you are considering a pregnancy or are pregnant.

• The majority of children enjoy normal growth and school performance and appear no differently than their classmates.

• Most patients with adult congenital heart disease can exercise. You should discuss any limitations with your cardiologist.

• The more serious forms of congenital heart disease are discovered in the newborn nursery or at prenatal ultrasound.

• You should make your primary care physician or cardiologist aware of any symptoms that might be related to your heart disease, such as chest pain, palpitations or fainting.

• The origins of these heart defects are beginning to be understood and in the future may be prevented.

8 Vol. 64, No. 3 2013 Northeast Florida Medicine

• Your adult congenital heart specialist may prescribe medications that might not change the way you feel, but still have long-term benefits for your heart.

www . DCMS online . org


Guest Editorial

Shared Successes and New Challenges To paraphrase John F. Dulles, Secretary of State under President Dwight Eisenhower, “the measure of success isn’t whether you have a tough problem in front of you, it’s whether you have the same problem.” Since it became apparent with the first successful arterial shunt to treat tetralogy of Fallot (the most common type of “blue baby syndrome”) that congenital heart disease can be palliated by a surgeon’s scapel or a cardiologist’s balloon, great men and women have made it their life’s calling to care for these children. The movie “Something the Lord Made” chronicles the early struggles that cardiac pioneers Alfred Blalock, Helen Taussig and Vivien Thomas faced in developing the arterial shunt to treat tetralogy of Fallot in the 1940’s. Since that time more sophisticated equipment (ventilators, cardiopulmonary bypass circuits), interventional procedures and surgical Randall M. Bryant, MD techniques have bred more Guest Editor successful outcomes. But in keeping with Mr. Dulles’ declaration, we now have a different problem. There are now more patients with congenital heart disease older than the age of 18 years than under. How do we care for these patients? Who cares for them? What future problems have we bred by our past successes? What other new problems will arise in the wake of our most recent successes? I am honored to be the guest editor of this Pediatric and Adult Congenital Cardiology edition of the Northeast Florida Medicine Journal in which we will address several of these issues. I have asked current and former University of Florida colleagues to assist in this endeavor. Adult congenital cardiologists Arwa Saidi, MD, Brandon Kuebler, MD, and Diego Moguillansky, MD will address care of the adult congenital patient in their article entitled “Adult Congenital Cardiac Care.” Cardiac imaging plays an escalating role in the management of neonatal to adult congenital heart patients. For that reason, I have asked our specialists in advanced congenital cardiac imaging Arun Chandran, MD and Thomas Moon, MD to review this topic in their article entitled “Role of Cardiac CT Angiography and Cardiac MRI in the diagnosis of Congenital Heart Disease.” Along with a better understanding of the anatomy, our interventionalists have found new ways to correct or palliate anatomic

www . DCMS online . org

anomalies without the need for surgery. I have asked two of our interventionalists Curt Fudge, MD and Bob English, MD to address some of these newer innovations in their article entitled “Interventional Catheterization in the Care of the Congenital Heart Disease Patient.” In the past pediatric cardiologists struggled with the care of patients with hypoplastic left heart syndrome, many offering pregnancy termination or compassionate care as options to parents. Through a staged repair these patients are now expected to live into adulthood. I have asked our congenital cardiac surgeons Michael Shillingford, MD, Eric Ceithaml, MD, and Mark Bleiweis, MD to discuss the advancements that have improved survival and longevity in these most complex patients in their article entitled, “The staged surgical approach to hypoplastic left heart syndrome.” The sequelae of surgical scars and chronic volume or pressure overload in congenital heart disease promotes atrial and ventricular arrhythmias. The Electrophysiology team consisting of myself, Sharon Redfearn, ARNP, and Jason Ho, MD has provided a review of the “Arrhythmia Management in Congenital Heart Disease.” The demands of longstanding congenital heart disease may eventually lead to pump failure. Our Heart Failure team Gonzalo Wallis, MD, Jay Fricker, MD and Mark Bleiweis, MD will discuss their approach to the failing heart in their article entitled “Heart Failure Management in Congenital Heart Disease.” All too often patients with congenital heart disease are restricted from competitive and recreational sports. Finally, care of the congenital heart patient “from birth to death” has taken on an entirely new meaning. Patients with very little hope of survival 30-40 years ago now overflow our Adult Congenital Heart Disease clinics. Certainly, their successes are not solely due to the singular efforts of current day pediatric cardiologists or CV surgeons, but to the heroic efforts of their parents, primary care physicians, critical care, surgical and neonatal nurses, respiratory therapists, cath/EP staff, hospital and university leaders, and others, including the team of specialists/subspecialists who have participated in their care. An old African proverb states, “If we stand tall, it is because we stand on the backs of those that came before us.” In Northeast Florida those backs upon which we stand belong to Drs. Gerold Schiebler, Robert H. Miller, Ira Gessner, Ben Victorica, Lodewyk “Bob” Van Mierop, Jim Alexander, Jay Fricker, William J. Marvin, Don Marangi, George Armstrong and Eric Ceithaml, as well as nurses Connie Nixon and Sharon Redfearn, among others. Their efforts have resulted in shared successes for the medical community.

Northeast Florida Medicine Vol. 64, No. 3 2013 9


The Jacksonville Bank thanks the hundreds of We Care physician volunteers who care for the uninsured and underserved in our community.

(Pictured left to right) We Care Jacksonville: Todd Sack, M.D., Board President, and Sue Nussbaum M.D., M.B.A., Executive Director; The Jacksonville Bank employees and We Care volunteers: Ann Van Voorst, Jennifer Hayes and Katie Perkins; and The Jacksonville Bank Medical Team Relationship Managers: Matt Wheeler, V.P., and Lou Vaccaro, S.V.P.

Join We Care Jacksonville by contacting (904) 253-2205 or visiting their website at www.wecarejacksonville.org The Jacksonville Bank (904) 421-3040 10 Vol. 64, No. 3 2013 www.jaxbank.com

Northeast Florida Medicine

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Cardiac Disease Section

Adult Congenital Cardiac Care Background:

The Duval County Medical Society (DCMS) is proud to provide its members with free continuing medical education (CME) opportunities in subject areas mandated and suggested by the State of Florida Board of Medicine to obtain and retain medical licensure. The DCMS would like to thank the St. Vincent’s Healthcare Committee on CME for reviewing and accrediting this activity in compliance with the Accreditation Council on Continuing Medical Education (ACCME). This issue of Northeast Florida Medicine includes an article, “Adult Congenital Cardiac Care” authored by Brandon E. Kuebler, MD, Diego Moguillansky, MD and Arwa Saidi, MD which has been approved for 1 AMA PRA Category 1 credit.TM For a full description of CME requirements for Florida physicians, please visit www.dcmsonline.org. *Brandon E. Kuebler, MD is currently the Assistant Professor for the Pediatric and Adult Congenital Cardiology at the University of Florida College of Medicine - Jacksonville.

Objectives: 1. 2. 3.

Know that the prevalence of adults born with congenital heart disease is increasing due to survivorship, requiring ongoing surveillance with adult congenital cardiac specialists for long-term complications or need for additional cardiac surgery or interventions. Recognize that primary care providers in pediatrics, family medicine, internal medicine and obstetrics-gynecology have an important role in the transition from pediatric care to adult-based services. With transition, management of recommended preventative care, contraception, pregnancy and mental health will continue to be adequately addressed throughout the patient’s life. Understand that specialized adult congenital cardiac care centers are regionalized and therefore comprehensive care of the adult patient with congenital heart disease involves a multidisciplinary effort and coordination with a patient’s local providers.

Date of release: Oct. 1, 2013

Date Credit Expires: Oct. 1, 2015

Estimated Completion Time: 1 hour

How to Earn this CME Credit: 4. 5. 6.

Read the “Adult Congenital Cardiac Care” article, complete posttest following the article and fax or email your test to Patti Ruscito at patti@dcmsonline.org or 904.353.5848. Go to www.dcmsonline.org to read the article and take the CME test online. All non-members must submit payment for their CME before their test can be graded.

CME Credit Eligibility:

A minimum passing grade of 70% must be achieved. Only one re-take opportunity will be granted. A certificate of credit/completion will be emailed within four to six weeks of submission. If you have any questions, please contact Patti Ruscito at 904.355.6561 or patti@dcmsonline.org.

Faculty Disclosure:

Brandon E. Kuebler, MD reports no significant relations to disclose, financial or otherwise with any commercial supporter or product manufacturer associated with this activity.

Disclosure of Conflicts of Interest:

St. Vincent’s Healthcare (SVHC) requires speakers, faculty, CME Committee and other individuals who are in a position to control the content of this educations activity to disclose any real or apparent conflict of interest they may have as related to the content of this activity. All identified conflicts of interest are thoroughly evaluated by SVHC for fair balance, scientific objectivity of studies mentioned in the presentation and educational materials used as basis for content, and appropriateness of patient care recommendations.

Joint Sponsorship Accreditation Statement

This activity has been planned and implemented in accordance with the Essential Areas and policies od the Accreditation Council for Continuing Medical Education through the joint sponsorship of St. Vincent’s Healthcare and the Duval County Medical Society. St. Vincent’s Healthcare designates this educational activity for a maximum of 1 AMA PRA Category 1 credit. TM Physicians should only claim credit commensurate with the extent of their participation in the activity.

www . DCMS online . org

Northeast Florida Medicine Vol. 64, No. 3 2013 11


Cardiac Disease Section

Adult Congenital Cardiac Care: Does it take a village to raise a child? Brandon Kuebler, MD

University of Florida, Jacksonville, FL

Diego Moguillansky, MD and Arwa Saidi, MD University of Florida, Gainesville, FL

Abstract: Successes within congenital cardiology are yielding an exponentially increasing adult population with varying degrees of complexity. Currently, 85 percent of patients with complex cardiac defects are expected to reach adulthood. Adequate preparation during childhood and adolescence increases the likelihood for patients to maintain adequate medical follow-up and successful decision-making in their personal life as adults. In addition, neurodevelopmental and psychosocial complications associated with congenital heart disease are frequent with the need for proactive screening and treatment for those with known risk factors. To provide comprehensive care to the adult congenital heart patient, collaboration amongst primary care, obstetrics-gynecology, and general cardiology are fundamentally necessary. Furthermore, comorbities related to congenital heart disease, associated syndromes, or chronic illness often requires other specialties to participate in the care of these patients. This multidisciplinary collaboration allows, for improved management of acute and chronic medical issues as well as deployment of other resources necessary for optimal care.

Introduction Read any recent scientific journal article about caring for the adult patient with congenital heart disease (CHD), and the introduction quickly tells you of the success that the pioneers in pediatric cardiology have achieved. Over the past 60 years, the efforts by various individuals and congenital heart teams at medical institutions around the world have yielded a thriving population of survivors with congenital heart disease. This effort has led to evolution of a new frontier in medicine that is shared by other pediatric and medical specialties. Not only have pediatric cardiovascular specialties increased the survivorship of a once lethal childhood diagnoses, but so has the therapy provided, for example, by pediatric oncologists and pulmonologists. Many pediatric subspecialists find that their adolescent patients with chronic illness have to navigate the realms of the adult healthcare system. With modern therapies and interventions, patients with congenital heart disease now have a longer lifespan. This resulted in more than one million CHD adult patients in the United States. The lifetime care of patients with CHD includes ongoing surveillance for potential long-term complications. Transition is the process that prepares patients for independent adult-based health care. This includes educating the patient about the importance of lifelong cardiac

Address correspondence: Brandon Kuebler, M.D., Assistant Professor of Pediatrics, University of Florida College of Medicine Jacksonville, Division of Pediatric Cardiology, 841 Prudential Drive Suite 100, Jacksonville, FL, 32207. Email: brandon.kuebler@jax.ufl.edu 12 Vol. 64, No. 3 2013 Northeast Florida Medicine

care and learning to make decisions regarding their health. This concept of transition and the encompassing role of primary care providers (PCP) is important to the overall comprehensive care. Greater awareness among PCPs to the increased likelihood for psychosocial problems can enable proactive identification and therapy. Since local physicians provide adult congenital cardiac care, there is a clear role for having general cardiologists and PCPs involved in the care for patients in their community, especially when acute medical issues arise. It is essential that these adults maintain access to care with a collaborative team.

Transition One of the most important aspects to providing comprehensive care for the pediatric patient is to clearly define the direction and necessary steps. Typically, in most physician-patient encounters this involves anticipatory guidance, red flag warning signs for when patients need to seek care, and a specific follow-up interval. The concept of transition for the CHD patient builds upon this concept on a larger scale. It defines what possible long-term complications might arise over a patient’s lifetime, possible procedures or surgeries that may be necessary to maximize health, and identifying which providers should be involved with caring for the active medical issues as they enter into the adult healthcare system. Transition has been defined as “purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health care systems.”1 This complex concept focuses on the patient and the family, whose support has been a fundamental element to the care already provided. Having a successful transition program facilitates a safe and effective transfer of care between providers when a patient begins interacting with the adult healthcare system. One of the keys to a successful transition involves early introduction to the pediatric patient and family and with the cooperation and support of the patient’s family. The family’s role is to help promote confidence while allowing greater patient involvement in decision making and responsibility for his/her own care.2 This process should begin several years prior to the actual transfer of care, and often continues for the ensuing several years.2 The goal here is to foster autonomy for the patient and develop increasing self-awareness of their congenital heart disease. Van Deyk has recently demonstrated there are serious gaps of knowledge regarding adolescents understanding their CHD and important factors related to daily life.3 Varying levels of understanding are demonstrated in Table 1.3 Several important facts are found in the low-level of knowledge category. This demonstrates that adolescents and young adults are not learning www . DCMS online . org


Cardiac Disease Section

Table 1 Levels of knowledge adolescents have regarding their congenital heart disease, treatment, and preventative measures based upon the percentage of respondents that answered questions correctly about their care3 Low-level of knowledge (< 50% correct) - - - - - - - - - -

Name of heart defect Reasons for follow-up Effects of competitive sports Symptoms that reflect deterioration of heart disease Definition, characteristics, risk factors for endocarditis Effect of smoking and alcohol on heart disease Hereditary nature of condition Suitability of intrauterine devices as contraceptives Appropriateness of oral contraceptives Risks of pregnancy

Moderate-level of knowledge (50-80% correct) - - - -

Hence, patient transition by the primary care and specialty providers is essential to promote many of these aspects in a graduated fashion. So when the transfer of care occurs from pediatric-based providers to an adult-focused organization, this can be done with minimal anxiety, and maintaining appropriate level of care without interruption. As the patient continues into the adult-focused healthcare environment, the ultimate goal will be autonomous medical decision-making with guidance from family, personal responsibility to maintain follow-up, and a fundamental knowledge base to minimize complications related to lifestyle choices. So, in addition to a patient understanding their cardiac condition, it is essential that providers convey the implications that their condition has on factors such as: career choice, physical activity and work demands, insurance coverage to maintain access to care, contraception or risks associated with pregnancy, and end-of-life care decisions.4 Published in 2011, the American Heart Association released a scientific statement that provides guidance for many of the issues described above www . DCMS online . org

- - - -

Frequency of follow-up Occupational choices Medication regimen Sexual activities

these important details, prior to becoming responsible for their own care at a time when entering college or the workforce. These are environments where often greater individual responsibility is required for success. Should medical emergencies arise, these details are important for first-responders and emergency department providers to best assess and provide treatment. Therefore, with the assistance and reinforcement by PCPs, we all need to foster effective patient education while clarifying misconceptions and encouraging recommended follow-up.

High-level of knowledge (>80% correct) Need for regular follow-up Required diet Past treatment Dental practices

and timing to begin reviewing this information with adolescents and young adults.4

Primary Care In the care of patients with congenital heart disease, the PCP is a fundamental member of the interdisciplinary care team. In this evolving era of the medical home, the PCP serves as the focal point for care. The demands of the PCP for CHD patients are similar to those of individuals who live without congenital problems. The medical home model is one that is ideal for identifying, coordinating, and tracking the multidisciplinary medical needs for this complex patient population. These needs may include recommending preventative measures, treating common ailments, guiding involvement by multiple specialists, providing contraceptive counseling, or managing psychosocial and neurocognitive issues. Throughout childhood, various medical and social needs arise, are identified, and treated. Some of these various pediatric comorbidities are overcome, while others gradually improve, and others yet persist into adulthood. Maintaining access to care is essential for the screening of these problems. In addition, primary care and emergency room providers are often the first-line for detection of these late-onset problems such as arrhythmias or heart failure. Mackie and others, identified that 61 percent of patients who had congenital heart disease significant enough to have follow-up at least every 12-24 months, did not have any congenital cardiology Northeast Florida Medicine Vol. 64, No. 3 2013 13


Cardiac Disease Section follow-up between 18 and 21 years of age.5 Ninety-three percent of this same population though, continued to seek care for various medical problems at different points of contact, such as emergency rooms and primary care offices.5 This identifies that many patients, either by choice or misinformation, become lost to recommended congenital cardiac follow-up, yet still interact with the healthcare system enough that they could be directed back to congenital cardiology. Increased awareness about congenital cardiology services in our region and the need for lifelong surveillance can help primary care and emergency care providers with this complex patient population stay ahead of potential severe complications by directing patients back to appropriate follow-up.

Womenâ&#x20AC;&#x2122;s Health Women with congenital heart disease face a unique set of challenges related to their fertility and pregnancy. Ideally, they should seek a thorough pre-pregnancy evaluation and planning to review potential risk factors that may complicate a pregnancy for either mother or fetus. Historically, many women were strongly discouraged from getting pregnant and elective termination or sterilization procedures were performed. In this recent medical era, it is rare that a woman interested in becoming pregnant is discouraged. Currently, the only absolute contraindications to pregnancy regarding this population are pulmonary hypertension, Eisenmengerâ&#x20AC;&#x2122;s syndrome and Marfan syndrome with a dilated aortic root6 Other high risk conditions where maternal morbidity and mortality are significant and may influence personal decisions regarding pregnancy include severe fixed left ventricular outflow obstruction, mitral stenosis, and severe systemic ventricular systolic dysfunction.6 For some patients, careful planning and evaluation prior to pregnancy may necessitate surgical or other intervention to improve maternal and fetal risks. In addition to possibly requiring interventions, this pre-pregnancy planning may involve discontinuing potentially teratogenic medications, such as ACE-inhibitors or warfarin. Collaboration with obstetricians throughout pregnancy is necessary to monitor for complications and create a safe and effective birthing plan. A patient with CHD who presents to gynecology for primary care or obstetrics should be advised to establish appropriate congenital cardiac care. When women are either not ready for pregnancy or high risk for complications and do not intend to carry a pregnancy, offering contraception is an important discussion. This discussion should occur with primary care or other womenâ&#x20AC;&#x2122;s health professionals. Various factors may determine if progesterone-only or estrogen-based medications are appropriate. Estrogen-based oral contraceptives have been known to contribute to thrombus formation in individuals with increased risk. Due to this risk of thrombus formation, this class of medication is contraindicated in individuals where intracardiac shunting between the pulmonary and systemic circulation occurs potentially contributing to a paradoxical embolus. Also, patients with sluggish blood flow such as Fontan cavo-pulmonary connections, endothelial disruption such as with intravascular stents or pacing leads, or those with prior history of thrombosis may have an increased risk of thrombus formation in the setting of estrogen-based contraception. Therefore, in these situations progesterone-based alternatives such as Depo-Provera or an intrauterine device are 14 Vol. 64, No. 3 2013 Northeast Florida Medicine

favored. Tubal ligation is an effective strategy for women who no longer desire to carry future pregnancies, or have contraindications to pregnancy.

Neurocognitive and Psychosocial Demands In a recent scientific statement published in 2012, the American Heart Association sought to increase awareness of neurodevelopmental disorders in patients with CHD with recommendations to aid identification of affected individuals.7 Recognition of this common comorbidity allows for timely intervention to promote successful academic and professional goals. Also, psychosocial factors such as anxiety and depression can be identified earlier in this at risk population in order to help prevent social difficulties. Entering adulthood unaware of these psychosocial difficulties and without understanding how to cope with these challenges, further burdens patients trying to manage the physical issues related to their CHD. Many factors contribute to the incidence of neurodevelopmental disorders in patients with CHD. Naturally, the severity and complexity of the defect which altered the physiology during fetal development and subsequent need for neonatal intervention or surgery is a strong predictor.8 There is a greater likelihood of mild developmental issues in conditions characterized by decreased cerebral perfusion or oxygenation such as hypoplastic left heart syndrome or transposition of the great arteries.8 These types of defects effect brain development during fetal development, plus require neonatal surgery where low cerebral blood flow states may occur perioperatively. Unfortunately, those with an associated genetic syndrome where multiple systems are at risk for abnormalities, are the most likely to be affected from a neurodevelopmental perspective. The identification of various high risk factors for developmental delay allows for increased capture and surveillance for those with the greatest potential benefit. These high risk factors are listed in Table 2.7 Recognition of these issues should prompt providers to be vigilant for developmental delay with early intervention referrals for dedicated developmental and medical evaluations.7 For those individuals without high risk features, ongoing surveillance for subtle neurocognitive difficulties should continue throughout childhood and adolescence. Progression through academic curricula and increasing challenges may uncover various difficulties. Evaluation should be comprehensive as subtle difficulties may not be apparent on global assessment scales.7 Psychosocial and behavioral issues such as depression, anxiety, withdrawal, somatization, attention, and aggression occur in 15-25 percent of the adolescents with congenital heart disease.7 With identification of issues, strategies can be developed to equip the individual so transition into adulthood can be increasingly successful. With screening and detection of possible psychiatric disorders, the comorbidities such as academic decline, medication non-compliance, substance abuse, or high-risk behaviors may be mitigated. This would hopefully carry forward for the many adults with congenital heart disease that at some point have a mood or anxiety disorder. Kovacs described that 50 percent of adult congenital heart disease patients met diagnostic criteria www . DCMS online . org


Cardiac Disease Section with at least 1 mood or anxiety disorder, but unfortunately 39 percent never received treatment.9 Therefore, more proactive screening, identification, and intervention performed across all ages can contribute to a greater ultimate quality of life for the adult patient with congenital heart disease.

Adult Cardiac Care Over the decades, few patients with significant congenital heart disease presented to a general cardiologist. Currently, the adult CHD population is growing and demonstrating an increased demand of the health care system. The adult congenital heart population has been found to have rates of hospitalization double that of the general population9 and over a five year interval, 50 percent are hospitalized for all causes.10 Hence, there is a clear need for collaboration between classically trained adult cardiologists and the congenital cardiology network, since these patients may be admitted to community or tertiary care hospitals. There are several advantages to this collaboration where each specialty has expertise to provide comprehensive care to the patient. As in the general population, people with congenital heart disease face the same choices about their diet, tobacco use, and level of physical activity. These individuals are also susceptible to the influences of their family history and potential hyperlipidemia, hypertension, obesity, and diabetes. There is building evidence about the increasing frequency of atherosclerosis in

Table 2 High risk features for neurodevelopmental disorders in patients with congenital heart disease7 - Conditions that require open heart surgery as a neonate or infant, either cyanotic or acyanotic, such as tetralogy of Fallot, transposition of the great arteries, total anomalous pulmonary venous return, tricuspid atresia, truncus arteriosus, hypoplastic left heart or other with single ventricle physiology - Cyanotic heart disease that can be repaired beyond the neonatal or infant period such as tetralogy of Fallot with multiple aorto-pulmonary collaterals, Ebstein anomaly - History of prematurity, <37 weeks - Suspected genetic syndrome that is known to be related to developmental disorders - History of extracorporeal membrane oxygenation or ventricular assist device use - Heart transplant - Cardiopulmonary resuscitation at any time - Postoperative hospitalization >2-weeks - Perioperative seizures associated with congenital heart surgery - Microcephaly or significant abnormalities on neuroimaging

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Northeast Florida Medicine Vol. 64, No. 3 2013 15


Cardiac Disease Section the aging congenital heart patient. Giannakoulas and colleagues, identified that approximately nine percent of adults with CHD had quantitatively significant coronary atherosclerotic disease on selective angiography, and 14 percent having visually identifiable disease.11 In the setting of complex CHD, acquired cardiovascular disease may precipitate a significant decline in health and quality of life. In the population with significant coronary artery disease, strong predictors included systemic hypertension and hyperlipidemia.11 When significant atherosclerotic disease is identified, the adult cardiologist can provide a valued service of guiding patients through appropriate revascularization strategies and maintenance to help achieve the desired goals. For many adult patients with CHD, especially those with complex anatomy and who have had multiple procedures, the likelihood for late complications increases over time. These complications often involve cardiac arrhythmias and heart failure. The general adult cardiology specialty has robust data and experience using many of the medications and modalities available to treat these complications. Congenital cardiology practice has extrapolated some of the data from the adult cardiac literature to guide treatment. local general cardiologist is an asset for patients who may not live near their adult congenital cardiology center.) This relationship becomes even more valuable when heart failure or arrhythmias worsen, and the patient can be readily evaluated and managed locally to prevent a possible serious deterioration. When inpatient care is needed, the decision to transfer to the nearest hospital for inpatient adult congenital cardiac care can be determined through physician-to-physician dialogue. Collaboration with the Adult CHD physician is convenient for the patient and encourages patient compliance to achieve therapeutic goals.

Conclusion The medical demands of CHD population can be widespread, encompassing multiple medical specialties beyond those discussed. Related to co-existing conditions, or sequelae of chronic illness and complications often require pulmonary, renal, gastrointestinal/hepatology, orthopedic, and neurologic specialties to be involved. For example, situations such as with Eisenmenger syndrome, where chronic cyanosis has effects with polycythemia, there is involvement of hematology and rheumatology specialists to address increased red blood cell mass, thrombocytopenia, and associated hyperuricemia and gout. Therefore, as more complex patients are surviving into adulthood, the demand for other non-cardiac expertise to provide comprehensive collaborative care is increasing. Thankfully the skill, technology, and teamwork utilized in pediatric medicine is producing remarkable outcomes; yielding an increasing population of survivors with CHD. The number of American adult patients with CHD has exceeded 1 million, outnumbering the entire pediatric population with CHD, and growing exponentiall.4 In order to minimize setbacks in young adulthood, effective transition by PCPs and subspecialists is necessary to equip patients with the self-knowledge to make informed lifestyle choices. The medical home model for comprehensive care is being emphasized as the cornerstone with PCPs for necessary local services and with coordination with the regional adult congenital cardiology center.4,6,7 Hence, as proposed in the ACC/AHA 2008 16 Vol. 64, No. 3 2013 Northeast Florida Medicine

Guidelines for the Management of Adults with Congenital Heart Disease Executive Summary, a multidisciplinary team will best meet the needs of this unique patient population.6 This team depends on effective communication amongst PCPs, adult congenital cardiology providers, general cardiologists and others involved in the care. When patients do not live near an adult congenital cardiac center, there is definite benefit for patients to have local specialists who participate in routine surveillance and when acute issues arise. This same guideline also discusses the utilization of regional adult congenital cardiology programs to facilitate comprehensive inpatient care when necessary to address acute medical and non-cardiac surgical conditions.6 Through collaboration, effective care can be best provided utilizing resources and expertise for this emerging complex patient population. v

References 1. Society for Adolescent Medicine. Transition from child-centered to adult health care systems for adolescents with chronic conditions. A position paper. J Adolesc Health. 1993; 14: 570–576. 2. Saidi A and Kovacs AH. Developing a transition program from pediatric- to adult-focused cardiology care: Practical considerations. Congenit Heart Dis. 2009; 4: 204–215. 3. Van Deyk K, Pelgrims E, Troost E, et al. Adolescents’ understanding of their congenital heart disease on transfer to adult-focused care. Am J Cardiol. 2010; 106: 1803-1807. 4. Sable C, Foster E, Uzark K, et al; on behalf of the American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, Council on Clinical Cardiology, and Council on Peripheral Vascular Disease. Best practices in managing transition to adulthood for adolescents with congenital heart disease; the transition process and medical and psychosocial issues: a scientific statement from the American Heart Association. Circulation. 2011; 123: 1454-1485. 5. Mackie AS, Ionescu-Ittu R,Therrien J, et al. Children and adults with congenital heart disease lost to follow-up who and when? Circulation. 2009; 120: 302-309. 6. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease). J Am Coll Cardiol. 2008; 52: 1890-1947. 7. Marino BS, Lipkin PH, Newburger JW, et al.; on behalf of the American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, and Stroke Council. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012; 126: 1143- 1172. 8. Wernovsky G. Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease. Cardiol Young. 2006; 16(suppl 1): 92-104. 9. Kovacs AH, Saidi AS, Kuhl EA, et al. Depression and anxiety in adult congenital heart disease: predictors and prevalence. Int J Cardiol. 2009; 137(2): 158- 164. 10. Mackie AS, Pilote L, Ionescu-Ittu R, et al. Health care resource utilization in adults with congenital heart disease. Am J Cardiol. 2007; 99: 839-843. 11. Giannakoulas G, Dimopoulos K, Engel R, et al. Burden of coronary artery disease in adults with congenital heart disease and its relation to congenital and traditional heart risk factors. Am J Cardiol. 2009; 103: 1445–145.

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Cardiac Disease Section

“Adult Congenital Cardiac Care” CME Questions & Answers (circle one answer)/Free to DCMS Members/$50.00 charge non-members* (Return by October 1, 2015 by FAX: 904-353-5848, by mail: 555 Bishopgate Lane, Jacksonville, FL 32204 OR online: www.dcmsonline.org.)

1.

2.

3.

4.

The intent and goal for transition is: a. An alteration in the focus of discussion with young adults b. A change in the clinical management of teenagers with chronic disease c. A purposeful planned process that supports adolescents with chronic health conditions into adult-centered care d. A shift in the way that we treat adolescents in clinic A 16 year old patient with congenital heart disease or other chronic illness should: a. Be compliant with some of the medication b. Have limited input in medical decisions c. Answer all physician questions without assistance d. Take increasing responsibility for their health care e. Not be seen alone by a medical provider Based upon the study by Van Deyk and others, patients had a low-degree of understanding of which features in regards to their own care? a. The name of their cardiac defect b. Reasons for follow-up c. Symptoms that reflect deterioration of heart disease d. Fundamental knowledge about endocarditis e. All of the above A medical care home is a: a. Foster home for teenagers requiring medical supervision b. Partnership between the subspecialists and primary health care provider c. Home health care for chronically ill patients d. Partnership between family and primary health care provider

5.

Women with congenital heart disease: a. All have the same risk for complications during pregnancy as the general population b. Should seek evaluation prior to pregnancy to evaluate potential risks for mother and fetus c. All require cesarean section at time of delivery d. Have an increased risk that their child may have some form of congenital heart disease e. Answers A & C f. Answers B & D g. All of the above

6.

Which of the following is considered a high risk features for neurodevelopmental disorders that should prompt increased surveillance and screening? a. History of birth >37 weeks b. Acyanotic heart disease requiring heart surgery as a toddler c. Cyanotic heart disease requiring heart surgery as a neonate or early infancy d. Post-operative hospitalization lasting > 1 week in duration e. All of the above

7.

What percentage of adults with congenital heart disease have met criteria for either a mood or anxiety disorder? a. 25% d. 66% b. 33% e. 75% c. 50%

8.

True or False? Adult patients with congenital heart disease are susceptible to atherosclerosis and acquired coronary disease.

9.

The number of adults living with congenital heart disease in the United States exceeds: a. 500 thousand c. 10 million b. 1 million d. 100 million

10. Regarding adolescents and adults with congenital heart disease, which of these guidelines or scientific statements are helpful to providing care? a. Best practices in managing transition to adulthood for adolescents with congenital heart disease; the transition process and medical and psychosocial issues: a scientific statement from the American Heart Association. Circulation. 2011; 123: 1454-1485. b. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease). J Am Coll Cardiol. 2008; 52: 1890-1947. c. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012; 126: 1143- 1172. d. All of the above

Evaluation questions & CME Credit Information (Please evaluate this article. Circle one number using this scale: 1= Strongly Agree to 5= Strongly Disagree) The article met the stated objectives: 1 2 3 4 5 The article was appropriate to my practice: 1 2 3 4 5 The topic was current and well presented: 1 2 3 4 5 Comments:   Name (Print) Email  Address/City/State/Zip  Phone Fax

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Cardiac Disease Section

Role of Cardiac CTA and Cardiac MRI in the diagnosis of Congenital Heart Disease Arun Chandran, MD and Thomas Moon, MD

Abstract: Congenital heart disease is one of the most common con-

ditions within the spectrum of congenital lesions that affect children. Advancements in technology have led to the current practice of early and precise diagnosis of these lesions thus paving the way towards early life-saving intervention. This article aims to review the role of Cardiac Computed Tomography Angiography (CTA) and Cardiac Magnetic Resonance Imaging (CMRI) in the evaluation and management of congenital heart disease.

Introduction Congenital Heart Disease (CHD) has a median prevalence rate of 7.7 for every 1,000 live-births.1 Advances in interventional cardiac catheterization and cardiac surgery provide an excellent chance for most children born with even complex congenital heart disease to survive into their adulthood. The achievement of these excellent results however lies in the ability of the pediatric cardiologist to provide the surgeon or interventionalist with the most accurate information regarding the underlying anatomy and physiology. Albeit the fact that cross-sectional echocardiography remains the mainstay of non-invasive diagnosis of CHD, cardiac MRI and CT have become the mainstream because of their ability to describe some congenital heart lesions without the need of a more invasive diagnostic means, such as direct intra-operative observation or cardiac catheterization.

Please address correspondence regarding Cardiac CTA to: Dr. Arun Chandran Congenital Heart Center UNIVERSITY OF Florida 1600 SW Archer Road, Gainesville, FL 32610 Ph : (352)273-7770 Please address correspondence regarding Cardiac MRI to: Dr. Thomas Moon 841 Prudential Drive, Suite 100 Jacksonville, FL 32207

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Cardiac CTA (Computed Tomographic Angiography) Computed tomography essentially utilizes X-rays to visualize anatomy. The x-rays are generated from the X-ray tube while special detectors located in a gantry opposite to this source are used to detect any remaining radiation after penetration of the organ being studied. This measured radiation data is then converted to an image format using special algorithms. CT scanners were initially introduced in 1974 and there has been a rapid progress in the type of scanner technology.2 Initial scanners were axial scanners but subsequent progress resulted in the introduction of both spiral and helical scanners in the 1980s and 1990s. In these scanners, the gantry moves continuously around the patient while the table moves constantly such that there is exposure to the X-rays in a spiral fashion thus resulting in a rapid acquisition. The concept of multi-slice acquisition (i.e. the ability to detect multiple slices of images at the same time) was introduced in 1989 starting with the ability to acquire two detector rows or slices in 1991, followed by four slices in 1998, eight to 16 slices in 2002, 64 slices in 2004 and finally the 320-slice scanner in 2008. The blood pool-tissue contrast ratio and so the spatial resolution for a CT is much better than cardiac MRI. Consequently, cardiac CTA in CHD is used predominantly for pre or post-operative delineation of tiny extra-cardiac lesions especially in neonates and infants. There is a growing body of evidence towards using it for congenital coronary artery imaging too. Rapid acquisition sequences (typically between 10 and 15 seconds) obviate the need for any sedation and the only requirement is for the placement of a peripheral IV for dye injection. Again, this is especially useful in small children many of whom are critically ill3 during this stage of their life. Adequate vascular enhancement is obtained using a protocol that utilizes an iodinated contrast agent (such as Visipaque) at the dose of 2ml/kg with an infusion rate of ½ ml/second.1 a. Aortic arch anomalies: Native coarctation of the aortic arch is probably one of the most extensively studied lesions by CTA. The lesions are typically focal and juxtaductal in location (Figures 1 and 2, page 20) but many Northeast Florida Medicine Vol. 64, No. 3 2013 19


Cardiac Disease Section

Figure 1

CTA sagittal plane showing focal coarctation of the aortic arch

Figure 2

CTA 3-Dl reconstruction showing focal coarctation of aorta with multiple collaterals

are associated with hypoplasia of the transverse arch. Anomalies of the aortic arch and great vessels (Figure 3) are also easily studied using this modality thus obviating the need for the more traditional and invasive barium swallows or bronchoscopy.4 Cardiac CTA is also used to delineate small aorto-pulmonary collaterals from the aortic arch in appropriate cases, such as the child with tetralogy of Fallot/pulmonary atresia (TOF/PA). This information assists in the decision-making process related to primary repair with unifocalization of the collaterals. b. Pulmonary artery (PA) anomalies: Post-operative or isolated pre-operative branch PA stenosis or dilation can be clearly demonstrated using the CTA (Figures 4 and 5) c. Pulmonary venous anomalies: Anomalous pulmonary venous return (APVR) is a lesion involving abnormal drainage of some (partial: PAPVR) or all (total: TAPVR) of the pulmonary veins to a vascular structure other than the morphological left atrium. PAPVR could occur to the left innominate vein (Figures 6 and 7) or coronary sinus while TAPVR typically involves drainage of a common pulmonary venous confluence to one of the abdominal venous structures (Figure 8, page 22). The latter is typically obstructed in the clinical setting of a critically ill neonate and the rapid time to diagnosis provided by the CTA contributes to a safe and usually life-saving outcome. d. Coronary artery anomalies: Congenital coronary artery anomalies involve anomalies of origin (Figure 9, page 22). Coronary CTA is also essential in delineating such rare entities as coronary-cardiac fistulae (Figure 10, page 23) thus providing a blueprint for subsequent interventional device closure. e. Extra-cardiac thoracic anatomy: The CTA rapidly evaluates the trachea-bronchial tree and other thoracic structures as they lie in their natural relationship to the adjacent cardiac structures. This is important considering the increased incidence of such anomalies in children

20 Vol. 64, No. 3 2013 Northeast Florida Medicine

Figure 3

CTA coronal view showing aberrant right subclavian artery (arrow) from left aortic arch

with CHD in addition to the secondary effect of cardiac chamber enlargement on surrounding extracardiac structures. (Figure 11, page 23) f. Radiation exposure and Safety: Radiation exposure and the long-term potential for malignancy remains the most important point of discussion when it comes to CTA imaging. All individuals are exposed to background radiation of approximately 3.6mSv/year.5 The dose of an AP/lateral chest x-ray is 0.1mSv and conventional diagnostic coronary angiography is 7mSv.6 Despite the lack of accurate and specific information regarding exact long-term malignancy risks from medical CTA exposure in small children, the general approach to this population is the risk of cancer increases at any level of ionizing radiation regardless of the dose. This has resulted in the ALARA (As Low As Reasonably Achievable) principle which recommends that protocols be designed to obtain diagnostic images at the lowest possible dose to the patient.6 In current cardiac CTA imaging this is achieved by dose-reduction modulation which involves modifying CT protocols and variables such as kilovoltage, current, exposure time, patient thickness and scan pitch). The University of Florida- Gainesville has published some of the lowest published pediatric cardiac CTA exposure rates with a mean effective radiation exposure rate of 0.8 Âą 0.39 mSv.3 In the right hands and with the appropriate technology and protocols, cardiac CTA continues to play a very important role in the diagnostic continuum of the pediatric cardiologist while maintaining appropriate usage and radiation exposure.

Cardiac MRI (Magnetic Resonance Imaging) The field of cardiology has benefitted greatly from advances in technology. Many decades ago, a cardiologist had very few diagnostic options and often an invasive cardiac catheterization was necessary to define the pathology in question. www . DCMS online . org


Cardiac Disease Section

Figure 4

CTA axial view showing severe right pulmonary artery hypoplasia in a patient following repair of tetralogy of Fallot (TOF)

Figure 5

CTA axial view showing severe pulmonary artery enlargement in a patient with unrepaired tetralogy of Fallot with absent pulmonary valve syndrome.

Figure 6 and 7

CTA coronal views (2-D and 3-D) showing supracardiac PAPVR with a common pulmonary vein connecting to the left innominate vein

The advent of cardiac ultrasound, or echocardiography, has certainly revolutionized the field but there are often times when an echocardiogram alone is insufficient and other imaging modalities become necessary. This is especially true in the adult population where frequently the lungs prevent optimal assessment of the heart and associated vasculature.

Table 1 Advantages and Disadvantages of Cardiac MRI

MRI takes advantage of the phenomenon of nuclear magnetic resonance. It was discovered that certain elements in nature will emit a detectable signal after exposed to radiofrequency energy. These elements include only those with an odd number of protons or neutrons in the nucleus to create a magnetic moment associated with its net spin. As hydrogen atoms are abundant in the human body (especially in fluid and fat tissue), the signal from these atoms are targeted in MRI. While the individual signal might be very small from any one given atom, the sheer number of hydrogen atoms in the human body compensates for this. Unfortunately, under normal circumstances, the individual magnetic moments are randomly oriented and there is no net magnetization. However, the individual spins of the hydrogen atoms will align when placed in a strong magnetic field. For the purposes of cardiac MRI, the magnetic field strength is typically 1.5 Tesla. With the patient in the magnet, a series of carefully controlled radiofrequency pulses are applied to a patient and the resulting signals are collected and translated into familiar images.7

• High quality images unaffected by lung interference or chest deformities • Able to quantify ventricular volume, mass and function • Able to measure chamber size, vessel and valve diameters • Calculate flow through a designated area such as a valve or vessel • Vascular angiography • Assess tissue properties • No radiation • Evaluation of extra-cardiac anatomy

There are several advantages and disadvantages to cardiac MRI (Table 1). Electrocardiography and echocardiography have advanced to the point that it has become quite portable so availability is not the issue it once was. An MRI scanner, however, has to be built to exact specifications and is quite an expense both from a hardware and software point of view. For this reason, relatively few scanners are available and those with the expertise necessary to perform the studies are also rare. For all these reasons, MRI’s are rather expensive when www . DCMS online . org

ADVANTAGES

DISADVANTAGES • Availability issues • Relatively expensive • Special expertise required to obtain, process and interpret images • Images subject to artifact (motion, metallic, etc.) • Long scan and post-processing time • Unable to determine ventricular and vascular pressure data • Not always practical in all patients • Potential dangers from strong magnet

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Cardiac Disease Section

Figure 8

3-D CTA reconstruction of infradiaphragmatic TAPVR

Figure 9

Axial CTA view of the left main coronary artery originating from the right coronary sinus with a subsequent inter-arterial course between the aorta and pulmonary artery.

compared to other non-invasive forms of imaging. As with any MRI study, cardiac MRIâ&#x20AC;&#x2122;s especially require the subject to be as still as possible for the duration of the scan to prevent image artifact. In addition, the simple motion of breathing can significantly impact image quality. To overcome this problem, the subject is asked to temporarily suspend respirations for short periods of time (typically for 10 to 15 seconds). If done properly, the resulting image quality is excellent. The tradeoff is that many breath-holds are required which can lead to long scan times, often taking an hour or more per study. Because of this issue most children and even some adults (i.e. those with developmental disorders) cannot cooperate effectively and more intrusive measures are required. These individuals must be anesthetized, intubated and paralyzed for the duration of the study to control to breathing motion which adds a layer of difficulty and risk. Cardiac MRI images often have a high spatial resolution but imaging smaller vessels like coronary arteries can sometimes be difficult and for this reason cardiac CTA is often chosen instead. Finally, a 1.5 Tesla magnet is extremely powerful and therefore potentially dangerous. Careful screening of each and every patient must be done prior to allowing an individual in the actual scanner. Certain implanted metallic objects preclude scanning due to safety concerns. This is especially true for individuals with pacemakers or defibrillators. On the other hand, there is a big upside to cardiac MRI. High quality images of the heart and vasculature can be ob22 Vol. 64, No. 3 2013 Northeast Florida Medicine

tained which is a significant problem with echocardiography in many individuals, especially larger adults. MRI imaging planes can determine the size of individual cardiac chambers. This is particularly noteworthy as images can be obtained at specific times during the cardiac cycle. In other words, the size of the ventricles can be determined at end-diastole, when the volume is the greatest, and at end-systole, when the volume is the smallest. From here, simple calculations can be used to determine the stroke volume, cardiac output, ejection fraction and ventricular mass.8,9 Similar calculations are routinely done on the left ventricle using echocardiography but only under ideal circumstances. This cannot reliably be done in the event of poor image quality or if the left ventricle is an unusual shape. Furthermore, the right ventricle has a much more complex 3-dimensional shape and therefore determining its volume by echocardiography is often inaccurate. Special techniques can be used during a cardiac MRI study to determine the flow of blood through a vessel. This is especially useful for cardiac lesions which have intra- or extra-cardiac shunts and can help determine differential pulmonary blood flow as well.7 This technique is also very informative as it can quantify the degree of regurgitation through valves. Magnetic resonance angiography (MRA) can also be done using intravenous contrast agents containing gadolinium. This can provide invaluable information about vascular anatomy. It was also discovered that once the gadolinium was given it would quickly become distributed into

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Cardiac Disease Section

Figure 10

CTA modified sagittal view showing a large fistula from the right coronary artery to the right ventricle.

Figure 11

3D CTA reconstruction showing compression of both main bronchi in addition to lateral compression of the trachea. 10

11

Figure 12

MR angiogram of a patient with DiGeorge syndrome and an interrupted right aortic arch with a patent ductus arteriosus providing blood flow to the descending aorta.

Figure 13

MRI axial black blood imaging of a patient with a vascular ring from a right aortic arch with a retroesophageal aberrant left subclavian artery. 12

Figure 14

13

Coronal MR angiogram of a patient who was born with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries s/p surgical unifocalization and right ventricle to pulmonary artery conduit. The image acquisition was obtained early after Gadolinium injection, thus highlighting the pulmonary vasculature.

Figure 15

MRA 3D reconstruction showing total anomalous pulmonary venous return (white circle) draining to the left innominate vein. 14

15

the interstitial space. However, the distribution is not equal and by taking advantage of this difference, MRI can help distinguish between viable and non-viable tissue to look for evidence of scar or edema. Along the same lines, different tissues have different density of hydrogen atoms and MRI can detect these variations. For example, this can help not only detect intracardiac tumors, but it can also differentiate one type of tumor from another.10 The latest MRI contrast agents are typically considered much less renal toxic than the iodinated contrast used for CTA. Other advantages of MRI include a lack of radiation so they do not carry the same risk as does CT scanning. For this reason, they are now routinely used on pregnant women to evaluate the fetus for various anomalies. Also, it is routine to obtain scout images of much of the chest and abdomen so non-cardiac anomalies are frequently detected.

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Cardiac MRI is often performed for many of the same reasons as cardiac CTA and below are some examples of these indications. a. Aortic arch anomalies: Echocardiograms in newborns can often provide all the necessary details to make a complete and accurate diagnosis. However, there are times when this is not the case and complicated arch anomalies can often cause confusion. Newborn infants suspected of arch anomalies are now commonly being scanned without the use of sedation (Figure 12). They are simply wrapped securely and given ear protection. Also, vascular rings might be suspected by echocardiogram but can usually be shown with great specificity by MRI (Figure 13).

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Cardiac Disease Section

Figure 16

Ventricular short axis stack cine images with calculation of right ventricular end-diastolic volume (left 14 images) and end-systolic volume (right 14 images) in a patient with repaired tetralogy of Fallot with chronic volume overload from pulmonary regurgitation.

b. Pulmonary artery (PA) anomalies: MRA will delineate the pulmonary vessels and calculate the differential pulmonary blood flow. (Figure 14, page 23) MRA illuminates the pulmonary vessels and allow the calculation ofthe differential pulmonary blood flow. c. Pulmonary venous anomalies: Partial or total anomalous pulmonary venous return can often present a diagnostic dilemma as it is hard to account for each pulmonary vein by echocardiography. MRA can fully detail the pulmonary venous anatomy and provide the surgeons with a good spatial roadmap on how best to approach surgical repair (Figure 15, page 23). d. Ventricular volume, mass, function: One of the most useful pieces of information that can be obtained from cardiac MRI is accurate determination of ventricular size and function. Chronic volume loading lesions such as pulmonary insufficiency following repair of tetralogy of Fallot will cause dilation of the right ventricle. Knowledge of the specific indexed right ventricular end-diastolic volume and systolic function can give valuable information when deciding timing of pulmonary valve replacement (Figure 16). Occasionally there is a neonate with a left

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ventricle that by echocardiogram might be too small to support an adequate cardiac output. The decision to pursue a single versus a two ventricular repair is not taken lightly. Cardiac MRI can help determine the adequacy of the left ventricle by better determining its systolic and diastolic volumes (Figure 17). e. Valve dysfunction: While the severity of valve regurgitation can be qualitatively estimated by echocardiography, a specific regurgitation fraction can be calculated using MRI by dividing the regurgitated volume of blood by the total volume of blood flowing forward across the valve. f. Extra-cardiac thoracic anatomy: Many thoracic structures exist in the chest apart from the heart and the incidence of anomalies of these structures is higher in the setting of congenital heart disease. Heterotaxy syndrome, diaphragmatic hernias, hiatal hernias, and tumors are among some of the problems that MRI can help delineate (Figure 18). In addition, post-surgical findings such as pseudo-aneurysms are optimally seen by MRI (Figure 19). v

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Cardiac Disease Section

Figure 17 Ventricular short axis stack cine images with calculation of left ventricular enddiastolic volume in a patient with a left ventricle which borders on being too small.

Figure 18

Axial and coronal black blood imaging of a patient with heterotaxy syndrome and a large hiatal hernia (white circle).

References: 1. Robert H. Anderson et al .Pediatric Cardiology Third Edition, 2010 2. Alec J. Megibow et al. CT for technologists, Sept 2002 3. Al-Mousily F, Shifrin RY, Fricker FJ, Feranec N, and Quinn NS, Chandran A: Use of 320 detector computed tomographic angiography for infants and young children with Congenital Heart Disease. Pediatric Cardiology. 2011 Apr; 32(4):426-32. 4. Chandran A, Fricker FJ, Schowengerdt KO, Cumming WA, Saidi A, Spencer CT, Paolillo J, and Samyn MM: An institutional review of the value of computed tomographic angiography in the diagnosis of congenital cardiac malformations. Cardiology in the Young. 2005; Feb; 15(1):47. 5. Gerber TC et al Radiation dose and risk of malignancy from cardiovascular imaging. Source: Up-to-date Updated: 6/15/2013 6. Gerber TC et al. Ionizing radiation in cardiac imaging: a science advisory from the American Heart Associawww . DCMS online . org

Figure 19

Chest x-ray (left image), axial black blood image (middle image) and MRA (right image) of a patient with tetralogy of Fallot who developed a pseudoaneurysm (white circle).

tion Committee on Cardiac Imaging of the Council on Clinical Cardiology and Committee on Cardiovascular Imaging and Intervention of the Council on Cardiovascular Radiology and Intervention. Circulation. 2009; 119(7):1056. 7. Fogel MA. Principles and Practice of Cardiac Magnetic Resonance in Congenital Heart Disease. Form, Function and Flow. Wiley-Blackwell Publishing. 2010. 8. Maddahi J, Crues J, Berman DS, et al. Noninvasive quantification of the left ventricular myocardial muscle mass by gated proton nuclear magnetic resonance imaging. J Am Coll Cardiol. 1987; 10: 682-92. 9. Dellâ&#x20AC;&#x2122;Italia LJ, Blackwell GG, Pearce DJ, et al. Assessment of ventricular volumes using cine magnetic resonance imaging in the intact dog. A comparison of measurement methods. Invest Radiol. 1994; 29:162-7. 10. Beroukhim RS, Prakash A, Buechel ER, et al. Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience. J Am Coll Cardiol. 2011 Aug 30; 58(10):1044-54. Northeast Florida Medicine Vol. 64, No. 3 2013 25


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Cardiac Disease Section

Interventional Catheterization in the Care of the Congenital Heart Disease Patient James C. Fudge, Jr., MD, MHS and Robert English, MD

Abstract: Interventional cardiac catheterization serves an ever increasing role in the treatment of patients with congenital heart disease (CHD). From infants with newly diagnosed CHD to the rapidly growing population of adults with CHD, therapeutic treatment options continue to expand. In this article, we discuss a number of types of CHD and the interventional approaches to their treatment. Specifically, we review the treatment of atrial septal defects, ventricular septal defects, patent ductus arteriosus, dysfunctional pulmonary valves and right ventricular to pulmonary artery conduits, and coarctation of the aorta.

Introduction Cardiac catheterization plays an integral role in the management of patients with congenital heart disease (CHD) from birth through adulthood. Historically, cardiac catheterization has been considered the gold standard for diagnosis of CHD. With the evolution of non-invasive imaging modalities such as echocardiography, computed tomography, and magnetic resonance imaging, the need for diagnostic catheterization has decreased substantially. The focus of cardiac catheterization in CHD has shifted to the development of interventional treatment strategies. Advancements in technique and equipment have allowed interventionists to provide a less invasive approach to the definitive treatment of many types of CHD while complimenting the surgical treatment of others. We discuss the interventional approach to the treatment of atrial septal defects (ASD), ventricular septal defects (VSD), patent ductus arteriosus (PDA), dysfunctional right ventricular to pulmonary artery conduits, and coarctation of the aorta (CoA).

atrial septum. Its location makes it amendable to transcatheter closure. The other types of ASD require surgical repair. Defects in the atrial septum lead to left to right shunting across the septum and an increase in pulmonary blood flow. The amount of blood flow across the septum is dependent on the size of the defect and the relative compliance of the ventricles. Large atrial shunts lead to symptoms from excess pulmonary blood flow and right-sided heart failure, including frequent pulmonary infections, fatigue, exercise intolerance, and palpitations. ASD are most commonly identified in asymptomatic children after evaluation of a murmur found during a routine physical examination. Symptoms are more common in young children with underlying lung disease such as bronchopulmonary dysplasia. Adults often present in the third and fourth decades of life with arrhythmias and symptoms of exercise intolerance. Recommendations for ASD closure include evidence of right atrial and right ventricular enlargement with or without symptoms2,3. Closure is also reasonable in the presence of paradoxical embolism or documented orthodeoxia-platypnea. Device closure is not recommended in an ASD without evidence of right heart enlargement, an ASD of the non-secundum type, and in patients with irreversible

Figure 1

Types of atrial septal defects.

Atrial Septal Defects ASD are a common type of congenital heart disease, accounting for approximately 10-15 percent of all congenital heart defects. ASD can be characterized into four different types including secundum, primum, sinus venous and coronary sinus septal defects (Figure 1)1. The most common type is the secundum ASD, which is usually centrally located within the

Please send correspondence to: James C. Fudge, Jr., MD, MHS; Congenital Heart Center, University of Florida COM, 1600 SW Archer Road, HD-303, Gainesville, FL, 32610; jcfudge@ufl.edu

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(1) Primum ASD (2) Secundum ASD (3) Superior and inferior sinus venosus ASD (4) Coronary sinus ASD

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Cardiac Disease Section

Figure 4

Echocardigoraphic images of ASD closure. A

Figure 2

Amplatzer Septal Occuder

Figure 3

Gore HELEX Septal Occluder

pulmonary hypertension. If left unrepaired, ASD can lead to arrhythmias and progressive pulmonary vascular disease. Transcatheter closure of ASD was first described in 1976 by Mills and King.4 Numerous studies have shown outcomes from transcatheter device closure of secundum ASD to be comparable to surgical outcomes in selected patients.5,6,7 Device closure of secundum ASD is associated with low complication rates and short hospitalizations. Transcatheter ASD closure has become the treatment of choice in many institutions. There are currently two devices approved for secundum ASD closure in the United States including the AMPLATZER Septal Occluder (St. Jude Medical, Inc.) and the HELEX Septal Occluder (WL Gore and Associates). The AMPLATZER Septal Occluder is available in a wide range of sizes and features a left and right atrial disk connected by a self centering connecting waist (Figure 2). It has been used to successfully close defects as large as 38 mm. The Gore HELEX Septal Occluder is also available is a variety of sizes and consists of a circular nitinol wire frame covered by a ePTFE membrane which forms two opposing disks that are locked into place when the device is deployed (Figure 3). The HELEX Septal Occluder is suitable for closure of small to moderate-sized defects less than or equal to18 mm in diameter. Percutaneous ASD closure can be performed under conscious sedation or general anesthesia in the catheterization lab. Standard hemodynamics are obtained with assessment of the pulmonary to systemic blood flow ratio and the pulmonary vascular resistance (PVR). ASD closure is most commonly performed using transesophageal or intracardiac echocardiography (Figure 4). Patients remain in the hospital for overnight observation. Antiplatelet or anticoagulation therapy and subacute bacterial endocarditis (SBE) prophylaxis is recommended for at least 6 months following device implantation. Follow up intervals may vary by implanting physician and the type of device implanted. The FDA requires follow up

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(A) TEE demonstrating a secundum ASD.

B

(B) TEE demonstrating device closure of an ASD.

at one week, one month, six months, and yearly following implantation of the Amplatzer Septal Occluder given the potential risk for device erosion. Erosion occurs when a device irritates through the wall of the heart or an adjacent structure, which can potentially result in a pericardial effusion, cardiac tamponade and even death. Patients are typically allowed to return to their normal level of activity four to six weeks following device implantation.

Ventricular Septal Defects VSD are the most common congenital heart defect, accounting for approximately 20 percent of congenital heart disease. Congenital VSD can be characterized into four different types including perimembranous, inlet, outlet and muscular defects (Figure 5). The proximity of aortic and atrioventricular valves to the first three types of defects has made them less than ideal for transcatheter closure although percutaneous closure of perimembranous VSD is common outside of the United States. The relative remote location of muscular VSD from these valve structures makes them more ideal defects for device closure. The US Food and Drug Administration has limited approval for device closure to muscular VSD. VSD vary in their size and timing of presentation. Spontaneous closure may occur in up to 30 to 40 percent of patients with membranous and muscular VSD in the first six months of life. Those with large VSD typically develop congestive heart failure(CHF) from pulmonary overcirculation within the first six to eight weeks of life and will likely require surgical or transcatheter closure. Presentation can be delayed into childhood or even adulthood in patients with restrictive VSD or patients whose symptoms of CHF were not recognized earlier in life. Recommendations for VSD closure include evidence of a hemodynamically significant VSD as defined by a pulmonary to systemic blood flow ratio of greater than or equal to 2:1 or left heart volume overload2,3. Closure is not www . DCMS online . org


Cardiac Disease Section

Figure 5

Types of ventricular septal defects including inlet, outlet, perimembranous and muscular VSD.

recommended in patients with pulmonary artery hypertension and a net right to left shunt. If left untreated, VSD can lead to development of arrhythmias from chronic volume overload and cardiac dilation, as well as progressive pulmonary vascular disease leading to irreversible pulmonary artery hypertension and Eisenmenger physiology. Patients with hemodynamically significant muscular VSD may be candidates for device closure. Individuals are selected for device closure based upon the echocardiographic assessment of their defect. The most favorable defects for transcatheter closure are apical, mid muscular and anterior muscular defects. The Amplatzer Muscular VSD Occluder (St. Jude Medical, Inc.) is the only device currently being manufactured in the United States with FDA approval for closure of muscular VSD (Figure 6). Percutaneous VSD closure is typically performed under general anesthesia in the catheterization lab. Standard hemodynamics are obtained with assessment of the PVR. VSD closure is usually performed with the use of transesophageal echocardiography and fluoroscopic guidance. Patients remain in the hospital for overnight observation. Antiplatelet or anticoagulation therapy and SBE prophylaxis is recommended for 6 months following device implantation. Follow up occurs at intervals determined by the implanting physician with echocardiographic evaluation at a minimum of every 6 months until complete closure of the defect has been confirmed. Patients are typically allowed to return to their normal level of activity four to six weeks following device implantation.

Patent Ductus Arteriosus As an isolated anomaly, a PDA represents approximately 10 percent of all congenital heart disease. A PDA is a postnatal communication usually between the main pulmonary artery and the descending thoracic aorta that is due to persistent patency of the fetal ductus arteriosus. With the development

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Figure 6

Amplatzer Muscular Ventricular Septal Defect Occluder

Figure 7

Amplatzer Ductal Occluder

of a many new devices and innovative techniques, transcatheter closure of the PDA has become the standard approach for closure. Surgical ligation is still utilized in select cases when device closure is not optimal, such as the PDA with unsuitable anatomy or in the premature infant. The PDA is typically functionally closed by 24 hours of life. In cases of persistent patency of the ductus, the pathophysiology relates to reversal of ductal flow due to the normal decline in the PVR. This results in a left to right shunt from the aorta to the pulmonary artery and ultimately increased volume and workload on the left heart. Symptoms of the left to right shunt are dependent on the size of the shunt and the ability of the left ventricle to handle the volume load. Most patients with a small to moderate PDA are asymptomatic and the diagnosis is suspected based on the characteristic continuous murmur. A large PDA may present with frequent respiratory infections, heart failure from pulmonary overcirculation, and failure to thrive. Recommendations for PDA closure include evidence of left heart enlargement and prevention of endocarditis/ endarteritis.2,3 Closure may also be indicated in the presence of pulmonary artery hypertension when there is a net left to right shunt. There is controversy regarding the closure of the so-called â&#x20AC;&#x153;silentâ&#x20AC;? or inaudible PDA. Closure is not recommended in patients with pulmonary artery hypertension and a net right to left shunt. When unrepaired, PDA, like VSD, can lead to progressive pulmonary hypertension and Eisenmenger physiology. Catheter occlusion of the PDA was first described in 1971 by Porstmann and colleagues.8 Throughout the last four decades, advancement in device technology and techniques has made trans-catheter closure of the PDA standard of therapy with few exceptions. The most common devices currently used for PDA closure include various types of coils, vascular plugs, and the AMPLATZER Ductal Occluder (St. Jude Medical, Inc.) (Figure 7). The AMPLATZER Ductal Occluder is the only

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Cardiac Disease Section FDA approved device for PDA closure. Transcatheter PDA closure can be performed under conscious sedation or general anesthesia in the catheterization lab. Standard hemodynamics are obtained with assessment of the pulmonary to systemic blood flow ratio and the PVR. PDA closure is most commonly performed using angiography and fluoroscopic guidance (Figure 8). Patients typically remain in the hospital for overnight observation. Antiplatelet therapy with aspirin may be used at the discretion of the implanting physician. SBE prophylaxis is recommended for at least six months following device implantation. Follow up intervals may vary by implanting physician. Patients are typically allowed to return to their normal level of activity a few weeks following device implantation.

Dysfunctional Pulmonary Valves and Right Ventricular to Pulmonary Artery Conduits Surgically placed pulmonary valves or right ventricular to pulmonary artery conduits are prone to fail over time leading to obstruction and/or insufficiency of the valve. The hemodynamic sequelae of conduit failure can be significant. Chronic pulmonary insufficiency is a treatable cause of right ventricular dilation and right ventricular failure. Likewise, conduit stenosis can lead to right ventricular hypertrophy and even failure depending on the severity of the stenosis. Percutaneous valvular pulmonary valve replacement offers a potential relief for both conduit insufficiency and stenosis. The optimal timing for pulmonary valve replacement remains controversial. Surgical pulmonary valve replacement has been shown to improve symptoms, decrease right ventricular size, improve exercise tolerance, and reduce the incidence of arrhythmias.9-13 Pulmonary valve replacement does not sig-

Figure 8

Example of PDA occlusion with an Amplatzer Duct Occluder. A

(A) Lateral aortic angiogram demonstrating a PDA (arrow).

B

(B) Lateral aortic angiogram demonstrating occlusion of the PDA following device implantation.

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nificantly alter the right ventricular systolic function.10 This advocates for pulmonary valve replacement prior to the onset of ventricular dysfunction and clinical symptomatology. Consequently, we must depend on early markers for right ventricular failure such as ventricular dilation and conduit obstruction to help determine when pulmonary valve replacement is optimal. Numerous studies have attempted to determine the threshold volume at which pulmonary valve replacement no longer leads to normalization of right ventricular volumes.9,13 End diastolic volumes greater than 160-170 ml/m2 and end systolic volumes greater than 80-82 ml/m2 represent the upper limit to which pulmonary valve replacement can be delayed as normalization of right ventricular volumes does not occur beyond these cutoffs.9,14 Transcatheter pulmonary valve replacement was first described by Bonhoeffer and colleagues in 2000.15 Since that time, the Medtronic Melody valve (Medtronic, Inc.), which is a bovine jugular vein sutured within a platinum iridium stent, has been FDA approved under the Humanitarian Device Exemption program (Figure 9). Post-approval studies are ongoing to assess the long term outcomes of the initial study patients. In the United States, the Melody valve is indicated in patients with an existing right ventricular outflow tract conduit greater than or equal to 16 mm in diameter at the time of surgical implantation and evidence of conduit failure. A second transcatheter valve, the Edwards SAPIEN valve was initially designed for percutaneous aortic valve replacement. It consists of three bovine pericardial leaflets sewn into a 14 or 16 mm stainless steel stent (Figure 10). The SAPIEN valve is currently being studied for use in the pulmonary position in the COMPASSION trial Figure 9 (National Clinical Trials ID The Medtronic Melody NCT00676689). The trial Transcatheter pulmonary is currently in the feasibility valve consists of bovine phase with a goal of receivjugular vein sutured in a ing Humanitarian Device 28mm platinum-iridium Exemption from the FDA. stent. The valve is inserted Early outcomes from the first using a 22 French balloon 36 implants were published in balloon catheter delivery by Kenny and colleagues system (Ensemble). and demonstrated a significant reduction in the peak Doppler gradient following device implantation along with an expected reduction in pulmonary regurgitation and an improved in New York Heart Association class.16 Transcatheter pulmonary valve implantation is performed under general anesthesia in the catheterization lab. After standard hemodynamics and angiography are performed, further information regarding the dimensions and distensibility of

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Cardiac Disease Section Figure 11

Lateral pulmonary artery angiograms pre and post Melody valve implantation. B

A

Figure 10

The Edwards SAPIEN Transcatheter heart valve consists of bovine pericardium sutured in a 14 or 16 mm stainless steel stent. The valve is mounted on a 23 or 26mm×3 cm high pressure balloon and delivered via a specially designed retroflex catheter through a 24–26 French sheath.

(A) Lateral pulmonary artery angiogram demonstrating stenosis of the right ventricle to pulmonary artery homograft.

the right ventricular outflow tract can be gained by balloon sizing and balloon compliance testing. Coronary anatomy should be defined with particular attention to the relationship of the coronary arteries to the right ventricular outflow tract. Pre-dilation and pre-stenting of the conduit may be required to prepare the conduit for valve implantation. Implantation of the valve is performed using fluoroscopic guidance and the valve is assessed by angiography (Figure 11) and transesophageal or intracardiac echocardiography. Patients remain in the hospital for overnight observation. Antiplatelet therapy with aspirin and SBE prophylaxis is recommended. Patients are typically allowed to return to their normal level of activity four to six weeks following device implantation.

Coarctation of the Aorta CoA is a rare congenital heart defect occurring in 6 to10 percent of all cases of CHD. The prevalence of CoA is increased in certain disorders such as Turner syndrome. The most common associated cardiac defect is a bicuspid aortic valve.17 The most important noncardiac associated anomaly is intracerebral aneurysm occurring in approximately 10 percent of all cases. CoA produces obstruction to blood flow through the aorta resulting in reduced blood flow to areas distal to the aortic obstruction. The most common location for CoA is just distal to the origin of the left subclavian artery.

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(B) Lateral pulmonary artery angiogram following Melody valve implantation demonstrating no residual homograft stenosis or insufficiency.

CoA presents with a spectrum of severity, in which younger age at presentation correlates closely with the severity of the obstruction. Neonates may present with acute circulatory shock as a result of ductal closure and severe obstruction to aortic blood flow. These patients are usually treated with prostaglandin E1 infusion to reestablish ductal patency and ionotropic support to improve cardiac output prior to repair. If medical therapy to stabilize these patients is unsuccessful then urgent intervention is required. Infants and children may present with symptoms of failure to thrive, whereas older children and adults may present with signs and symptoms such as upper extremity hypertension, headaches and claudication. These patients usually present for elective repair of their CoA. Recommendations for repair of CoA include any patient who presents with CHF or circulatory shock and patients with ≥ 20 mmHg systolic gradient across the CoA. If left untreated, patients with CoA are at high risk for mortality from CHF, aortic rupture, infectious bacterial endarteritis, and intracranial hemorrhage.18 Treatment for CoA has evolved over the years. Traditionally, surgical repair has been the primary treatment for native CoA and remains the “gold standard” at most centers. In recent years, balloon angioplasty with or without stent implantation has emerged as a less invasive alternative. Balloon angioplasty for native CoA can be performed safely and with excellent acute results in patients outside of the neonatal period; how-

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Cardiac Disease Section

A

B

Figure 12

Lateral aortic angiograms of CoA. (A) Lateral aortic angiogram of CoA prior to stent angioplasty. (B) Lateral aortic angiogram of CoA following stent angioplasty.

ever there is a higher incidence of recurrent CoA in patients less than 6 months of age and a small risk of aneurysm formation with balloon dilation of native CoA at any age19-22. Recurrent CoA can occur in a many as 5 to 20 percent of surgical cases and even more frequently in cases treated with balloon angioplasty. Balloon or stent angioplasty has become accepted as a viable treatment option for recurrent CoA. Stent implantation for native or recurrent CoA has emerged as a therapeutic option for patients who can have a stent placed which can be expanded to an adult size. The perceived benefits of stent implantation include the less invasive nature when compared to surgical repair and the potential for better long term results with a reduced risk of recurrent CoA and aneurysmal formation. Until recently, there were no prospective studies evaluating the use of stents for treatment of CoA. The Coarctation Of the Aorta Stent Trial (COAST) was recently performed to determine if Cheatham Platiunum (CP) bare metal stents are safe and effective in the treatment of native and recurrent CoA selected children, adolescents and adults (National Clinical Trials ID NCT00552812). The results of this trial are pending. COAST II has now been initiated to evaluate the safety and efficacy of covered CP stents to repair or prevent aortic wall injury associated with CoA (National Clinical Trials ID NCT01278303). Balloon or stent angioplasty of native or recurrent CoA is usually performed under general anesthesia in the catheterization lab. Standard hemodynamics are obtained along with aortic angiography to define the anatomy of the CoA. Balloon or stent angioplasty is performed with the use of fluoroscopic guidance. The aortic arch is assessed by angiography following intervention (Figure 12). Patients remain in the hospital for overnight observation. Antiplatelet therapy with aspirin and SBE prophylaxis is recommended for minimum of 6

32 Vol. 64, No. 3 2013 Northeast Florida Medicine

months. Follow up occurs at intervals determined by the implanting physician. Cardiovascular restrictions to activity are determined on a case by case basis.

Conclusion Interventional cardiac catheterization has become a key tool in the treatment of CHD. As interventional techniques and devices evolve, the therapeutic options for treatment of CHD seem almost limitless. v

References 1. Allen HD. Moss and Adams heart disease in infants, children, and adolescents : including the fetus and young adult. 8th ed. Philadelphia: Wolters Kluwer Health/ Lippincott Williams & Wilkins; 2013. 2. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008;118:e714-833. 3. Feltes TF, Bacha E, Beekman RH, 3rd, et al. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation 2011;123:2607-52. 4. Mills NL, King TD. Nonoperative closure of left-to-right shunts. The Journal of thoracic and cardiovascular surgery 1976;72:371-8.

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Northeast Florida Medicine Vol. 64, No. 3 2013 33


Cardiac Disease Section

5. Suchon E, Pieculewicz M, Tracz W, Przewlocki T, Sadowski J, Podolec P. Transcatheter closure as an alternative and equivalent method to the surgical treatment of atrial septal defect in adults: comparison of early and late results. Medical science monitor : international medical journal of experimental and clinical research 2009;15:CR612-7. 6. Kaya MG, Baykan A, Dogan A, et al. Intermediate-term effects of transcatheter secundum atrial septal defect closure on cardiac remodeling in children and adults. Pediatric cardiology 2010;31:474-82. 7. Knepp MD, Rocchini AP, Lloyd TR, Aiyagari RM. Long-term follow up of secundum atrial septal defect closure with the amplatzer septal occluder. Congenital heart disease 2010;5:32-7. 8. Porstmann W, Wierny L, Warnke H, Gerstberger G, Romaniuk PA. Catheter closure of patent ductus arteriosus. 62 cases treated without thoracotomy. Radiologic clinics of North America 1971;9:203-18. 9. Therrien J, Provost Y, Merchant N, Williams W, Colman J, Webb G. Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair. The American journal of cardiology 2005;95:779-82. 10. Therrien J, Siu SC, McLaughlin PR, Liu PP, Williams WG, Webb GD. Pulmonary valve replacement in adults late after repair of tetralogy of fallot: are we operating too late? Journal of the American College of Cardiology 2000;36:1670-5. 11. Dave HH, Buechel ER, Dodge-Khatami A, et al. Early insertion of a pulmonary valve for chronic regurgitation helps restoration of ventricular dimensions. The Annals of thoracic surgery 2005;80:1615-20; discussion 20-1.

12. Eyskens B, Reybrouck T, Bogaert J, et al. Homograft insertion for pulmonary regurgitation after repair of tetralogy of fallot improves cardiorespiratory exercise performance. The American journal of cardiology 2000;85:221-5. 13. Therrien J, Siu SC, Harris L, et al. Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot. Circulation 2001;103:2489-94. 14. Oosterhof T, van Straten A, Vliegen HW, et al. Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation 2007;116:545-51. 15. Bonhoeffer P, Boudjemline Y, Saliba Z, et al. Percutaneous replacement of pulmonary valve in a right-ventricle to pulmonary-artery prosthetic conduit with valve dysfunction. Lancet 2000;356:1403-5. 16. Kenny D, Hijazi ZM, Kar S, et al. Percutaneous implantation of the Edwards SAPIEN transcatheter heart valve for conduit failure in the pulmonary position: early phase 1 results from an international multicenter clinical trial. Journal of the American College of Cardiology 2011;58:2248-56. 17. Gotzsche CO, Krag-Olsen B, Nielsen J, Sorensen KE, Kristensen BO. Prevalence of cardiovascular malformations and association with karyotypes in Turnerâ&#x20AC;&#x2122;s syndrome. Archives of disease in childhood 1994;71:433-6. 18. Jenkins NP, Ward C. Coarctation of the aorta: natural history and outcome after surgical treatment. QJM : monthly journal of the Association of Physicians 1999;92:365-71. 19. Morrow WR, Vick GW, 3rd, Nihill MR, et al. Balloon dilation of unoperated coarctation of the aorta: shortand intermediate-term results. Journal of the American College of Cardiology 1988;11:133-8.

Florida Training Academy

20. Fletcher SE, Nihill MR, Grifka RG, Oâ&#x20AC;&#x2122;Laughlin MP, Mullins CE. Balloon angioplasty of native coarctation of the aorta: midterm follow-up and prognostic factors. Journal of the American College of Cardiology 1995;25:730-4.

CNA, CPR, BLS, FA & AED

21. Patel HT, Madani A, Paris YM, Warner KG, Hijazi ZM. Balloon angioplasty of native coarctation of the aorta in infants and neonates: is it worth the hassle? Pediatric cardiology 2001;22:53-7.

Group rates and same day classes available. FLtraining.com or PerfectCNA.com for info. 34 Vol. 64, No. 3 2013 Northeast Florida Medicine

22. Fawzy ME, Fathala A, Osman A, et al. Twenty-two years of follow-up results of balloon angioplasty for discreet native coarctation of the aorta in adolescents and adults. American heart journal 2008;156:910-7.

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Cardiac Disease Section

The Staged Surgical Approach to Hypoplastic Left Heart Syndrome Michael Shillingford, MD; Eric Ceithaml, MD and Mark Bleiweis, MD

Abstract: Hypoplastic Left Heart Syndrome is a congenital heart defect

characterized by hypoplasia of left heart structures. Over the last three decades there have been considerable advances in the surgical techniques and management of these patients. We discuss shunt selection, operative strategies, transplantation and various facets of care. This is a review of the literature highlighting important aspects of the preoperative, operative, and postoperative care treatment of Hypoplastic Left Heart Syndrome (HLHS).

Introduction Hypoplastic left heart syndrome (HLHS) is a congenital heart defect in which the left heart is severely underdeveloped. Although the segmental anatomy is normal, the left heart structures are unable to support the systemic circulation. Among the larger diagnosis of congenital heart disease, the incidence ranges between four and nine percent. The first reports of left heart hypoplasia associated with aortic atresia were described by Canton in the 1850’s. In the mid 1950’s Lev and Brockman further identified cases of mitral atresia and aortic atresia in conjunction with hypoplasia of the left heart.1, 2 It wasn’t until 1958 that Noonan and Nadas formally coined the phrase “hypoplastic left heart syndrome.”1, 3 Patients with this syndrome, present with respiratory distress, tachycardia, and cyanosis. Typically, patients die within the first six weeks of life secondary to cardiovascular collapse, poor peripheral perfusion, and pulmonary edema. The use of Prostaglandin (PGE1) has revolutionized the management of infants with ductal dependent circulation. Prostaglandin allows for prolonged ductal patency which facilitates better resuscitation and time for a methodical workup. With the mixing of blood at the atrial level and an open ductus, these patients can now be managed more electively than emergently.

Please send correspondence to: University of Florida Congenital Heart Center, Gainesville, Florida Michael S. Shillingford, MD, Clinical Assistant Professor Division of Cardiothoracic Surgery, Congenital Heart Center, UF Health, 820 Prudential Drive Suite #202, Jacksonville, Florida 32207. Office 904-202-8290 Fax 904-202-8171 Email Michael.shillingford@jax.ufl.edu

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Anatomy and Diagnosis In babies with HLHS or hypoplast variant, the left sided cardiac structures are partially or completely underdeveloped before birth. The aortic and mitral valves are either too small to allow sufficient blood flow or are simply atretic altogether.4 As blood returns from the lungs to the left atrium, it must pass through an atrial septal defect to the right side of the heart, thus allowing the egress of blood out of the heart to the lungs via the main pulmonary artery, and to the body via the patent ductus arteriosus. Preoperative characteristics have major implication for postoperative outcome. For example, of the anatomic subtypes, mitral stenosis/aortic atresia may be associated with worse outcome. The ascending aorta is frequently 2mm in diameter from the sinotubular junction to the arch, and coronary perfusion is often compromised with the presence of coronary sinusoids or fistulae. A generalized thickening of the left atrium and pulmonary veins may also further complicate these cases. Often times, prominent endocardial fibroelastosis, scarring of the myocardium and ventriculocoronary communications are part of this subtype.5 It is becoming clear that they are a higher risk subsets with lower five year survival. Mitral stenosis / aortic atresia, lower birth weight, additional congenital anomalies, a genetic syndrome, or those with a highly restrictive atrial septum have worse outcomes. Even in our modern era of treatment and therapeutic interventions the outcome still remains guarded for these subsets of patients.6, 7, 8, 9 The main diagnostic modality is 2D trans-thoracic echocardiography. Prenatal ultrasounds should be highly sensitive and specific for revealing cardiac anomalies, and HLHS is diagnosed prenatally in 28 percent of affected patients.10,11 Once the child is born, post natal imaging will verify the diagnosis and help tailor the acute medical management. Patients diagnosed with HLHS prenatally have lower rate of preoperative acidosis, tricuspid regurgitation, ventricular dysfunction, and better early postoperative survival.12 There is rarely the need for more invasive studies unless the infant has a restrictive atrial septum requiring an urgent balloon septostomy in the catheterization lab for optimal left atrial decompression.8

Northeast Florida Medicine Vol. 64, No. 3 2013 35


Cardiac Disease Section Preoperative Management The importance of preoperative management cannot be overstated and the transfer to a tertiary care center with specialization in congenital heart disease is crucial.13 Resuscitative and supportive therapies include PGE1, ventilatory support, and inotropic and other vasoactive agents. The goal is to achieve a balanced pulmonary (Qp) and systemic circulation (Qs) while resuscitation continues.14 Acidosis, or an increase in PaCO2 will cause pulmonary vasoconstriction (decrease Qp), while alkalosis or increases in the FIO2 will cause pulmonary vasodilation (increase Qp). In limited circumstances intubation may be necessary. Stieh et al demonstrated that in-hospital survival decreases significantly with intubation at any time before surgery.14 Nevertheless, ventilator support and controlled hypoventilation can be important resuscitative measures in the critically ill or significantly overcirculated HLHS child. Inotropes are often used to augment cardiac output; however, this must be done with caution. These agents have a tendency to increase Systemic Vascular Resistance (SVR) which may acutely change the Qp:Qs ratio with more of the cardiac output will be shunted to the pulmonary circulation leading to congestion, reduced peripheral perfusion, and acidosis. The optimal timing of surgery is usually within the first week of life. This allows adequate time for medical management, resuscitation, and stabilization. As Pulmonary Vascular Resistance (PVR) drops shortly after birth, the balance of Qp:Qs will constantly need to be evaluated. A restrictive atrial septum will cause pulmonary venous congestion. With the severely restricted or intact atrial septum, cardiovascular collapse will ensue. Although a poor prognostic indicator for long-term survival, prompt intervention in the catheterization lab to open the atrial septum should be undertaken.8

Surgical Management and Overview Historically most patients with HLHS had a dismal outcome. Dr. Norwood described and popularized stage I palliation in the mid 1980s. Currently, the surgical procedure has been refined and the outcomes have improved; the tenets of the procedure remain unchanged. These include ascending/arch reconstruction, a non-restrictive atrial septal defect, a single RV as the systemic ventricle, and a reliable source of pulmonary blood flow via a subclavian artery (BT shunt -classic Norwood), Polytetraflouroethyline (PTFE) graft from the subclavian to the Right Pulmonary Artery (RPA) (Modified Norwood), or PTFE graft from the right ventricle to the pulmonary artery (Norwood-Sano).15,16,17 The objectives of the first-stage reconstructive procedure for HLHS involve preservation of ventricular function by excellent myocardial protection during the operation and by avoiding a residual pressure gradient after completion of the arch reconstruction. Ohye and colleagues have shown a 12 month survival advantage when RV-PA conduit was employed over modified BT 36 Vol. 64, No. 3 2013 Northeast Florida Medicine

shunt.18 In our practice, we create a Gore-Tex RV-PA conduit (with a homograft patch extension) curving in to the left chest to avoid sternal compression at the time of chest closure.19 In patients below approximately 2 kg, a 4 mm shunt is utilized; otherwise, a 5 mm graft is used. Currently, infants undergo the staged reconstructive surgery (Norwood procedure) within a week after birth, followed by the Bidirectional Glenn (superior cavopulmonary) shunt at three to six months of age, and finally an extracardiac conduit Fontan procedure at two to four years of age.

Operation with Continuous Perfusion â&#x20AC;&#x201C; Stage I Palliation These patients are very brittle and diligent communication between the anesthesia and surgical teams is crucial before and during the procedure. Even slight aberrations in ventilation, or inotropic manipulations may substantially alter systemic or pulmonary blood flow and increase early morbidity and / or mortality for this procedure. A median sternotomy is made and the heart is accessed. The head vessels are dissected to enhance arch mobilization. Some centers perform this procedure with circulatory arrest with good results. There is growing concern about the effects of hypothermic circulatory arrest on the body and brain.20,21,22,23 Moderate to deep hypothermia with selective cerebral perfusion is an accepted perfusion strategy for arch reconstruction.21,24 It is our preference to employ continuous, regional cerebral perfusion via the innominate artery during arch augmentation thereby avoiding deep hypothermic circulatory arrest (DCHA) completely.25,26 Following heparinization, cardiopulmonary bypass is initiated Arterial â&#x20AC;&#x201C; Innominate, Ductus; Venous â&#x20AC;&#x201C; Right atrial appendage. (Figure 1) Immediately after beginning bypass and cooling to 18 to 22 oC, perfusion flow is decreased as appropriate for the metabolic demands at the reduced temperature. The proximal main pulmonary artery (MPA) is divided two to three mm above the pulmonary valve commisures. The distal anastomosis of the RV to PA shunt to the pulmonary artery is now performed. For the neonate above 2.0 kg, a 5 mm stretch PTFE tube graft should be selected.27 (Figure 2, Figure 3) After completion of the MPA anastomosis, a complete atrial septectomy is performed, which allows for full systemic and pulmonary venous mixing postoperatively and prevents any chance of developing a restrictive atrial septum in the future. Once the heart myocardium is protected and quiescent, the arch and descending are completely mobilized. Proximally, the arch and ascending aorta are filleted open to the level of the division of the main pulmonary artery. The entire arch is now augmented with a generous patch of pulmonary homograft. Attempts to do the repair without some tissue (either autologous pericardium or homograft) may lead to pulmonary artery stenosis or left bronchial compression.28

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Cardiac Disease Section

Figure 1

HLHS cannulation strategy: Innominate arterial and SVC venous cannulation.

Figure 2

Figure 3

Dissected anatomy prior to Modified Norwood: Ductus ligation and division, pulmonary artery transection. Underside of the hypoplastic arch is fillet open.

Distal Sano anastomosis: 5mm gortex graft sutured to pulmonary artery in end to side fashion.

Figure 4

Pitfalls Although there are many steps of this operation, much hinges on size and patency of the anastomosis to the hypoplastic, ascending aorta. There must be very little twist or torque to the anastomosis or coronary hypoperfusion can result leading to a devastating outcome. Precise approximation of the neoaorta and the small, native ascending aorta is done with three to four simple interrupted stitches. (Figure 4)

Shunts Although some centers may perform the Classic Norwood or the Modified Norwood operation, it is our preference to perform the Norwood with Sano modification, or right ventricle to pulmonary artery (RV-PA) conduit. A general consensus is that the initial post-operative course is more stable. An important difference is that the RV to PA conduit leads to a higher diastolic pressure, and because there is no direct competition for coronary blood flow, there is less coronary steal and less coronary hypo-perfusion.19,29 The usual size of shunt selected is a five mm shunt that curves into the left chest to avoid any unwanted bending or sternal compression.19 (Figure 5, page 38) Once the Sano shunt anastomosis is completed, the patient can be weaned slowly from circulatory support.

Weaning from Bypass Ionotropes aid in separating from cardiopulmonary bypass and are important in the immediate postoperative period. Intraoperative assessment of contractility is guided by transesophageal echocardiography. Nitroprusside is used to rapidly address increases in ventricular afterload. The heart can be irritable during separation from bypass and attention to the rhythm is critical. Atrial and ventricular epicardial leads are www . DCMS online . org

A

B

(A) Proximal NeoAorta: Precise placement of sutures between the hypoplastic ascending and NeoAortic/ Pulmonary valve.

(B) Arch Reconstruction: Completion of NeoAortic arch with homograft patch.

placed and pacing at approximately 150 beats per minute can be instituted for bradycardia or junctional rhythms. The target saturations should be in the mid 70s to mid-80s.

Post-Operative Period The initial priority in the early post-operative course is to achieve hemodynamic stability. The chest is left open for planned delayed sternal closure in two to five days.30 Although there is some data that associates worse outcomes with delayed sternal closure,9 we have not seen this particular trend at our institutions. It is our feeling that management of HLHS with delayed sternal closure more readily accommodates for chest wall and abdominal swelling as well as facilitates gradual improvement in pulmonary compliance. After achieving hemodynamic stability, early aggressive diuresis is used to reduce tissue edema, which will result in improved cardiac Northeast Florida Medicine Vol. 64, No. 3 2013 37


Cardiac Disease Section

Figure 5

Proximal Sano Anastomosis: After arch reconstruction, 5mm Sano is anastomosed to the right ventriculotomy.

Figure 6

Bidirectional Glenn: The SVC is amputated from the right atrium and an end to side anastomosis is made between the SVC and pulmonary artery.

function and pulmonary compliance. We initiate and titrate furosemide infusions at 0.1 â&#x20AC;&#x201C; 0.4 mg/kg/hr to achieve an effective diuresis without the profound intravascular volume shifts often seen with bolus dosing. Once the chest wall edema dissipates, the sternum is closed in the intensive care unit, and weaning to extubation usually occurs over a one to three day period after sternal reapproximation. Inotropes, inodilators, and vasodilators are then weaned as tolerated. Although nutritional support is instituted within the first few days post-Norwood procedure, resuming oral feeding may be delayed because of numerous factors, such as: vocal cord paresis, pharyngo-esophageal or gastrointestinal dysmotility, and neurologic conditions. In our experience, feeding issues are the major factor delaying hospital discharges. Ohye and associates note that death is often associated with a prodrome of gut related problems.43 Many of these neonates are prone to GI complications such as necrotizing enterocolitis (NEC), prolonged feeding and need for long-term feeding access. All patients with HLHS in our institution get a formal speech / swallow evaluation and the feeding regimen will be tailored accordingly.13,31,32,33,34 Jefferies et al reported a need for gastrostomy tube placement in approximately 18 percent.34 Once chest closure has occurred, and swallowing has been formally evaluated, parenteral/enteral nutrition is maximized. Within a few months these patients may outgrow their RV to PA conduit or modified BT shunt and the saturations will gradually decrease (70 to 75 percent). It is important at that point in time (three to six month) that they undergo cardiac catheterization to evaluate candidacy for Stage 2 â&#x20AC;&#x201C; Bidirectional Glenn (BDG) procedure. Some institutions may proceed to BDG stage on the basis of cardiac MRI imaging in the absence of hemodynamic data.35 Catherization and MRI images provide a detailed road map of the cardiac anatomy. In particular, the arch and/or the pulmonary arteries may be kinked or narrowed as the child grows. These areas may need revision at the time of the bidirectional glen or may need preoperative angioplasty. 38 Vol. 64, No. 3 2013 Northeast Florida Medicine

Figure 7

Modified Extracardiac Fontan: Extracardiac gortex graft is used to provide flow from the SVC to the pulmonary artery.

Transthoracic or transesophageal echos also are critical in the decision-making. The aortic valve (AV) valve must be evaluated for regurgitation. This valve may need posterior annuloplasty at the time of the second stage procedure or may prove to be prohibitive for future Fontan completion, rendering the patient a transplant candidate. AV valve regurgitation is an independent risk factor for death or transplantation.36,37,38

Stage 2: Bidirectional Glenn Procedure During the second stage palliation, the bi-directional cavopulmonary connection or bidirectional Glenn shunt, the superior vena cava is separated from the heart and connected to the pulmonary circulation (Figure 6). The pulmonary artery shunt is disconnected and the lungs are no longer exposed to systemic arterial pressures, but rather to lower central venous pressure. The bidirectional Glenn procedure unloads the single ventricle and cardiac function often improves. The patient will still remain desaturated as deoxygenated blood from the lower extremities still circulates through the heart mixing with oxygenated blood in the left atrium.

Stage 3: Modified Fontan Procedure The modified Fontan operation completes the series of repairs for those diagnosed with hypoplastic left heart syndrome. This was originally described by Fontan in 1971 and has been modified to a lateral tunnel or an extracardiac conduit.39,40,41 The functional effect is to redirect venous blood from the lower body (through the inferior vena cava) away from the right atrium to the pulmonary artery. There no longer is any mixing of oxygenated and deoxygenated blood in the right ventricle. The right ventricle performs the traditional job of the left ventricle, supplying the body with oxygenated blood. The relatively low pressures in the pulmonary circulation allows for the passive flow of venous deoxygenated blood through the lungs. The extracardiac conduit procedure at times uses a Gore-Tex extracardiac tube graft (usually 16 to 20 mm in

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Cardiac Disease Section diameter) to connect the IVC to the Pulmonary Artery Circulation (PAS). (Figure 7) This limits atrial clot formation, atrial arrhythmias, and left atrial dilation more common with the lateral tunnel Fontan.42

Hybrid Approach While there have been many advances in the surgical management of HLHS. Surgical morbidity and mortality are still substantial. This has prompted alternative efforts to successfully manage this disease process. The hybrid approach is a collaborative attempt between interventional cardiologists and congenital surgeons to jointly address the reduction of pulmonary flow via surgical pulmonary artery banding and stent deployment in the patent ductus arteriosus thereby establishing reliable systemic perfusion.29, 43 This approach performed via sternotomy in a hybrid operating/interventional radiology suite allows for real time images and hemodynamics.44 This procedure potentially decreases the adverse effects of cardiopulmonary bypass in stage I palliation; however, this potential risk reduction in the first stage is shifted to stage II palliation, which now becomes more complicated. The second-stage is conducted at four to six months and consists of stent removal, aortic arch reconstruction, pulmonary artery reconstruction, and bidirectional cavopulmonary anastomosis superimposed on a redo-sternotomy.29,45,46 Centers committed to this approach achieve results comparable to the conventional Norwood approach.

Outcomes When dealing with HLHS, a successful outcome depends on a multidisciplinary approach to all phases of care. Even after all three stages, patients with HLHS will require lifelong follow up by a Pediatric Cardiologist. Currently, the operative survival ranges between 75 and 95 percent. The most current data reflects a 90 percent Stage I survival from the 1/2007 â&#x20AC;&#x201C; 1/2013. Short term survival (one-year) approaches 75 percent with medium and long-term survival (10 years) approaching 50 percent to 60 percent.3 The major losses during this time period often come between the 1st and 2nd operations or as a result of progressive heart failure. Interstage mortality can account for four percent to 16 percent resulting from RV dysfunction, arrhythmias or obstruction at the pulmonary or neoaortic arch.7,47 Many congenital programs have implemented home monitoring systems with hopes to lessen interstage mortality.7,48 Patients are discharged with oxygen saturation monitoring and a scale to monitor adequate weight gain. Parents are given clear instruction and parameters that should trigger early evaluation to avoid potential crises. Long-term complications include gradual worsening of cardiac function, protein losing enteropathy, worsening AV regurgitation, and plastic bronchitis. Many of these patients eventually require intensive heart failure management and heart transplantation.49 Unfortunately, 25 percent to 35 percent of these patients waiting for organs will expire.50, 29, 51 Those that do undergo cardiac transplantation have a five-year survival of approximately 50 to 60 percent as a group. The role of Ventricular Assist Devices (VADs) in these patients www . DCMS online . org

is in its infancy and may play a greater role as we better understand how to manage VADs in single ventricular physiology.

Conclusion Hypoplastic left heart syndrome requires a staged approach to achieve palliation to Fontan circulation. In our experience, the Norwood modification utilizing an RV to PA conduit results in stable postoperative hemodynamics and improved survival. A multidisciplinary approach is required to ensure excellence in preoperative and postoperative care. Transplantation is still required for a significant number of patients at all stages of palliation. The role of VADs for the patient with HLHS and heart failure is unknown. v

References 1. Kouchoukos NT, Kirklin JK, et al. Aortic atresia and other forms of hypoplastic left heart physiology. Cardiac Surg. 2013; Vol 2;49:1780-1812 2. Sakurai T, Brawn, WJ, et al. Single-Center experience of arch reconstruction in the setting of Norwood operation. Ann Thorac Surg 2012;94:1534-9 3. Noonan JA, Nadas AS. The hypoplastic left heart syndrome: an analysis of 101 cases. Pediatr Clin North Am 1958;5:1029 4. Emani SM, Del Nido PJ, et al. Staged left ventricular recruitment after single-ventricle palliation in patient with borderline left heart hypoplasia. J Am Coll Cardiol 2012;60:1966-74 5. Sugiyama H, Kamiya T, et al. The relation between right ventricular function and left ventricular morphology in hypoplastic left heart syndrome: Angiographic and pathological studies. Pediatr Cardiol 1999;20:422-427 6. Vlahos AP, Lock JE, McElhinney DB, van der velde ME. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: outcome after neonatal transcatheter atrial septostomy. Circulation. 2004;109:2326-2330 7. Glatz JA, Tweddell JS, et al. Impact of mitral stenosis and aortic atresia on survival in hypoplastic left heart syndrome. Ann Thorac Surg 2008;85:2057-62 8. Glatz JA, Rychik J, et al. Hypoplastic left heart syndrome with atrial level restriction in the era of prenatal diagnosis. Ann Thorac Surg 2007;84:1633-9 9. Tabbutt S, Laussen P, et al. Risk factors for hospital morbidity and mortality after the Norwood procedure: A report from the pediatric heart network single ventricle reconstruction trial. J Thorac Cardiovasc Surg 2012;144:882-95 10. Montana E, Khoury MJ, Cragan JD, Sharma S, Dhar P, Fyfe D. Trends and outcomes after prenatal diagnosis of congenital cardiac malformations by fetal echocardiography in a well-defined birth population, Atlanta, Georgia, 1990-1994. J Am Coll Cardiol 1996;28:1805-1809 11. Blake DM, Copel JA, Kleinman CS. Hypoplastic left heart syndrome: prenatal diagnosis, clinical profile, and management. Am J Obstet Gynecol. 1991;165:529-534 12. Tworetzky W, McElhinney DB, Reddy VM, Brook MM, Hanley FL, Silverman NH. Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome. Circulation. 2001;103:1269-1273 13. Berry JG, Srivastava R, et al. In-Hospital mortality for children with hypoplastic left heart syndrome after stage 1 surgical palliation: Teaching versus nonteaching hospitals. Pediatrics 2006;117;1307-1313

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Cardiac Disease Section 14. Stieh J, Kramer HH, et al. Impact of preoperative treatment strategies on the early perioperative outcome in neonates with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2006;131:1122-9 15. Poirier NC, Drumond-Webb JJ, Hisamochi K, Imamura M, Harrison AM, Mee R. Modified Norwood procedure with a high flow cardiopulmonary bypass strategy results in low mortality without late arch obstruction. J Thorac Cardiovasc Surg. 2000;120:875-884 16. Burkhart HM, Ashburn DA, Konstantinov IE, et al. Interdigitating arch reconstruction eliminates recurrent coarctation after the Norwood procedure. J Thorac Cardiovasc Surg. 2005;130:61-65 17. Sano S, Ishino K, Kawada M, Honjo O. Right ventricle pulmonary artery shunt in first stage palliation of hypoplastic left heart syndrome. Semin Cardiovasc Surg Pediatr Card Surg Annu. 2004;7:22-31 18. Ohye RG, Gaynor JW, et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 2010;362:1980-92 19. Murtuza B, Brawn WJ, et al. The effect of morphologic subtype on outcomes following the Sano-Norwood procedure. European Journal of Cardio-Thoracic Surg 2012;42:787-793 20. Burkhart HM, Ashburn DA, Konstantinov IE, et al. Interdigitating arch reconstruction eliminates recurrent coarctation after the Norwood procedure. J Thorac Cardiovasc Surg. 2005;130:61-65 21. Oppido G, Gardiulo G, et al. Moderately hypothermic cardiopulmonary bypass and low-flow antegrade selective cerebral perfusion for neonatal aortic arch surgery. Ann Thorac Surg 2006;82:2233-9 22. Visconti KJ, Pigula FA, et al. Regional low-flow perfusion versus circulatory arrest in neonates: One year neurodevelopmental outcome. Ann Thorac Surg 2006;82:2207-13 23. Gaynor JW, Clancy RR, et al. The relationship of postoperative electrographic seizures to neurodevelopmental outcome at 1 year of age after neonatal and infant cardiac surgery. J Thorac Cardiovasc Surg 2006;131:181-9 24. Stasik CN, Ohye RG, et al. Current outcomes and risk factors for the Norwood procedure. J Thorac Cardiovasc Surg 2006;131:412-7 25. Dent CL, Kurth CD, et al. Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion. J Thorac Cardiovasc Surg 2005;130:1523-30 26. Hannan RL, Burke RP, et al. Complex neonatal single ventricle palliation using antegrade cerebral perfusion. Ann Thorac Surg 2006;82:1278-85 27. Griselli M, Brawn WJ, et al. Influence of surgical strategies on outcome after the Norwood procedure. J Thorac Cardiovasc Surg 2006;131:418-26 28. Bautista-Hernandez V, Del Nido PJ, et al. Coarctectomy reduces neoaortic arch obstruction in hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2007;133:1540-6 29. Alsoufi B, Caldarone C, et al. New development in the treatment of hypoplastic left heart syndrome. Pediatrics 2007;119;109-117 30. Pasquali SK, Pearson GD, et al. Variation in perioperative care across center for infants undergoing the Norwood procedure. J Thorac Cardiovasc Surg 2012;144:915-21 31. Sarajuuri A, Lonnqvist T, et al. Neurodevelopment and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: Related risk factor analysis: J Thorac Surg. 2007;133:1524-32 32. Kelleher DK, Laussen P, Teixeira-Pinto A, Duggan C. Growth and correlates of nutritional status among infants with hypoplastic left heart syndrome (HLHS) after stage 1 Norwood procedure. 40 Vol. 64, No. 3 2013 Northeast Florida Medicine

Nutrition. 2006;22:237-244 33. Jeffries HE, Wells WJ, Starnes VA, Wetzel RC, Moromisato DY. Gastrointestinal morbidity after Norwood palliation for hypoplastic left heart syndrome. Ann Thorac Surg. 2006;81:982-987 34. Jeffries HE, Moromisato DY, et al. Gastrointestinal morbidity after Norwood palliation for Hypoplastic left heart syndrome. Ann Thorac Surg 2006;81:982-7 35. Muthurangu V, Razavi R, et al. Cardiac magnetic resonance imaging after stage 1 Norwood operation for hypoplastic left heart syndrome. Circulation 2005;112:3256-3263 36. Scheurer MA, Bradley SM, et al. Survival after bidirectional cavopulmonary anastomosis: Analysis of preoperative risk factors. J Thorac Cardiovasc Surg 2007;134:82-9 37. Ugaki S, Adatia I, et al. Tricuspid valve repair improves early right ventricular and tricuspid valve remodeling in patients with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2012; :1-5 38. Elmi M, McCrindle BW, et al. Long-term tricuspid valve function after Norwood operation. J Thorac Cardiovasc Surg 2011;142:1341-7 39. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26:240-248 40. De Leval MR, Kilner P, Gewillingm, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. J Thorac Cardiovasc Surg. 1988;96:682-695 41. Petrossian F, Reddy VM, McElhinney DB, et al. Early results of the extracardiac conduit Fontan operation. J Thorac Cardiovasc Surg. 1999;117:688-696 42. Jonas R, DiNardo J, Laussen P, Howe R, LaPierre R, Matte G., Hypoplastic left heart syndrome, Single ventricle. Comprehensive surgical management of congenital heart disease. 2004;19,20:341-385 43. Pizarro C, Radtke WA, et al. Improving the outcome of highrisk neonates with hypoplastic left heart syndrome: Hybrid procedure or conventional surgical palliation. European Journal of Cardio-thoracic Surgery 2008;33:613-618 44. Bacha E, Hijazi ZM et al. Single-ventricle palliation for high-risk neonates: The emergence of an alternative hybrid stage 1 strategy. J Thorac Cardiovasc Surg. 2006;131:163-71 45. Caldarone CA, Van Arsdell GS, et al. Initial experience with hybrid palliation for neonates with single-ventricle physiology. Ann Thorac Surg 2007;84;1294-300 46. Baba K, Honjo O, et al. Hybrid versus Norwood strategies for single-ventricle palliation. Circulation 2012;126[suppl 1]:S123-S131 47. Ghanayem NS, Goldberg CS, et al. Interstage mortality after the Norwood procedure: Results of the multicenter single ventricle reconstruction trial. J Thorac Cardiovasc Surg 2012;144:896-906 48. Ohye RG, Pearson GD, et al. Cause, timing, and location of death in the single ventricle reconstruction trial. J Thorac Cardiovasc Surg 2012;144:907-14 49. Chu MWA, Cecere R, et al. Berlin heart ventricular assist device in a child with hypoplastic left heart syndrome. Ann Thorac Surg 2007;83:1179-81 50. Chrisant MR, Naftel DC, Drummond-Webb J, et al, Pediatric Heart Transplant Study Group. Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study. J Heart Lung Transplant. 2005;24:576-582t 51. Artrip JH, Lacour-Gayet F, et al. Birth weight and complexity are significant factors for the management of hypoplastic left heart syndrome. Ann Thorac Surg. 2006;82:1252-9

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Cardiac Disease Section

Arrhythmias in Congenital Heart Disease Randall M. Bryant, MD; Jason Ho, MD and Sharon P. Redfearn, ARNP

Abstract: Arrhythmia management can be complex given preexisting

inclinations towards arrhythmias, anatomic variations and postoperative considerations. Increased postoperative survival may require a hybrid management of arrhythmias utilizing multiple treatment modalities such as medications, device therapies and surgical or transcatheter ablations. We will discuss briefly arrhythmia management in congenital heart disease, identify specific congenital heart defects that are predisposed to pre- and postoperative arrhythmias and discuss treatment strategies for these patients.

Introduction Per the Center for Disease Control, congenital heart defects are the most common birth defects in the United States. They affect about 1 percent of births or 40,000 per year. The population of adults with CHD is currently estimated at 1.3 million in the USA, 1.2 million in Europe and 96,000 in Canada.1,2,3,4 Improvements in surgical and interventional techniques over the last several decades account for an older and growing adult CHD population. The increased life expectancy is accompanied by recurrent surgery and an increased incidence of postoperative arrhythmias.5,6 Referrals for arrhythmias (mostly supraventricular) account for 31 percent of hospital admissions in adults with CHD.7 Furthermore, the leading cause of death in adults with CHD is sudden cardiac death (SCD), presumably due to arrhythmia (15 to 26 percent).8,9,10,11

abnormal findings may be from improper autonomic nervous system control.13 Atrial flutter and supraventricular tachycardia have been associated with secundum ASDs.14 In addition to the risk of bradycardia, those undergoing intervention for secundum ASDs can also be at risk for post-procedure tachyarrhythmias, including atrial flutter and fibrillation.15 In addition, atrial flutter and fibrillation have been found in association with aneurysmal tissue in the area of secundum atrial septal defects. 16

Ebstein’s Anomaly Ebstein’s anomaly describes a spectrum of congenital heart disease wherein the tricuspid valve is apically displaced to a varying degree. This results in individuals who have no sequelae or hemodynamic derangements to those who essentially have a hypoplastic right ventricle (Figure 1- Ebstein’s CXR). The very process of malformation during tricuspid valve development explains the high incidence of Wolff-Parkinson White syndrome (WPW) in those with Ebstein’s anomaly. Premature cessation

Atrial Septal Defects (ASD) The literature reports that individuals with closed or repaired ASD are at risk for developing sinus node dysfunction or atrioventricular nodal block.12 A secundum ASD is not clinically affected by bradycardia. However, electrophysiology studies performed on individuals with secundum ASDs in the past have shown evidence of delayed conduction times through the sinoatrial, atrio-Hisian, and atrioventricular pathways. One study suggests that these

Please address correspondence to: Randall Bryant, MD 841 Prudential Drive, #100, Jacksonville, FL 32207

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Figure 1

Pathognomonic chest x-ray of infant with Ebstein’s anomaly.

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Cardiac Disease Section Figure 2

Electrocardiographic tracing demonstrated conducted normal sinus rhythm, AV block and WPW in a patient with corrected transposition of the great arteries

of tricuspid valve formation may result in residual specialized tissue that crosses the atrioventricular groove and results in anomalous conduction. These accessory pathways (AP) may conduct in a bidirectional, antegrade-only, or retrograde-only manner. In addition, individuals with WPW are susceptible to inducible arrhythmias including typical supraventricular tachycardia mechanisms such as orthodromic reciprocating tachycardia (AV node › AP › AV node), antidromic reciprocating tachycardia (AP › AV node › AP), and atrioventricular nodal re-entrant tachycardia.17

Coronary artery malformations While the heart supplies the body with oxygen via a complex network of vessels, the heart itself receives oxygen during diastole via coronary arteries that normally arise from specific aortic root sinuses. Multiple theories exist on the exact order of coronary artery development—whether endothelial tissue grows from or towards the aortic wall—but abnormalities in the development can lead to various types of coronary artery anomalies (CAA), including single coronary systems, ectopic coronary origins, or abnormal vessel coursing. Sinus node dysfunction and complete heart block may arise if CAAs result in compromise of the arterial supply to either nodal structure. In addition, post-operative complete heart block is a rare result from CAA repair.18 Systolic compression at the origin of an anomalous coronary artery has been documented as one mechanism in which this phenomenon may occur.19 CAAs can be associated with slit-like orifices and acute angle take-offs that may compromise diastolic flow. Coronary artery anomalies can present in a devastating manner, as the initial symptom may be sudden death. Often patients describe prior episodes of syncope. CAAs can lead to progressive hypotension, bradycardia, persistent ischemia, and eventually the development of ventricular fibrillation or pulseless electrical activity.20 Also, ventricular fibrillation has been listed as a complication of CAA repair. Congenital coronary anomalies are rare with an estimated prevalence of approximately 1 percent in the general population. However, among patients with symptomatic congenital

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heart disease, coronary anomalies are much more common with a prevalence of five to 10 percent. While the majority of these anomalies are hemodynamically insignificant posing little risk of sudden death, they may have surgical implications. For instance, five percent of tetralogy of Fallot (TOF) patients have a CAA. Approximately two-thirds of these anomalies cross the RVOT, most commonly the left anterior descending (LAD) or accessory LAD as the most common anomalous artery to cross the right ventricular outflow tract (RVOT).21 The congenital heart surgeon has to be aware of this and may need to modify the surgical approach to avoid coronary artery injury.

Ventricular septal defects There are four types of septal defects based on their location in the interventricular septum: infundibular, membranous, inlet or muscular. Pre-operatively there are no significant dysrhythmias associated with VSDs. There are scant case reports on progressive conduction block with spontaneously closing VSDs. Postoperatively the most common arrhythmia is complete heart block. The risk of post-operative permanent CHB following VSD closure has decreased since Lev and others delineated the course of the conduction tissue in various types of congenital cardiac malformations of the ventricular and adjacent atrial septa.22 Although in earlier reports the risk for heart block was as high as 25 percent, most recent reports have reduced this risk to approximately one to four percent. If heart block persists for seven to 10 postoperatively, pacemaker implantation is indicated. There are some patients who develop transient heart block in the postoperative period who do not require pacemaker implantation based upon ACC/ AHA guidelines.23 A small subset of those patients who have recovered AV node function may have late recurrence of AV block and present with severe bradycardia or sudden death. Congenitally corrected transposition of the great arteries (ccTGA), CCTGA, also known as L-transposition of the great arteries with ventricular inversion, is characterized by ventricular inversion and arterial transposition such that the cyanotic blood that returns from the vena cavae to the right atrium is www . DCMS online . org


Cardiac Disease Section directed to the left ventricle and pulmonary artery. Sequentially the oxygenated pulmonary venous return enters the left atrium and is directed to the right ventricle and the aorta. Complete heart block can also occur spontaneously, particularly in patients with ccTGA. This is probably due to displacement and depressed conduction through the AV node. Dual AV nodes have been frequently reported in many of these patients. The incidence of spontaneous complete AV block in patients with ccTGA has been reported to range between 17 and 22 percent during long-term follow-up and the overall risk of spontaneous heart block is 2 percent per year.24,25 Isolated ccTGA without associated congenital defects is rare. One of the most common associated congenital defects is Ebstein’s malformation of the tricuspid valve. This is associated with Wolff-Parkinson-White syndrome (WPW) and supraventricular tachycardias (SVTs) due to multiple accessory pathways (Figure 2). Also, a nodal-nodal SVT has been described via the dual AV nodes. This can be amenable to ablation of the subsidiary AV node with a relatively low risk of AV node block. The systemic right ventricle is prone towards failure in the fourth decade of life due to the demands of the systemic arterial circulation. The failing RV may not be as responsive to beta-blockers or afterload reduction as left ventricles26,27 and over time may become arrhythmogenic resulting in life threatening arrhythmias. This may require an ICD for recalcitrant arrhythmias as a bridge towards transplantation.

A

B

Figure 3

MRI demonstrating the atrial switch procedure with baffling of the pulmonary veins to the systemic right ventricle.

Figure 4

Sinus bradycardia (35 bpm) in a 24 year-old elementary school teacher with d-TGA who has undergone a Senning procedure.

A

B

C

D-Transposition of the great arteries (d-TGA) The developing heart is essentially a straight tube that curves to the right as part of normal embryologic development. When associated with transposition of the great arteries, this “d-looping” gives the characteristic aortic attachment to the right ventricle and pulmonary attachment to the left ventricle as seen with d-TGA. These infants are typically very cyanotic and require palliation early in life, e.g. a balloon atrial septostomy (Rashkind, 1966). Surgical “correction” with an atrial switch procedure has been possible since the Senning procedure in 1959, a technique that was later refined in the Mustard procedure in 1964 (Figure 3). The arterial switch was introduced by Jatene in 1976 and gained widespread use in the 1980s.

Baffle obstruction (A), baffle stenting (B) and pacemaker leads inserted through baffle stent (C) in a 19 year-old patient with d-transposition of the great arteries who has undergone a Mustard (atrial switch) procedure.

Sinus node function is abnormal in 86 percent of d-TGA patients who have undergone an atrial switch procedure (Senning or Mustard) due to direct injury to the sinus node or sinus node artery.28 (Figure 4) Because these patients have systemic right ventricles and chronic dysfunction, they require a presystolic atrial kick for optimal hemodynamics. Pacemaker implantation can be complicated by atrial baffle obstruction that may require stenting at the time of pacemaker implantation (Figure 5). Because there is a low incidence of an AV node conduction disorder, often atrial pacing is sufficient. However, if the initial surgery also required VSD closure, a postoperative

AV node conduction disorder may necessitate a dual chamber device. Atrial tachyarrhythmias, in the form of incisional atrial flutter, are often associated with an atrial switch procedure. As such, if a pacemaker is required, we implant atrial antitachycardia pacemakers in all patients with atrial switch procedures. These atrial tachycardias are often amenable to transcatheter ablation via a trans-baffle puncture to place a radiofrequency ablation line from the tricuspid valve to the inferior vena cava. Oftentimes atrial reentrant arrhythmias can be mapped to the atrial incision, ASD patch (used to repair the atrial septostomy)

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Figure 5

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Cardiac Disease Section or cannulation sites, and are also amenable to transcatheter ablation with three-dimensional electroanatomic mapping. The systemic right ventricle demonstrates signs of strain on the electrocardiogram (Figure 6). As the systemic RV fails, its arrhythmogenicity increases. Nonsustained ventricular tachycardia is a harbinger of a more malignant arrhythmia and should be addressed promptly. Because beta-blockers have not been shown to statistically increase survival in this group of patients, oftentimes patients are placed on potassium-channel blockers (e.g. sotalol, dofetilide or amiodarone) with a low threshold for ICD implantation.

Tetralogy of Fallot (TOF) The tetralogy of Fallot is the most common cyanotic heart defect and consists of a malalignment VSD, overriding aorta, subpulmonary stenosis and right ventricular hypertrophy. Preoperatively there may be syncope, but it is more likely due to hypoxia during a “tet spell” and not arrhythmic in origin. Postoperatively, arrhythmias originate from atrial/ventricular scars and chronic RV volume/pressure overload due to residual subpulmonary stenosis or chronic pulmonary regurgitation. The atrial arrhythmias consist of intra-atrial reentry tachycardia (IART) or incisional atrial flutter due to reentry around the lateral right atrial incision that the surgeon makes at the time of VSD repair. If an atrial septal defect is present (pentalogy of Fallot), following closure there can be reentry around the surgical patch. Scar-related atrial arrhythmias may be difficult to treat with antiarrhythmics but are amenable to curative ablation therapy. Ventricular arrhythmias may originate from reentry around the surgical transannular patch or, less commonly, reentry around the VSD patch. Chronic pulmonary insufficiency in addition to the postoperative hemodynamics and surgical scars place these patients at highest risk for malignant arrhythmias. Although most patients have a right bundle branch block on their postoperative ECG, it is the electroanatomic effects of ventricular dilation that lead to prolonged RV activation that in turn results in QRS prolongation. A QRS duration of 140 to 160 msec has been identified as an independent predictor of ventricular arrhythmias (Figures 7 and 8). Other noninvasive tests, e.g. signal averaged ECG or T-wave alternans studies, have proven less predictive. Transcatheter ablation of ventricular arrhythmias in TOF patients, although possible, is more difficult than ablation of atrial arrhythmias and may change the arrhythmia substrate in a negative way resulting in unstable ventricular arrhythmias. With symptomatic documented arrhythmias, the hemodynamic issues (e.g. chronic pulmonary regurgitation) should be addressed as well as the arrhythmia.

Single Ventricles The final surgical palliation of the single ventricle is the Fontan procedure during which the systemic (cyanotic) 44 Vol. 64, No. 3 2013 Northeast Florida Medicine

Figure 6

Classic electrocardiogram from a 34 year-old homemaker with d-TGA and sick sinus syndrome who has undergone an atrial switch (Mustard or Senning) procedure and atrial pacemaker. There is evidence for increased voltages in lead V1 with ST-T wave changes consistent with RVH with strain (V1). Also, there are diminished left side forces in lead V6.

venous return is baffled directly to the pulmonary arteries and the pulmonary (oxygenated) venous blood returns to the left or common atrium. There are several “modifications” of the Fontan procedure, each of which has unique arrhythmia substrates. See the article “The staged surgical approach to hypoplastic left heart syndrome” for a more complete discussion. Sinus node dysfunction has been previously reported to occur in nine to 60 percent of patients after the Fontan operation.19 The presystolic atrial kick is essential in these patients for optimal hemodynamics. Implantation of a pacemaker is complicated by chronically scarred and dilated atria in the case of the classic Fontan and by lack of transvenous access in the external conduit Fontan (Figure 9). Epicardial atrial lead implantation is hindered by high atrial capture thresholds with a lead failure rate of 17 percent.29 Intra-atrial reentrant tachycardias (incisional atrial flutters) occur in approximately 50 percent of patients within 10 to15 years of the Fontan procedure.30 IART rates are typically slower (120-200 bpm) as compared to typical atrial flutter (300 bpm) because of the scar and fibrosis that is the substrate of the flutter circuit. Ventricular response rates as low as 120 bpm may be intolerable due to multiple factors, e.g. ventricular dysfunction, elevated end-diastolic pressures, AV valve regurgitation, all of which are dependent on atrial systole for the best hemodynamics. Treatment of this arrhythmia is difficult. Transvenous access may be difficult due to femoral venous obstruction as a result of multiple neonatal catheterizations or central venous lines. Even in the presence of transvenous atrial access, the risk of recurrence following successful ablation may be as high as 40-50 percent, as there may be multiple flutter circuits and chronic scarring (Figure 10). Often effective antiarrhythmic therapy requires a hybrid approach including preventive atrial pacing, antitachycardia atrial pacing and antiarrhythmic therapy along with ablation therapy (Figure 11, page 46). Fontan “revisions” with surgical Maze procedures have a lower IART recurrence rate, but they www . DCMS online . org


Cardiac Disease Section

Figure 7

Right bundle branch block in a 26 year-old medical assistant with tetralogy of Fallot patient with a residual VSD as well as chronic pulmonary regurgitation. The QRS duration of 220 msec correlates with the patient’s severely dilated RV and is a predictor of ventricular arrhythmias.

Figure 9

MRI of a 28 year-old cable TV technician with tricuspid atresia who had undergone a classic Fontan procedure with right atrial appendage anastomosis directly into the pulmonary arteries. Note the severely dilated right atrium that is larger than the LV and the dilated hepatic veins from chronic congestion.

Figure 8

: The same patient (Figure 7) presented with syncope and a wide QRS tachycardia resulting in multiple (16) ICD shocks. Attempts at ablation had limited success due to extensive hypertrophy and surgical scarring. The patient has been well controlled on sotalol with addition of a beta-blocker.

still range in the 30 to 40 percentiles in experienced hands. Rarely AV node ablation with ventricular pacing is required. The latest modification of the Fontan procedure, the external conduit, promises to eliminate many of the surgical suture lines that are felt to be primary substrates for IART. However, this “modification” will also eliminate transfemoral access to the atria for transcatheter ablation to treat any residual IART circuits. Sudden deaths have been reported in nine to 17 percent of late Fontan deaths, many of these due to thromboembolism and heart failure. In the older Fontan patient, atrial arrhyth-

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Figure 10

Four distinct atrial tachycardias with distinctly different p-waves in the same patient (Figure 9). Three circuits were ablated successfully. The other was amenable to antitachycardia pacing.

mias with rapid ventricular responses may in turn produce life threatening ventricular arrhythmias due to ischemia (Figure 12, page 46). As more Sano procedures are performed for hypoplastic left heart syndrome, there is a growing concern that the ventricular incision made during this procedure will be a substrate for ventricular dysrhythmias in the future. Treatment of ventricular arrhythmias may necessitate the use of complex antiarrhythmics and novel epicardial ICD systems.

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Cardiac Disease Section

Figure 11

Anti-tachycardia pacing to convert Figure 9 patient out of incisional atrial flutter.

Figure 12

A 31-year-old baker with doubleinlet left ventricle who has undergone a classic Fontan procedure. Incisional atrial flutter with a rapid ventricular response is not tolerated hemodynamically. This patient refused a Fontan revision with surgical Maze procedure and subsequently died suddenly while playing Frisbee.

Interventional Electrophysiology in Adult CHD patients From 2007 to 2012 we performed 121 electrophysiology procedures in 110 patients in the Electrophysiology Lab at Wolfson Children’s Hospital where we house state-of-the-art EP technology, including three-dimensional mapping, cryoablation and radiofrequency ablation systems. In this group of patients, the average and median patient ages were 27 years and 26 years, respectively. The most common primary congenital heart defect was d-transposition of the great arteries, followed by tetralogy of Fallot and single ventricle patients who have undergone a Fontan modification (most commonly, double-inlet LV and tricuspid atresia) (Chart 1). The most common primary arrhythmia diagnosis was incisional atrial flutter, followed by ventricular tachycardia and AV block. The average number of arrhythmias at the time of the procedure was 1.7 with sinus node dysfunction being the most common secondary arrhythmia diagnosis. Transcatheter ablation was the most common procedure performed followed by pacemaker implantation and then ICD implantation (Chart 2).

Summary As the congenital heart patient grows from a child to an adult, the incidence of arrhythmias may increase due to extension of surgical scars, additional surgical procedures and chronic ventricular and atrial pressure and volume overload, compounded by existent risk factors for acquired heart disease. 46 Vol. 64, No. 3 2013 Northeast Florida Medicine

Figure 13

Chest x-ray of 44 year-old vice principal with corrected transposition of the great arteries, VSD, Ebstein’s anomaly of the tricuspid valve and pulmonary stenosis. She developed postoperative heart block after VSD closure at 7 years of age. She has undergone multiple pacemaker and lead revisions. Her CXR demonstrates residual epicardial and transvenous leads with fractures. At her request an epicardial pacemaker has been abandoned underneath her left breast. She has undergone several ablation procedures for incisional atrial flutter and SVT associated with her Ebstein’s anomaly. She has developed systemic RV failure with ventricular tachycardia and recently was upgraded to an ICD.

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Cardiac Disease Section Chart 1

Congenital heart diagnoses in patients >18 years old who have undergone interventional EP procedures at Wolfson Children’s Hospital.

D-TGA TOF Single V AV/MV L-TGA Other AVC HCM VSD Ebstein’s Complex Truncus TAPVR ASD

Chart 2

Procedures performed in patients >18 years old with congenital heart disease who have undergone interventional EP procedures at Wolfson Children’s Hospital.

Ablation

Pacemaker

ICD

Cardioversion

Other

ILR

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Cardiac Disease Section This increased arrhythmia burden will necessitate a hybrid approach to therapy including antiarrhythmics, surgical/ transcatheter ablation and device implantation (Figure 13, page 46). In our experience a successful Congenital Heart Electrophysiology program is needed to address these complex arrhythmias in these complex patients. This requires a dedicated team of subspecialists trained in the care of these patients, including electrophysiologists, interventionalists, heart surgeons and anesthesiologists, in addition to a welltrained EP lab staff and generous support from the hospital administration. With this level of expertise and support, good outcomes are achievable in this growing patient population. v

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13. Finley JP, Nugent ST, Hellenbrand W, et al. Sinus arrhythmia in children with atrial septal defect: An analysis of heart rate variability before and after surgical repair. Br Heart J 1989; 61:280-284. 14. Garson A Jr, Bink-Boelkens M, Hesslein PS, et al. Atrial flutter in the young: A collaborative study of 380 cases. J Am Coll Cardiol 1985;6:871-878. 15. John B, George OK, Joseph G, Lokhandwala YY. Incessant atrial flutter after device closure of atrial septal defect: successful radiofrequency ablation. Indian Pediatr 2007;44:700-702. 16. Janion M, Kurzawski J. Atrial fibrillation in patients with atrial septal aneurysm. Cardiol J 2007;14:580-584. 17. Saul JP. “Electrophysiologic Therapeutic Catheterization.” Moss and Adams’ Heart Disease in infants, fetus, and young adult. Ed. Hugh Allen et al.. Philaldelphia: Lippincott, Williams, and Wilkins, 2008. 419-420. 18. Ruel RM, Cooley DA, Hallman GL, Ruel GJ. Surgical treatment of coronary artery anomalies. Tex Heart Inst J 2002;29:299-307.

2. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J. Am. Coll. Cardiol. 2002;39(12), 1890-1900.

19. Benge W, Martins JB, Funk DC. Morbidity associated with anomalous origin of the right coronary artery from the left sinus of Valsalva. Am Heart J 1980;99:96-100.

3. Hoffman JI, Kaplan S, Liberthson RR. Prevalence of congenital heart disease. Am. Heart J. 2004;147(3), 425-439.

20. Angelini P. Anomalous origin of the left coronary artery from the opposite sinus of valsalva. Tex Heart Inst J 2009;36:313-315.

4. Engelfriet P, Boersma E, Oechslin E et al. The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro Heart Survey on adult congenital heart disease. Eur. Heart J. 2005;26(21), 2325-2333.

21. Lowry AW, Olaybiyi OO, Adachi I, Moodie DS, Knudson JD. Coronary artery anatomy in Congenital Heart Disease. Congenit Heart Dis. 2013;8:187–202.

5. Verheugt CL, Uiterwaal CS, Grobbee DE, Mulder BJ. Longterm prognosis of congenital heart defects: a systematic review. Int. J. Cardiol. 2008;131(1), 25-32. 6. Moons P, Engelfriet P, Kaemmerer H et al. Delivery of care for adult patients with congenital heart disease in Europe: results from the Euro Heart Survey. Eur. Heart J. 2006;27(11), 1324-1330. 7. Verheugt CL, Uiterwaal CS, van der Velde ET et al. The emerging burden of hospital admissions of adults with congenital heart disease. Heart 2010; 96(11), 872-878. 8. Nieminen HP, Jokinen EV, Sairanen HI. Causes of late deaths after pediatric cardiac surgery: a population-based study. J. Am. Coll. Cardiol. 2007; 50(13), 1263-1271. 9. Oechslin EN, Harrison DA, Connelly MS, Webb GD, Siu SC. Mode of death in adults with congenital heart disease. Am. J. Cardiol. 2000; 86(10), 1111-1116. 10. Silka MJ, Hardy BG, Menashe VD, Morris CD. A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects. J. Am. Coll. Cardiol. 1998; 32(1), 245-251. 11. Verheugt CL, Uiterwaal CS, van der Velde ET et al. Mortality in adult congenital heart disease. Eur. Heart J. 2010; 31(10), 1220-1229. 12. Chen Q, Cao H, Zhang GC, Chen LW, Chen DZ, Li QZ, Qui ZH. Atrioventricular block subsequent to intraoperative device closure atrial septal defect with transthoracic minimal invasion; a rare and serious complication. PLoS One. 2012; 7:e52726.

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22. Lev M. The architecture of the conduction system in congenital heart disease III: ventricular septal defect. Arch Pathol. 1960;70:530–549 23. Gregoratos, et al. ACC/AHA Guidelines for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices: Executive Summary. Circulation.1998; 97: 1325-1335 24. Connelly MS, Liu PP, Williams WG et al. Congenitally corrected transposition of the great arteries in the adult: functional status and complications. J. Am. Coll. Cardiol. 1996; 27(5), 1238-1243. 25. Lundstrom U, Bull C, Wyse RK, Somerville J. The natural and unnatural history of congenitally corrected transposition. Am. J. Cardiol. 1990;65(18), 1222-1229. 26. Dore A, Houde C, Chan KL, et al. Angiotensin receptor blockade and exercise capacity in adults with systemic right ventricles: a multicenter, randomized, placebo-controlled clinical trial. Circulation. 2005; 112(16):2411-2416. 27. Shaddy, RE et al. Carvedilol for Children and Adolescents with Heart Failure-A Randomized Controlled Trial. JAMA, September 12, 2007—Vol 298, No. 10 1171. 28. Vetter VL, Tanner CS, Horowitz LN. Electrophysiologic consequences of the Mustard repair of d-transposition of the great arteries. J Am Coll Cardiol. 1987 Dec;10(6):1265-73. 29. Cohen MI, Vetter VL, Wernovsky G, et al. Epicardial pacemaker implantation and follow-up in patients with a single ventricle after the Fontan operation. J Thorac Cardiovasc Surg. 2001;121:804–811. 30. Collins KK. The Spectrum of Long-term Electrophysiologic Abnormalities in Patients with Univentricular Hearts. Congenit Heart Dis. 2009;4:310–317

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Cardiac Disease Section

Heart Failure Management in End-Stage Congenital Heart Disease Gonzalo Wallis, MD

Stages of Heart Failure

Abstract: Heart failure is a complex clinical syndrome that results

from any structural or functional impairment of ventricular filling or ejection of blood, with clinical manifestations of dyspnea and fatigue. The process can occur over a prolonged period of time with symptoms occurring quite late in the disease process, with non-specific symptoms initially and hemodynamic decompensating as a late finding. Infants, children and young adults with congenital heart disease represent a significant percentage of the population who are at risk of developing heart failure. Our intention is to discuss briefly the management of end-stage cardiac failure in patients with congenital heart disease.

Introduction Heart failure is a complex clinical syndrome that results from any structural or functional impairment of ventricular filling or ejection of blood, with clinical manifestations of dyspnea and fatigue. This may limit exercise tolerance, and create fluid retention, causing pulmonary and/or splanchnic congestion and/or peripheral edema.6 Systolic heart failure is a latent condition and can take an extended period of time to establish itself, sometimes over the course of years, which is why not all patients present with the same symptomatology. Some have exercise intolerance but little evidence of fluid retention and others complain primarily of edema, dyspnea or fatigue. Also it is known that the systolic ejection fraction in this prolonged time course can steadily deteriorate, and lead ultimately to symptoms occurring quite late in the disease process. There is no one good test to diagnose heart failure, but the clinical diagnosis is largely based on careful history, physical examination, hemodynamic data and neurohumoral evaluation. The lifetime risk of developing heart failure for Americans has remained stable for the past several decades.1 It is estimated that approximately 20 percent for Americans older than 40 years of age2 are newly diagnosed, representing approximately 650,000 new cases diagnosed per year.3,4 Infants, children and young adults with congenital heart disease represent a significant percentage of the population who are at risk of developing heart failure due to their anatomical substrate and increased survival due to successful surgical palliations. These patients will live longer, but may develop heart failure requiring further medical management or surgical support as a bridge to transplantation. Please address correspondence to: Gonzalo Wallis, MD, 1600 SW Archer Road, HD-303, Gainesville, Florida 32610 or Email: gwallis@peds.ufl.edu www . DCMS online . org

The scale that is used to classify symptomatic heart failure in the pediatric population is the Ross classification (Table 1, page 50). In the adult patients the American College of Cardiology (ACC)/American Heart Association (AHA) and the New York Heart Association (NYHA) (Table 2, page 51) is commonly used. Both the NYHA and Ross scales concentrate only on current symptomatology, while the ACC/AHA classification identifies patients who are at risk of developing heart failure and require early intervention to prolong the asymptomatic state. The International Society of Heart and Lung transplantation has published guidelines in the management of heart failure in children as well as advocate for the use of an adapted ACCF/AHA classification that is modified to children (Table 3, page 51).5

Medical Management of Heart Failure Compensated heart failure

The medical management of heart failure over the past two decades has consisted of diuretics, afterload-reducing agents, angiotensin-converting enzyme inhibitors, potassium-sparing agents and Ă&#x;-blockers. The first key to the management of a failing heart is to identify the status of the volume load in the heart and the contractility of the right and left ventricle. Management requires a basic understanding of the contributors to cardiac output, preload, afterload, contractility and heart rate.

Diuretics

Diuretics are the preferred agent to use in the patient with heart failure. Diuretics inhibit the reabsorption of sodium or chloride in the renal tubules. Loop diuretics like bumetanide, furosemide and torsemide act at the ascending limb of the loop of Henle, on the Na+/K+/2Cl- transporter, decreasing the sodium reabsorption. The thiazides diuretics, such as metolazone or hydrochlorothiazide, act by inhibiting the Na+/ Cl- co-transporter in the distal tubule, and potassium-sparing agents (spironolactone) act in the distal tubule. Diuretics decrease symptoms of systemic and pulmonary venous congestion6,7 and improve exercise tolerance. The current recommendations are to use diuretics in all patients with heart failure and fluid retention in order to achieve a euvolemic state. Spironolactone is a potassium sparing diuretic that has

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Cardiac Disease Section Table 1 Pediatric classification of heart failure (Ross Classification) Ross RD, Daniels SR, Schwartz DC, Hannon DW, Shukla R, Kaplan S. Plasma norepinephrine levels in infants and children with congestive heart failure. Am J Cardiol 1987;59:911– 4.

CLASS

INTERPRETATION

I Asymptomatic
 II

Mild tachypnea or diaphoresis with feeding in infants. Dyspnea on exertion in older children.

III

Marked tachypnea or diaphoresis with feeding in infants. Prolonged feeding times with growth failure due to heart failure. In older children, marked dyspnea on exertion

IV

Symptoms such as tachypnea, retractions, grunting, or diaphoresis at rest

proven specifically to improve survival in adults with advanced heart failure8 by blockade of aldosterone and not the diuretic effect. The activation of the renin-aldosterone-angiotensin system (RAAS) is thought to be critical in the pathogenesis of heart failure, and interruption of the RAAS is a foundation of modern heart failure therapy.9 In the pediatric population, there’s no published data to support the use of diuretics in reducing mortality or morbidity recommendations can only be extrapolated from the adult data.

Angiotensin Converting Enzyme Inhibitors (ACEI) and Angiotensin Receptor Blockers (ARB)

ACEI and ARB in heart failure is related to the inhibition of the RAAS,10 which contributes to the progression of heart failure over time, by increasing adrenergic tone, which increases afterload and myocardial oxygen demand, causing myocardial hypertrophy and fibrosis.11,12 Multiple large clinical trials have demonstrated that treatment with ACEIs improves symptoms and survival in adults and reduces disease progression in asymptomatic patients.13,14,15 There is limited data regarding the use of ACEIs in patients with structural heart disease, but there are benefits regarding the use of these medications related to LV volume, dimensions, mass index, wall stress and reduction in the volume-loaded ventricle in adult patients with aortic regurgitation.16 The ARBs are competitive antagonists for the angiotensin II receptor in the cell surface,17 blocking angiotensin rather than acting on the angiotensin-converting enzyme. This has benefits, like not inhibiting the bradykinin breakdown, which has been implicated the persistent cough side effect of ACEIs. Despite their mechanism of action, trials in adult patients with HF have not shown any important differences in hemodynamic effects, efficacy and safety between ARBs and ACEIs.18,19 Currently ARBs are recommended in adults intolerant to ACEIs. In the pediatric populations small observational studies have demonstrated that ACEIs benefit patients with heart failure caused by systemic ventricular systolic dysfunction,20,21,22,23 but with no proven effects on mortality. 50 Vol. 64, No. 3 2013 Northeast Florida Medicine

Beta-blockers

Chronic activation of the sympathetic nervous system in the failing heart contribute to the progression of HF by chronic systemic peripheral vasoconstriction, and salt/water retention by the kidneys.24,25 Beta-blockers are used to antagonize the deleterious effects. The risks related to the use of beta-adrenergic blockade include hypotension and deterioration in myocardial performance,26,27 usually occurring in the first 24 to 48 hours after initiation of therapy, or at the time of up-titration of the drug. Metoprolol and carvedilol have been extensively evaluated in the adult population with randomized placebo control trials in patients with ischemic and non-ischemic cardiomyopathy and mild to moderate CHF, demonstrating a reduced mortality by 25 to 34 percent,28 and a decrease the risk of clinical progression of heart failure in adults.29,30,31 The evidence has been inconclusive in children.32

Digoxin

Digoxin has not been shown to improve heart failure survival, but has been shown to improve symptoms and re-hospitalizations in adults with heart failure, even at low levels.33,34 Even though digoxin is widely used to treat heart failure in infants and children, other than what’s extrapolated from the adult data there is very little pediatric data available to support its use. Digoxin does have a long track record of clinical efficacy and safety in the pediatric population that is reported by experienced clinicians.

Special considerations The Systemic Right Ventricle

The morphologic right ventricle will be the systemic ventricle in some patients with complex congenital heart disease. In patients with d-transposition of the great arteries who were treated with atrial baffle procedures (Mustard or Senning repair), patients born with congenitally-corrected transposition of the great arteries (l-Transposition of the great arteries or ventricular inversion) or hypoplastic left ventricle (mitral atresia or aortic atresia) all have their systemic ventricle as a www . DCMS online . org


Cardiac Disease Section Table 2 Adult classification of heart failure Clyde W Yancy, M. M. F. F. (2013). 2013 ACCF/AHA Guideline for the Management of Heart Failure. JAC, 1â&#x20AC;&#x201C;377. doi:10.1016/j.jacc.2013.05.019

ACCF/AHA Stages of HF

NYHA Functional Classification

A

At high risk for HF but without structural heart disease or symptoms of HF

B

Structural heart disease but without signs or symptoms of HF

I

No limitation of physical activity. Ordinary physical activity does not cause symptoms of HF.

C

Structural heart disease with prior or current symptoms of HF

I

No limitation of physical activity. Ordinary physical activity does not cause symptoms of HF.

II

Slight limitation of physical activity. Comfortable at rest, but ordinary physical activity results in symptoms of HF.

III

Marked limitation of physical activity. Comfortable at rest, but less than ordinary activity causes symptoms of HF.

IV

Unable to carry on any physical activity without symptoms of HF, or symptoms of HF at rest.

D

None

Refractory HF requiring specialized interventions

*ACCF indicates American College of Cardiology Foundation; AHA, American Heart Association; HF, heart failure; and NYHA, New York Heart Association

Table 3 ISHLT proposed Heart Failure staging for infants and children.5 STAGE

INTERPRETATION

I

Patients with increased risk of developing HF, but who have normal cardiac function and no evidence of cardiac chamber volume overload. Examples: previous exposure to cardiotoxic agents, family history of heritable cardiomyopathy, univentricular heart, congenitally corrected transposition of the great arteries. â&#x20AC;¨

II

Patients with abnormal cardiac morphology or cardiac function, with no symptoms of HF, past or present. Examples: aortic insufficiency with LV enlargement, history of anthracycline with decreased LV systolic function.

III

Patients with underlying structural or functional heart disease, and past or current symptoms of HF.

IV

Patients with end-stage HF requiring continuous infusion of inotropic agents, mechanical circulatory support, cardiac transplantation or hospice care.

*HF, heart failure; LV, left ventricular.

www . DCMS online . org

Northeast Florida Medicine Vol. 64, No. 3 2013 51


Cardiac Disease Section morphologic right ventricle. Usually the right ventricle (RV) has normal systolic function for several years and for poorly understood reasons the ventricle will fail in the adolescent or young adult. It has been hypothesized that the failure is related to a sub-optimal myocardial fiber arrangement and mechanics in the right ventricle, adverse pattern and reduced heterogeneity of ventricular strain, tricuspid insufficiency and/or myocardial fibrosis secondary to prolonged hypoxemia during infancy while waiting for surgery.35 Heart failure with fluid retention associated with systemic right ventricular dysfunction should be treated with diuretics to achieve a euvolemic state. Asymptomatic systemic right ventricular dysfunction should be treated with ACE inhibitors should be routinely employed unless there is a specific contraindication. These medications should be employed at standard doses. Digoxin is not currently recommended for patients with asymptomatic systemic right ventricular dysfunction, but is recommended for patients with symptomatic systemic RV dysfunction.6 It is important to note that single ventricle patients do not develop the typical symptoms that occur in patients with two ventricles. Symptoms of fatigue, peripheral edema, and pleural and pericardial effusions dominate the clinical picture.6

The Single Ventricle

The survival of patients with systemic left ventricles versus those with systemic right ventricles is better in the early postoperative period, but within six months after completion of the Fontan procedure, ventricular morphology does not influence survival.6,42 The thought behind an early failure of the systemic right ventricle is that left ventricular myocytes are arranged in layers of counter-wound helixes that surround the ventricular cavity, conferring a special twisting motion during systole and early diastole, and providing the optimal stress and strain to generate the necessary forces to sustain the demand on a systemic ventricle. The morphologic right ventricle lacks the helical myocyte arrangement and the twisting or torsion component conferred by the helical arrangement, and thus are unable to sustain the demands of a systemic ventricle. When in the systemic position, the morphologic right ventricle is also unable to respond to an increasing work demand, such as exercise, in the fashion of the systemic left ventricle.36 When evaluating the single ventricle patient it is also important to assess the presence of atrioventricular synchrony, since the atrial contraction can provide up to 25 percent of the stoke volume at a given heart rate. Once there is fluid retention due to systemic ventricular dysfunction, the goal is to achieve a euvolemic state. Digoxin can be used to relieve symptoms of systemic ventricular dysfunction in a univentricular circulation, but is not currently recommended in the asymptomatic patient. This recommendation is an expert consensus, with no specific evidence of safety or benefit in this setting.6 ACE inhibitors should be employed in patients with symptomatic or asymptomatic systemic ventricular dysfunction,37 unless there is a specific contraindication. Finally, the use of beta-blockers are not routinely recommended for systemic ventricular dysfunction in the single 52 Vol. 64, No. 3 2013 Northeast Florida Medicine

ventricle patient, given the lack of evidence to support their benefit, and the potential complication of impairing chronotropy and potentially worsening exercise tolerance.

Decompensated heart failure

Acute decompensated heart failure is when the patient starts to develop signs or symptoms of heart failure due to pulmonary and systemic congestion due to increased left and right heart filling pressures.38 Indications for hospitalization include systemic manifestations such as (1) a change in mental status; (2) tachypnea, dyspnea and orthopnea; (3) anorexia, nausea and vomiting; (4) peripheral edema and inadequate urine output; and (5) resting tachycardia.39 Once there’s an exacerbation of heart failure, management with inotropic support may be necessary to increase contractility with pharmacologic agents that, through a common pathway, increase intracellular levels of cyclic adenylate monophosphate (cAMP), which in turn increase calcium release from the sarcoplasmic reticulum and increase the contractile force of the myocardium. There are two pathways that increase the cAMP, one mediated by beta-adrenergic-mediated stimulation (increased production) and another by phosphodiesterase III (PDE III) inhibition (decreased degradation).40 Inotropes with vasoconstrictive properties working on beta-stimulation (dobutamine, dopamine and epinephrine) are the most potent positive inotropic agents, and their effects are not limited to inotropy. They have chronotropic properties, as well as dose-dependent effects on vascular beds of organs. Therefore, the choice of an agent will depend on the state of the circulation and the myocardium.6 As a negative effect, these drugs increase the oxygen consumption of the myocardium, which is already compromised in the failing myocardium. Dopamine effects are dose dependent. At a low-dose dopamine (1–2 μg/kg per minute) stimulates dopamine receptors (D1, D2) located at the vascular beds in the kidney, mesentery and coronary arteries. The primary mechanism is to decrease systemic vascular resistance through vasodilation increasing blood flow. (The presence of this effect remains controversial). Medium-dose dopamine (2–10 μg/kg per minute) stimulates beta-adrenergic receptors, increasing myocardial contractility, heart rate, and norepinephrine release. High-dose dopamine (10–20 μg/kg per minute) stimulates alpha-receptors, leading to peripheral vasoconstriction and increases in systemic and pulmonary vascular resistance.47 Dobutamine used at low infusion rates (< 5 μg/kg per minute) decreases the pulmonary capillary wedge pressure and increases in cardiac output and blood pressure without an increase in heart rate.41 At higher dosages (> 5 μg/kg per minute) the heart rate will increase, while the systemic vascular resistance will remain either unchanged42 or decrease.43 Some studies have demonstrated deleterious effects of dobutamine caused by the neurohormonal activation and increased oxygen consumption by the myocardium.44,45 Epinephrine in low infusion doses (0.01–0.05 μg/kg per minute) will preferentially activate the β1 receptors in the myocardium and the conduction system. This will decrease systemic vascular resistance and increase in heart rate and systolic blood pressure, which overall will increase the cardiac output. www . DCMS online . org


Cardiac Disease Section

Figure 1

Several of the available pump sizes of the Berlin Heart EXCOR®.

Milrinone, is considered by some experts as the preferred inotrope to be used in the hemodynamically stable child presenting with decompensated heart failure. The mechanism of action of this drug is inhibition of phosphodiesterase III, resulting in increased cAMP levels in myocardial and vascular smooth muscle, which increases the intracellular calcium concentration, improving myocardial contractility and relaxing the systemic vascular resistance.47 Milrinone increases cardiac output and reduces cardiac filling pressures, pulmonary vascular resistance, and systemic vascular resistance with minimal effect on the heart rate and systemic blood pressure of adult patients. It is a great drug to use in heart failure patients, because overall it increases myocardial contractility and decreases afterload on the failing heart without a significant increase in myocardial oxygen consumption.46,47,48,49 When the low cardiac output is not associated with hypotension (or with elevated systemic vascular resistance), failing heart will benefit from afterload reduction with nitroprusside. This is a vasodilator that causes the release of nitric oxide, activating guanylate cyclase and increasing intracellular cGMP which causes smooth muscle dilatation of the arterial and venous blood vessels.50 This drug has a very short half-life so titration to effect can be done on a minute-to-minute basis with usual doses of 0.5 to 8 μg/kg per minute. Arterial line monitoring is necessary when using this agent. Nitroglycerine has the same mechanism of action, but has much more venodilatation than arterial dilatation.

Newer agents

Levosimendan is a newer agent that is in clinical trials in the United States and Europe, but being used currently in other countries. This drug has a dual mechanism of action: it enhances calcium myofilament responsiveness by binding to cardiac troponin C, thereby increasing contraction, and opens adenosine triphosphate–sensitive potassium channels in myocytes and vascular smooth muscle cells, promoting vasodilation.51

Surgical Management of Acute Heart Failure (ventricular assist devices)

Mechanical circulatory support has now matured as a viable amd effective management of severe heart failure unresponsive to pharmacological maneuvers in infants, children and www . DCMS online . org

adults. Over the past two decades there have been advances in the engineering of better and smaller devices that try to mimic the heart pump. Currently there are multiple options for mechanical support, ranging from temporary measures like Extracorporeal Membrane Oxygenation (ECMO) to ventricular assist devices VAD). The most incredible advances have been made in the pediatric arena with multiple pump sizes for children of all sizes, ranging from neonates to young adults. Pediatric assist devices are usually used as a bridge to transplantation, while adult ventricular assist devices are now used as a destination therapy. The timing of ventricular assist device implantation is critical. The indications are evolving and depend on clinical judgment. Implantation of VADs too early exposes the patient to unnecessary surgery and potential device-related morbidity. It is mandatory to institute mechanical assist device therapy prior to any end-organ dysfunction. In the adult population a VAD is indicated when there is secondary organ dysfunction.52 Another decision that needs to be made is regarding the need to support the left ventricle only or both ventricles. Other than prolonged operative time, we have observed in our population few disadvantages to supporting the right ventricle as well as the left ventricle mechanically. Device options currently approved by the FDA in the adult population have demonstrated equal safety and morbidity. 53,54,55 The Thoratec and Heartmate VADs (Thoratec Corp., Pleasanton, CA), the BVS 5000 (ABIOMED, Inc., Danvers, MA), and the NovaCor LVAS (World-Heart Inc., Oakland, CA). The Thoratec device has been the most commonly used VAD in children. The criteria for implantation of a Thoratec device in the pediatric patient is to have a body surface area of at least 0.73 m2. The Berlin Heart EXCOR® (Berlin Heart AG Berlin, Germany) device has recently been approved by the FDA for the use in pediatric patients (previously used as a humanitarian device [HUD]). The Berlin Heart consists of a paracorporeal, pneumatically driven polyurethane blood pump with a multilayer flexible membrane that separates the blood and air chambers (Figure 1). Pump stroke volumes of 10, 25, 30, 50, 60, and 80 mL, are available making it ideal for children and adults of all sizes. The pump is driven by a pulsatile electropneumatic system. Northeast Florida Medicine Vol. 64, No. 3 2013 53


Cardiac Disease Section

Figure 2

The usual arrangement of the Syncardia device once implanted in the heart, substituting the ventricles and connecting each pump to the pulmonary artery in the right side and the aorta on the left ventricle. Courtesy of syncardia.com

More recently the Syncardia Total Artificial Heart (TAH) has been used. This device is a biventricular, pneumatic, pulsatile blood pump that completely replaces the patient’s native ventricles and all four cardiac valves orthotopically (Figure 2 and 3). It has a pneumatically driven diaphragm with pump sizes of 70 and 50 ml. In the United States, the device is powered by a large console on wheels that prevents hospital discharge; portable drivers permitting discharge from the hospital are currently used in Europe.56 v

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Figure 3

70 ml Syncardia pumps. Courtesy of syncardia.com

8. Pitt B, Zannad F, Remme WJ, et al. The effect of spironolactone on morbidity and mortality in patients with severe heart failure. Randomized Aldactone Evaluation Study Investigators. N Engl J Med 1999;341:709– 17. 9. Brunner-La Rocca HP, Vaddadi G, Esler MD. Recent insight into therapy of congestive heart failure: focus on ACE inhibition and angiotensin-II antagonism. J Am Coll Cardiol 1999;33:1163–73. 10. Braunwald E, Bristow MR. Congestive heart failure: fifty years of progress. Circulation 2000;102:IV14 –23. 11. Brunner-La Rocca HP, Vaddadi G, Esler MD. Recent insight into therapy of congestive heart failure: focus on ACE inhibition and angiotensin-II antagonism. J Am Coll Cardiol 1999;33:1163–73. 12. Swedberg K, Eneroth P, Kjekshus J, Wilhelmsen L. Hormones regulating cardiovascular function in patients with severe congestive heart failure and their relation to mortality. CONSENSUS Trial Study Group. Circulation 1990;82:1730 – 6. 13. Effects of enalapril on mortality in severe congestive heart failure. Results of the Cooperative North Scandi- navian Enalapril Survival Study (CONSENSUS). The CONSENSUS Trial Study Group. N Engl J Med 1987; 316:1429- 35. , 14. Cohn JN, Johnson G, Ziesche S, et al. A comparison of enalapril with hydralazine-isosorbide dinitrate in the treatment of chronic congestive heart failure. N Engl J Med 1991;325:303–10. 15. Packer M. Do angiotensin-converting enzyme inhibitors prolong life in patients with heart failure treated in clinical practice? J Am Coll Cardiol 1996;28:1323–7. 16. Lin M, Chiang HT, Lin SL, et al. Vasodilator therapy in chronic asymptomatic aortic regurgitation: enalapril versus hydralazine therapy. J Am Coll Cardiol 1994;24: 1046 –53. 17. Pitt B, Konstam MA. Overview of angiotensin II-receptor antagonists. Am J Cardiol 1998;82:47S–9S. 18. Pitt B, Segal R, Martinez FA, et al. Randomised trial of losartan versus captopril in patients over 65 with heart failure (Evaluation of Losartan in the Elderly Study, ELITE). Lancet 1997;349:747–52. 19. Pitt B, Poole-Wilson P, Segal R, et al. Effects of losartan versus captopril on mortality in patients with symptomatic heart failure: rationale, design, and baseline characteristics of patients in the Losartan Heart Failure Survival Study–ELITE II. J Card Fail www . DCMS online . org


Cardiac Disease Section 1999;5:146 –54. 20. Bengur AR, Beekman RH, Rocchini AP, Crowley DC, Schork MA, Rosenthal A. Acute hemodynamic effects of captopril in children with a congestive or restrictive cardiomyopathy. Circulation 1991;83:523–7. 21. Leversha AM, Wilson NJ, Clarkson PM, Calder AL, Ram- age MC, Neutze JM. Efficacy and dosage of enalapril in congenital and acquired heart disease. Arch Dis Child 1994;70:35–9. 22. Stern H, Weil J, Genz T, Vogt W, Buhlmeyer K. Captopril in children with dilated cardiomyopathy: acute and long-term effects in a prospective study of hemodynamic and hormonal effects. Pediatr Cardiol 1990;11:22– 8. 23. Lewis AB, Chabot M. The effect of treatment with angiotensin-converting enzyme inhibitors on survival of pediatric patients with dilated cardiomyopathy. Pediatr Cardiol 1993;14:9 –12. 24. Communal C, Singh K, Pimentel DR, Colucci WS. Nor- epinephrine stimulates apoptosis in adult rat ventricular myocytes by activation of the beta-adrenergic pathway. Circulation 1998;98:1329 –34. 25. Smith KM, Macmillan JB, McGrath JC. Investigation of alpha1-adrenoceptor subtypes mediating vasoconstriction in rabbit cutaneous resistance arteries. Br J Pharmacol 1997;122:825–32. 26. Packer M. Current role of beta-adrenergic blockers in the management of chronic heart failure. Am J Med 2001; 110(Suppl 7A):81S–94S. 27. Rusconi P, Gomez-Marin O, Rossique-Gonzalez M, et al. Carvedilol in children with cardiomyopathy: 3-year experience at a single institution. J Heart Lung Transplant 2004;23:832-38. 28. Hjalmarson A, Goldstein S, Fagerberg B, et al. Effects of controlled-release metoprolol on total mortality, hospitalizations, and well-being in patients with heart failure: the Metoprolol CR/XL Randomized Intervention Trial in congestive heart failure (MERIT-HF). MERIT-HF Study Group. Jama 2000;283:1295–302. 29. Colucci WS, Packer M, Bristow MR, et al. Carvedilol inhibits clinical progression in patients with mild symp- toms of heart failure. US Carvedilol Heart Failure Study Group. Circulation 1996;94:2800 – 6. 30. Packer M, Colucci WS, Sackner-Bernstein JD, et al. Double-blind, placebo-controlled study of the effects of carvedilol in patients with moderate to severe heart failure. The PRECISE Trial. Prospective Randomized Evaluation of Carvedilol on Symptoms and Exercise. Circulation 1996;94:2793–9. 31. Packer M, Coats AJ, Fowler MB, et al. Effect of carvedilol on survival in severe chronic heart failure. N Engl J Med 2001;344:1651– 8. 32. Shaddy R, Boucek M, Hsu D, Boucek R, Canter C, Mahony L, Ross R, Pahl E, Blume E, Dodd D, Rosenthal D, Tani L. Multicenter, randomized, placebo-controlled double-blind trial of carvedilol in children with heart failure. Late-Breaking Clinical Trials JACC Supplement D: 47 (11), D18-19, 2006. 33. Adams Jr, KF, Gheorghiade M, Uretsky BF, Patterson JH, Schwartz TA, Young JB. Clinical benefits of low serum digoxin concentrations in heart failure. J Am Coll Cardiol 2002;39:946 –53. 34. Packer M, Gheorghiade M, Young JB, et al. Withdrawal of digoxin from patients with chronic heart failure treated with angiotensin-converting-enzyme inhibitors. RADIANCE Study. N Engl J Med 1993;329:1–7. 35. Fogel MA, Weinberg PM, Fellows KE, Hoffman EA. A study in ventricular-ventricular interaction. Single right ventricles compared with systemic right ventricles in a dual-chamber circulation. Circulation 1995;92:219 –30. 36. Wallis, G. A., Debich-Spicer, D., & Anderson, R. H. (2011). Congenitally corrected transposition. Orphanet Journal of Rare Diseases, 6(1), 22. doi:10.1186/1750-1172-6-22 www . DCMS online . org

37. Hjortdal VE, Stenbog EV, Ravn HB, et al. Neurohormonal activation late after cavopulmonary connection. Heart 2000;83:439 – 43. 38. Gheorghiade M, Zannad F, Sopko G, Klein L, Pina IL, Konstam MA, et al. Acute heart failure syndromes: current state and framework for future research. Circulation 2005;112(25): 3958-68. 39. Gazit, A. Z., & Oren, P. P. (2009). Pharmaceutical management of decompensated heart failure syndrome in children: current state of the art and a new approach. Current treatment options in cardiovascular medicine, 11(5), 403–409. 40. Lowes BD, Simon MA, Tsvetkova TO, Bristow MR. Inotropes in the beta-blocker era. Clin Cardiol 2000;23: III11– 6. 41. Berg RA, Donnerstein RL, Padbury JF: Dobutamine in stable, critically ill children: pharmacokinetics and hemodynamic actions. Crit Care Med 1993, 21:678–686. 42. Driscoll DJ, Gillette PC, Duff DF, et al.: Hemodynamic effects of dobutamine in children. Am J Cardiol 1979, 45:581–585. 43. Perkin RM, Levin DL, Webb R, et al.: Dobutamine: a hemodynamic evaluation in children with shock. J Pediatr. 1982, 100:977–983. 44. O’Connor CM, Gattis WA, Uretsky BF, et al.: Continuous intravenous dobutamine is associated with an increased risk
of death in patients with advanced heart failure: insights from the Flolan International Randomized survival Trial
(FIRST). Am Heart J 1999, 138:78–86. 45. Van Bakel AB, Chidsey G: Management of advanced heart
failure. Clin Cornerstone 2000, 3:25–35. 46. Wessel DL. Managing low cardiac output syndrome after congenital heart surgery. Crit Care Med 2001;29: S220 –30. 47. Tabbutt S. Heart failure in pediatric septic shock: utilizing inotropic support. Crit Care Med 2001;29:S231–6. 48. Chang AC, Atz AM, Wernovsky G, Burke RP, Wessel DL. Milrinone: systemic and pulmonary hemodynamic effects in neonates after cardiac surgery. Crit Care Med 1995;23:1907–14. 49. Hoffman TM, Wernovsky G, Atz AM, et al. Prophylactic intravenous use of milrinone after cardiac operation in pediatrics (PRIMACORP) study. Prophylactic Intrave- nous Use of Milrinone After Cardiac Operation in Pedi- atrics. Am Heart J 2002;143:15–21. 50. Smith RP, Kruszyna H: Nitroprusside produces cyanide 
 poisoning via a reaction with hemoglobin. J Pharmacol 
Exp Ther 1974, 191:557–563. 51. Gheorghiade M, Teerlink JR, Mebazaa A: Pharmacology of new agents for acute heart failure syndromes. Am J Cardiol 2005, 96:68G–73G. 52. Deng MC, Loebe M, El-Banayosy A, Gronda E, Jansen PG, Vigano M, et al. Mechanical circulatory support for advanced heart failure: effect of patient selection on outcome. Circulation 2001;103:231–7. 53. Helman DN, Addonizio LJ, Morales DL, Catanese KA, Flannery MA, Quagebeur JM, et al. Implantable left ventricular assist devices can successfully bridge adolescent patients to transplant. J Heart Lung Transplant 2000;19:121–6. 54. Hill JD, Reinhartz O. Clinical outcomes in pediatric patients implanted with Thoratec ventricular assist. Semin Thorac Cardiovasc Surg Pediatr Card Annu 2006:115–22. 55. ReinhartzO, KeithFM, El-BanayosyA, McBrideLR, RobbinsRC, Copeland JG, et al. Multicenter experience with the Thoratec ventricular assist device in children and adolescents. J Heart Lung Transplant 2001;20(4):439–48. 56. Copeland JG, et al. Cardiac Replacement with a Total Artificial Heart as a Bridge to Transplantation.N Engl J Med 2004;351:859-67

Northeast Florida Medicine Vol. 64, No. 3 2013 55


Nominating Committee Report

Duval County Medical Society 555 Bishopgate Lane • Jacksonville FL 32204 Phone (904) 355-6561 • Fax (904) 353-5848 • Email: dcms@dcmsonline.org

To: From: Date: Subj:

Members, Duval County Medical Society Ashley B. Norse, MD, Chair, 2013 DCMS Nominating Committee September 9, 2013 2013 DCMS Nominating Committee Report

The 2013 Duval County Medical Society (DCMS) Nominating Committee has completed its work and is pleased to present to the membership a proposed slate of Officers, Directors, FMA Delegates and Alternate Delegates for 2012. The membership will be voting on the slate of nominees at the DCMS Annual Meeting, which will be held on December 4, 2013, 5:45 pm, at the Hyatt Regency Jacksonville Riverfront Hotel. Officers, Board Members, Delegates and Alternate Delegates will begin their terms on January 1, 2014. The Nominating Committee is composed of the following physicians: Ashley Norse, MD, Chair; Eli Lerner, MD (Ex-Officio/President); Mobeen Rathore (Ex-Officio/President-Elect); Cynthia Anderson, MD; Raed Assar, MD; TraChella Johnson Foy, MD; Sunil Joshi, MD (2013 Committee Member); and Daniel Kantor, MD. The Nominating Committee proposes the following: OFFICERS President-Elect: Vice Presidents: Secretary: Treasurer:

Daniel Kantor, MD Raed Assar, MD; Tra’Chella Johnson-Foy, MD; Stephen Mandia, MD Ruple Galani, MD Sunil Joshi, MD

BOARD OF DIRECTORS Term to Expire December 31, 2016 James St. George, MD Alexander Pogrebniak, MD Uday Deshmukh, MD Term to Expire December 31, 2014 Jason Meier, MD (filling unexpired term of Ruple Galani, MD, if elected Secretary) Iris Eisenberg, MD (filling unexpired term of TraChella Johnson-Foy, MD, if elected Vice President) James Joyce, MD (filling unexpired term of Stephen Mandia, MD, if elected Vice President) FMA DELEGATES Term to Expire December 31, 2016 Jessica Bahari-Kashani, MD Patrick DeMarco, MD Uday Deshmukh, MD E. Rawson Griffin, MD

TraChella Johnson-Foy, MD Glenn W. Knox, MD Harry M. Koslowski, MD Nathan Newman, MD

H. Martin Northup, MD Alexander Pogrebniak, MD James St. George, MD

Orlando Florete, MD Malcolm Foster, MD Michael Fox, MD Michael Lewis, MD Charles B. McIntosh, MD Jesse McRae, MD

Michael Nussbaum, MD Robert A. Ponte, MD Keith L. Stein, MD Sanjay Swami, MD Floyd B. Willis, MD

FMA ALTERNATE DELEGATES Term to Expire December 31, 2014 Hernan Chang, MD Paul Chappano, MD Joseph Costa, MD Jefferson Edwards, MD Rui Fernandes, MD Mark Fleisher, MD

56 Vol. 64, No. 3 2013 Northeast Florida Medicine

www . DCMS online . org


Trends in Public Health

The Ups and Downs of Sickle Cell Disease Trends Ikechi Konkwo, MD, MPH; Kelli Wells, MD; Kathryn Lukens-Bull, MPH; Radley Remo, MPH and Luninita Razaila One of the new Healthy People 2020 topics is Blood Disorders and Blood Safety.1 There is no current baseline data for the major hemoglobinopathies, sickle cell disease (SCD) and the thalassemias, included in this topic. A recent Public Health Report2 indicated generally improving survival among SCD patients, but a concurrent worsening of outcomes in the adult population. In 2010, all deaths following an admission from the Emergency Department for Sickle Cell crises from the Healthcare Cost and Utilization Project (HCUPnet) were in the adult population- ages 18-64 years old.3 There has not been a recent article on healthcare utilization at the national level, but the last estimate in 2009 was that the annual cost for the then 70,000 SCD patients in the US stood at about $1.1 billion.4 Now, there are an estimated 70,000 to 100,000 people living with the condition, with 3 million living with the sickle trait.5 There is no comprehensive network of services for this hereditary condition as there is for Cystic Fibrosis and Hemophilia, though the number of SCD cases in the US is estimated to be much more than CF and hemophilia put together. Sickle Cell Disease results in abnormal sickle shaped red blood cells causing various clinical presentations. The abnormal red blood cells can block small arteries to organs leading to painful episodes called sickle cell crises, a common presentation of these patients in the emergency room. The frequency of these crises, like in any other chronic condition, is related to the quality of and access to healthcare and directly impacts the quality of life of the SCD patient. Prompt and proper treatment of the crises with adequate follow up according to the guidelines for treatment often results in a discharge of the majority of patients from the Emergency Department without admissions or repeated visits. Kauf et al4 showed that the major portion of Healthcare costs (85 percent) was due to hospitalizations. HCUP estimate for discharged SCD Hospital stays in 2011 was more than $650 million. One in four of these patients were readmitted within 30 days with the same principal diagnosis, accounting for an extra estimated $182.4 million in medical costs. Regionally, the South (including Florida) accounted for more than half (55 percent) of all emergency department visits from sickle cell crises in the U.S. in 2010, surpassing the total for the other three regions. In Florida, unlike other states reporting to the HCUPnet, a greater percentage of SCD cases in crisis that presents in the ED end up in hospital admission. Rates of ED visits have also been increasing in Florida. The majority of the ED visits (and admissions) occur in the age group 18-44years (75 percent), the most productive years of the patients’ lives. In 2011, the total charges for ED visits for SCD crises stood at $19.7 million. Duval County accounted for about one in 10 of all SCD crises ED visits in Florida in 2011. There were 147 SCD patients and a total of 474 ED visits for SCD crises, of which 60 were www . DCMS online . org

repeat visits accounting for an extra $232,440 in charges. Total charges for ED for sickle cell crises in Duval County stood at about $1.3million ($16.1 million for 608 hospitalizations) in 2011. The majority of cases were also in the adult productive years. The quality of life in this population is greatly impacted by repeated episodes of painful crises and hospital admissions. SCD appears to be a significant source of healthcare costs in Duval County, Florida and the U.S. There is a need to look into developing a comprehensive system of care as with other chronic conditions that have benefitted from the medical home model like diabetes and heart failure. Pediatric care in the U.S. has been noted to be more efficient in this patient population than adult care, prompting further inquiry into the failure in the transition of care for SCD patients. Treatment centers like those for Cystic Fibrosis and hemophilia would go a long way to improve the quality of life of SCD patients.5 Barriers to proper care in these patients and the regional and subpopulation differences in hospitalizations and ED use are possible areas for further study. SCD is easily an Ambulatory Sensitive Condition with standard comprehensive care delivered out of ambulatory clinics as in Africa, where it is most prevalent. SCD could benefit from closer attention to the standards of care easily hardwired by an appropriate electronic tracking system like the registries supported by electronic medical records. In addition to meeting specific objectives for blood disorders, improving outcomes in SCD could also address health disparities, another focus of the Healthy People 2020 vision.

Resources: 1. “Blood Disorders and Blood Safety - Healthy People.” Healthy People 2020 - Improving the Health of Americans. http://www. healthypeople.gov/2020/topicsobjectives2020/nationaldata.aspx?topicId=4 (accessed June 20, 2013). 2. Lanzcron, S, CP Carroll, and C Haywood. “Mortality rates and age at death from sickle cell disease: U.S., 1979-2005.” Public Health Rep. 2, no. 128 (2013): 110-116. http://www.ncbi.nlm.nih.gov/ pubmed/23450875 (accessed June 20, 2013). 3. Agency for Healthcare Research and Quality. “HCUPnet: A tool for identifying, tracking, and analyzing national hospital statistics.” http://hcupnet.ahrq.gov/HCUPnet.jsp (accessed June 20, 2013). 4. Kauf, TL. , TD. Coates, L Huazhi, N Mody-Patel, and A Hartzema. “5. The cost of health care for children and adults with sickle cell disease..” American Journal of Hematology 84 (2009): 323-327. http://onlinelibrary.wiley.com/doi/10.1002/ajh.21408/pdf 5. Grosse, SD, MS Schechter, R Kulkarni, MA Loyd-Puryear, B Strickland, and E Trevathan. “5. Models of Comprehensive Multidisciplinary Care for Individuals in the United States With Genetic Disorders..” Pediatrics 123 (2009): 407-412. http://pediatrics. aappublications.org/content/123/1/407.full.html Northeast Florida Medicine Vol. 64, No. 3 2013 57


From the President’s Desk

As we celebrate our 160th birthday, our year at DCMS continues to become more exciting. Programs that we initiated earlier in the year are taking hold in a positive manner. Our linkage with UF Health is tighter now than at any previous time, and other community organizations enjoy our mutual participation. We have moved forward with Public Health issues and have taken a leadership role in the Florida Medical Association. Most importantly, we will be in a new home before the end of the year located centrally in downtown Jacksonville as a result of the sale of our building this year. We are enjoying a positive cash flow for the first time in a good number of years with the successful growth and management of the organization. Programs aimed at young members have been a huge success and are offering a new path for participation for DCMS members with young families, new practice configurations, and not a lot of time. Meetings are rotated around the county and feature speakers in areas Eli Lerner, MD not covered in medical school 2013 DCMS President or Residencies and which are essential for surviving and thriving in today’s medical world. These are especially important since we are able to offer material to these youngest members of our profession that is not available to them in their formal training programs. We also have begun a series of similar programs for Residents at UF Health with various experts in practice development. The series is an ongoing partnership with UF Health and will cover the non-medical essentials of a successful practice. This promises to be an excellent introductory course and is being monitored closely by Dean Daniel Wilson and the DCMS. This effort also is helping us build stronger relations with UF Health. As the fortunes of Obamacare change almost on a daily basis, we understand the importance of this legislation on the future of health care on the local level and are preparing an in-depth conference on this topic to precede our annual meeting on December 4, 2013 entitled “ACOs and the Future of Healthcare” at the Hyatt Regency Jacksonville Riverfront Hotel. This meeting is presented as a service to our membership and will include topics like how to negotiate ACO contracts, how to get the best leverage in these groups, how the ACOs work, and the legal ups and downs of our relationship with ACOs. The idea is to discuss these points on the local level where we are all practicing with local experts. It is essential for us and our patients

58 Vol. 64, No. 3 2013 Northeast Florida Medicine

that we understand ACOs and how they work to interface with the practice of medicine. It will be the most important meeting of the year in terms of our practice futures. The meeting will be followed immediately by the DCMS Annual Meeting. We were instrumental in helping in recruitment of Dr. Kelli Wells as the Director of Public Health for Duval County. Dr. Daniel Kantor was on the Search Committee and did a yeoman’s job of keeping us at the forefront of the hiring of our new County Director. We look forward to a long relationship with the county Board of Health under her tutelage. Dr. Mobeen Rathore, our President-Elect is monitoring a new viral disease, Middle East Respiratory Syndrome. His report is present in the early August edition of our newsletter and is available to all concerned with patients with possible exposure who have recently traveled to the Middle East. We are deeply involved with the leadership of the Florida Medical Association. Dr. Alan Harmon became President of the FMA at the last Annual Meeting. We are honored to have his steady, well-reasoned hand at the helm as we embark on a sea of troubled waters with the advent of ObamaCare and ACOs. His clear, concise vision will help navigate us through the tough spots and will be very effective in interactions with the state legislature. Dr. Ashley Norse continues as our District Representative on the Board of Directors. I am Council Chair for CEJA. We have Drs. Alan Harmon, John Montgomery, Ashley Norse, Ross Griffin, Nate Newman and Tom Peters representing us in the Florida Delegation of the AMA. Past Presidents Drs. Yank Coble and Cecil Wilson also represent us in the House of Delegates. Alan Harmon sits on the Council on Medical Economics. And we are moving! We sold our old headquarters and will be moving in to a new home at the Gate Riverplace Tower building on the South Bank, which also houses the University Club. This move out of the old headquarters was necessary because of the escalating expenses of staying there with yearly increases in repair bills and services. We will have new offices, and Board meetings will be held at the University Club. We will also host other member meetings at the University Club. I believe that this will be our most important change for the year. It should give us a new home at a new address that can only enhance our expanding activities. In essence, we’re moving “uptown” to a new location with a new look. If that’s not enough, we will have enjoyed several new events before my message is published including a behind the scenes tour with the Jacksonville Jaguars, the annual DCMS & Navy dinner and the DCMS Night at the Symphony. A lot to digest, but there’s more coming… Eli Lerner, M.D.; F.A.C.S

www . DCMS online . org


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The Doctors Company is devoted to helping doctors avoid potential lawsuits. For us, this starts with patient safety. In fact, we have the largest Department of Patient Safety of any medical malpractice insurer. And, local physician advisory boards across the country. Why do we go this far? Because sometimes the best way to look out for the doctor is to start with the patient. To learn more about our medical malpractice insurance program, call our Jacksonville office at (800) 741-3742 or visit www.thedoctors.com.

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Northeast Florida Medicine - Autumn 2013 - Cardiac Disease  

Our Autumn 2013 issue on Cardiac Disease is guest edited by DCMS member Dr. Randall Bryant. It offers CME credit on Adult Congenital Cardiac...

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