Cleveland Clinic Florida Cardiac Care - Summer/Fall 2021

SUMMER/FALL 2021

Cardiac Care An Update for Physicians from the Heart, Vascular and Thoracic Institute

Feature Article

Cardiac Amyloidosis – our center of excellence for diagnosis and management strategies p.12

Expanding the field of transcatheter heart valve therapies p.4 Shockwave intravascular lithotripsy in the coronary circulation p.10 Long-term support with LVAD – an alternative to transplant p.18

José L. Navia, MD, FACC Director of Florida Regional Heart, Vascular and Thoracic Institute Director of Heart and Vascular Center, Weston Hospital Chairman of Cardiothoracic Surgery, Cleveland Clinic Florida S. Donald Sussman Distinguished Chair in Heart and Vascular Research

Dear Colleagues, When you are trying to find something, it is helpful to know what you are looking for. This truth applies to many situations in life as well as in medicine. It is, perhaps, one of the reasons for the recent rise in diagnoses of cardiac amyloidosis. We have come to understand this disease so much better in recent years that we now know to look for it as a possible cause of congestive heart failure – particularly when coronary artery disease and hypertension have been ruled out and other symptoms are present.

Here at Cleveland Clinic Florida, we have established the Cardiac Amyloidosis Center of Excellence under the leadership of David Wolinsky, MD, a nuclear cardiologist and renowned expert in the disease. Within the center we are currently treating about 200 patients, conducting clinical research trials and developing treatment protocols for the growing number of people facing this deadly diagnosis. You can read more about it all on page 12.

As we continue to expand our heart, vascular and thoracic program here in Florida our structural heart disease program is growing as well. Our expert team of interventional cardiologists offers the latest minimally invasive catheter-based procedures for patients with advanced and complex structural heart disease. In the story on page 4, Emad Hakemi, MD, highlights the innovative procedures such as TAVR, MitraClipTM and transcatheter mitral valve replacement that we are using to help our patients.

With innovation comes the opportunity to carefully tailor treatments to individual patients. For example, Mariano Brizzio, MD, explains in the article on page 6 how using the off-pump technique for coronary artery bypass grafting has become a more common approach at Cleveland Clinic Indian River Hospital due to the benefits it provides the elderly population served in the region. And on page 10, Stephen McIntyre, MD, of Cleveland Clinic Martin Health, details his use of shockwave intravascular lithotripsy to treat severely calcified lesions in the coronary arteries. This innovative use of existing technology has been a breakthrough for treating difficult cases.

On page 16, we introduce you to one of our patients, a local physician and avid cyclist with a strong family history of heart disease who found himself facing his own health challenges. His path led him to the care of our cardiology team and eventually a heart transplant at Cleveland Clinic Weston Hospital. At 64 years old he is filled with renewed energy to carry on his medical practice and continue enjoying his family – and his bike rides.

We hope you enjoy this issue of Cardiac Care. Should you be looking for a partner in specialized care for your patients, we welcome the opportunity to work with you.

Respectfully,

José L. Navia, MD, FACC

Table of Contents Cardiac Care is produced by Cleveland Clinic Florida’s Heart, Vascular and Thoracic Institute.

Medical Editors

José L. Navia, MD, FACC naviaj@ccf.org Nicolas Brozzi, MD, FACC brozzin@ccf.org

Administrator

Bradley Tipper, MSHA, MSHI

Expanding the Field of Transcatheter Heart Valve Therapies at Cleveland Clinic Florida............................................................. 4 Coronary Bypass Surgery in a Beating Heart: Off-Pump Procedures Prevalent at Cleveland Clinic Indian River Hospital........................... 6 Enhanced Operating Rooms Accommodate Multispecialty Use While Improving the Patient Experience................................... 7 Enhanced Recovery After Cardiac Surgery Provides Many Perioperative Benefits and Favorable Long-Term Outcomes............... 8

Christine Harrell

A New Tool for the Non-Dilatable Lesion: Shockwave Intravascular Lithotripsy Proves Beneficial in Treating Deep Calcified Coronary Lesions..................................10

Art Director

Cardiac Amyloidosis Center of Excellence .....................................12

Marketing

The Ride of a Lifetime: Heart Transplant Gets Physician Cyclist Back on the Trails after Years of Progressive Heart Failure...............16

Managing Editor

Suzette Lopez

Evelyn Arias, Senior Director Christina Garcia, Regional Service Line Manager

Long-Term Support with a Left Ventricular Assist Device as an Alternative to Cardiac Transplantation...........................................18 Growing Conduction System Pacing Program at Cleveland Clinic Florida: An Alternative that Preserves Normal Physiology................20

Imaging Coronary Anomalies...................................................21 New Staff.................................................................................22 Cleveland Clinic Florida’s Weston Hospital has once again earned the top spot as the #1 hospital in the Miami-Fort Lauderdale metro area for 2021-2022, according to the newly released annual ranking of Best Hospitals by U.S. News & World Report. It is the fourth consecutive year Cleveland Clinic Weston Hospital has earned the top ranking, the only hospital to be ranked #1 for four straight years in South Florida.

COVER: Top Row, Left — Thioflavin-s stain shows bright blue amyloid fibrils in arterioles and interstitium. Center — Transthoracic echo showing marked left ventricular hypertrophy, bi-atrial enlargement, and thickened mitral valve leaflets. Right — Short axis SPECT slice showing diffuse intense myocardial uptake of technetium pyrophosphate confirmed diagnosis of ATTR cardiac amyloidosis. Bottom Graphic — Transverse SPECT-CT demonstrating clear-cut myocardial uptake of technetium pyrophosphate with mild apical sparing. Addition of CT helps differentiate myocardial uptake from blood pool uptake.

NOTE: The photo of Brett Faulknier, DO, in the previous publication (top photo, page 11), was taken without a patient. It was solely a representation of a procedure.

To reach a staff member or to inquire about our services, please call:

clevelandclinicflorida.org/heart

Cleveland Clinic Weston Hospital

Cleveland Clinic Indian River Hospital

Cleveland Clinic Martin Health

Cardiology 954.659.5290

Cardiology 772.778.8687

Cardiothoracic Surgery 954.659.5320

Cardiothoracic Surgery 772.563.4580

Cardiology and Cardiothoracic Surgery 772.419.2137

Expanding the Field of Transcatheter Heart Valve Therapies at Cleveland Clinic Florida

By Emad Hakemi, MD, MS

Until recent years, open-heart surgery has been the standard of care for most patients with valvular disease, including aortic stenosis. However, minimally invasive procedures such as transcatheter aortic valve replacement (TAVR) and MitraClip™ have emerged as safe and effective minimally invasive alternatives for patients with advanced and complex valvular disease.

Through Cleveland Clinic Florida’s Structural Heart Disease Program and Multidisciplinary Valve Clinic, our team of interventional cardiologists provides innovative, multispecialty care and access to the industry’s most advanced technology and procedures, including the use of balloon-expandable transcatheter valve bioprosthesis for patients with degenerated mitral valve bioprosthesis or failing mitral valve repair.

Transcatheter Aortic Valve Replacement (TAVR) In recent years, the minimally invasive TAVR procedure has revolutionized the management of valvular heart disease. A multitude of studies has shown TAVR to be a safe and effective alternative to traditional valve replacement.

TAVR is available for patients of all risk levels with severe aortic stenosis, including those with degenerative aortic bioprosthesis stenosis or regurgitation.

Figure 1: Transcatheter aortic valve replacement in patient with severe aortic valve stenosis.

At Cleveland Clinic Florida, TAVR is routinely performed using a minimalistic approach. With the patient under local anesthesia and mild sedation, a new valve is inserted within the diseased valve through percutaneous transfemoral access. This approach provides patients with an expedited recovery with early discharge at one to two days. Our specialists have more than 10 years of experience with TAVR procedures, and the center performs among the highest volume of implants in Florida. Our expertise in structural heart disease combined with an extensive experience in TAVR translate to the best possible patient outcomes.

Figure 2: Transcatheter aortic valve replacement in patient with degenerated prior aortic bioprosthesis.

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Transcatheter Mitral Valve Edge-to-Edge Repair The transcatheter mitral valve edge-to-edge repair procedure has evolved to become an important solution for patients with mitral regurgitation for whom medical therapy fails. Initially approved for non-surgical candidate patients with severe degenerative mitral regurgitation (MR), this therapy is now also available for those with functional mitral regurgitation. In the multicenter randomized COAPT trial of patients with severe functional MR, this therapy resulted in marked improvement in survival, hospitalizations and heart failure class. MitraClip™ is presently the only FDA-approved transcatheter therapy for this indication. The procedure is performed under general anesthesia using 3D transesophageal echocardiography. Following transeptal access, the MitraClip is delivered to create a double orifice mitral valve that reduces the severity of the mitral regurgitation. One or more clips may be necessary. The device has been shown to provide benefit and improvement in survival and quality of life when used in patients with reduced heart function.

... minimally invasive procedures such as transcatheter aortic valve replacement (TAVR) and MitraClip™ have emerged as safe and effective minimally invasive alternatives ...

Figure 3: A transcatheter implanted clip improves coaptation of the mitral valve leaflets.

Transcatheter Mitral Valve Replacement The use of balloon-expandable transcatheter valve bioprosthesis has been broadened to cover patients with degenerated mitral valve bioprosthesis or failing mitral valve repair.

This groundbreaking minimally invasive procedure is done under general anesthesia, and the femoral vein is used to advance a transcatheter bioprosthesis through the inter-atrial septum into the left atrial cavity. From there, the valve is positioned and deployed within the degenerated mitral valve bioprosthesis or mitral valve ring. Patients benefit from the less invasive nature of this therapy with a faster recovery in comparison to the extensive surgery that had traditionally been performed, which required repeat sternotomy for redo mitral valve replacement.

Figure 4: Transcatheter mitral valve replacement in patient with degenerated mitral valve bioprosthesis.

Dr. Hakemi hakemie@ccf.org CLEVELAND CLINIC FLORIDA

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Coronary Bypass Surgery in a Beating Heart Off-Pump Procedures Prevalent at Cleveland Clinic Indian River Hospital

By Mariano Brizzio, MD Coronary artery bypass grafting (CABG) surgery was developed more than 50 years ago. Rene Favaloro, MD, a pioneer of the surgical technique, published the world’s first reported CABG in 1968 at Cleveland Clinic. CABG surgery withstands the test of time: Few other therapies have long-term outcomes for report 20 to 30 years later.

Good results in CABG surgery in selected centers are a function of appropriate patient selection and a systematic approach to the surgical technique that is based on meticulous construction of a conduit-coronary anastomosis and efficiency in the management of the operation.

Expeditious and accurate performance of coronary anastomosis was imperative at a time when the best a surgeon could do to minimize myocardial injury was to reduce the period of ischemic time. The introduction of the systematic use of cardioplegia combined with retrograde delivery techniques in the 1980s leveled the playing field.

These techniques allowed surgeons the opportunity to operate using the cardiopulmonary bypass machine in an arrested heart, thereby eliminating the pressure exerted by the original techniques. In this context results improved, and the operation was 6

accepted, making it a significant treatment in the management of patients with coronary disease.

Benefits of off-pump CABG surgery However, CABG surgery was impacted in the late 1990s by the introduction of off-pump techniques – performing coronary bypasses without the utilization of extracorporeal circulation. Later, a few publications including randomized clinical trials have shown no difference in results between off-pump and conventional coronary bypass.

Still, there is a select group of patients for whom off-pump CABG techniques are a better option.

Many publications have demonstrated the disadvantages of extracorporeal circulation in patients with the following: compromised renal function preoperatively, calcified ascending aortas, depressed pulmonary function and significant cerebrovascular disease. These patients definitively would see benefit from an off-pump CABG approach.

The off-pump technique also has been demonstrated to decrease both blood product transfusions and the incidence and severity of postoperative stroke.

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Soft stabilizer facilitates construction of coronary bypass anastomosis during beating heart surgery.

At Cleveland Clinic Indian River Hospital, the off-pump CABG technique is utilized routinely due to our particularly elderly population with complex coronary disease and multiple co-morbidities. We have had a 0 percent mortality rate in the last year and hold a 3-star rating by the Society of Thoracic Surgeons for isolated CABG procedures.

Dr. Brizzio began practicing at Cleveland Clinic Indian River Hospital about a year ago. Since then, he has performed more than 100 CABG procedures, most of which were off-pump.

Dr. Brizzio’s experience over the past 14 years includes more than 900 CABG procedures, with the majority being off-pump.

Dr. Brizzio brizzim@ccf.org

Enhanced Operating Rooms Accommodate Multispecialty Use While Improving the Patient Experience

By Philip Bongiorno, MD

New surgical techniques and advanced technology require the modern operating suite to easily accommodate multiple specialists working alongside each other.

Cleveland Clinic Weston Hospital’s five-story, 221,000-square-foot expansion wing has allowed the space for three enhanced operating rooms to fill this need — giving our surgeons access to the additional room along with the necessary technology, including robotic surgical platforms, angiography and an array of mechanical circulatory support options.

The evolved ORs are in close proximity to the intensive care units and create an efficiency in patient transport and enhanced patient safety. Each features almost 1,000 square feet of open floor space created by overhead booms that lift lights, monitors and equipment off the floor. The additional space is welcomed by the OR staff, surgical residents and medical students, and is key for moveable equipment such as the surgical robots and mechanical circulatory support machines.

will allow us to continue to provide the highest quality care for our patients. Ultimately, the new ORs will optimize surgical scheduling and provide the ability to care for more patients at Weston Hospital. They also may help with shorter patient transport and anesthesia times and, possibly, shorter hospital stays for patients.

Dr. Bongiorno bongiop@ccf.org

Our surgeons are enthusiastic about the enhanced facilities and how they

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Enhanced Recovery After Cardiac Surgery Provides Many Perioperative Benefits and Favorable Long-Term Outcomes

By Tatiana Jamroz, MD

Two years ago, the Cleveland Clinic Florida Heart, Vascular and Thoracic Institute (HVTI) cardiac team at Weston Hospital was among the first in the nation to adopt a revolutionary approach to all open-heart procedures – enhanced recovery after cardiac surgery (ERAS® Cardiac).

of the operating room. Cleveland Clinic Florida HVTI anesthesiologists’ experience in ERAS Cardiac is the foundation for ERAS protocol development across several surgical specialties within the Anesthesia

Cardiac intraoperative protocols limit opioid use and its side effects.

Excellent analgesia can be obtained with a combination of non-opioid medications (e.g., ketamine, lidocaine, dexmedetomidine) and

Colorectal surgeons began using the enhanced recovery after surgery (ERAS) approach about a decade ago.

Since then, many other specialties, such as orthopaedics, have begun to use it and adapt it to their needs.

Cardiac surgery has recently joined the ERAS® Society with cardiacspecific guidelines proposed by the Cardiothoracic Surgery Task Force.

The goal of ERAS Cardiac is to promote healing and optimize recovery to allow patients to get back to their baseline levels faster postoperatively.

While Cleveland Clinic Florida at Weston cardiac anesthesiologists have been using components of ERAS Cardiac for some time, it requires the participation of many other types of practitioners on the cardiac team in the pre- and postoperative period to maximize its effect.

It is important to take a multimodal, multidisciplinary approach to ERAS Cardiac for perioperative care because a high percentage of preventable perioperative morbidity occurs outside 8

Williams JB, McConnell G, Allender JE, Woltz P, Kane K, Smith PK, Engelman DT, Bradford WT. One-year results from the first US-based enhanced recovery after cardiac surgery (ERAS Cardiac) program. J Thorac Cardiovasc Surg. 2019 May;157(5):1881-1888. doi: 10.1016/j.jtcvs.2018.10.164. Epub 2018 Dec 8. PMID: 30665758.

Institute at Cleveland Clinic Florida.

Reduced narcotic use improves recovery process

regional anesthesia of the chest wall under ultrasound guidance.

Around-the-clock acetaminophen is used pre- and postoperatively unless contraindicated.

Traditionally, cardiac anesthesia was opioid-based and patients generally stayed in bed for two to three days postoperatively. ERAS

The specifics of multimodal analgesia may vary among patients, depending on different procedures, patient comorbidities and local resources.

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The ERAS Cardiac approach allows for earlier extubation and earlier mobilization of the patient after surgery, thereby also reducing the risk for blood clots and pulmonary complications. Average length of stay is shortened and complications are reduced, according to the ERAS® Cardiac Society.

Active participation from patient and loved ones is important before and after surgery Patients’ families and their caretakers must be active supporters and participants in the ERACS protocols in the pre- and postoperative periods to achieve optimal recovery for the patient. When patients are actively involved in pre-procedure optimization rather than waiting fearfully and anxiously for the operation itself, better postoperative outcomes result.

Future goals of ERAS Cardiac at Cleveland Clinic Florida at Weston will include optimizing patients’ nutrition, blood glucose and hemoglobin levels before surgery to achieve the best outcome. In addition, identifying patients who may be at highest risk of postoperative complications (e.g., frail patients and patients on a cognitive decline trajectory) will allow proper diagnosis and early intervention if necessary postoperatively.

The postoperative course is also important for faster recovery after cardiac surgery. Close coordination of care, with the ICU team focusing on proper hand-off to highly skilled nursing staff, makes a substantial difference in the patient’s recovery.

Our ERAS Cardiac patients are identified during hand-off and generally are extubated within four to six hours after surgery.

Proper selection and identification of patients for ERAS Cardiac protocol prior to surgery is important to allow time for health optimization. The involvement of patients, families, surgeons, anesthesiologists, intensivists, therapists and nursing staff is critical to successfully implementing ERAS Cardiac protocol.

Dr. Jamroz jamrozt@ccf.org

Cardiac anesthesiologists provide highly specialized care Due to an expansion in the number of cardiac procedures we offer (including growth in the heart transplant program and heart failure therapy, and many transfers from hospitals in the region), as well as advances in the field of cardiac surgery and structural interventional procedures, we felt it was important for Cleveland Clinic Weston Hospital to have a dedicated team of cardiac anesthesiologists.

From about 20 anesthesiologists we selected seven with formal cardiac fellowship training to create our team.

Weston Hospital’s cardiac anesthesiologists focus on technical excellence and fast clinical judgement in critical scenarios and have experience and clinical knowledge of mechanical circulatory support for the treatment of heart failure. They are board-certified in perioperative echocardiography to allow for a highly specialized monitoring method. Their expertise in perioperative echocardiography also is used for diagnosis to aid in surgeon decision-making.

The cardiac anesthesia team works together to reduce variability in outcomes by: • concentrating a higher volume of specialized surgeries to a dedicated group of fellowshiptrained professionals • allowing for a focused interest on research and advances in the specialized care of cardiac surgical patients • standardizing easily achievable perioperative practices across small teams • building operating room teams consisting of surgeons, nurses, perfusionists, anesthesiologists, assistants, technologists and technicians, because operating room performance depends on a team’s expertise and experience, as well as communication and teamwork • collaborating closely with critical care specialistsintensivists to provide for a smooth transition in care in the postoperative period

The goal of ERAS Cardiac is to promote healing and optimize recovery to allow patients to get back to their baseline levels faster postoperatively.

• establishing a specialized team to ensure that highly trained cardiac anesthesiologists are available year round, on weeknights, weekends, holidays and every day.

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A New Tool for the Non-Dilatable Lesion Shockwave Intravascular Lithotripsy Proves Beneficial in Treating Deep Calcified Coronary Lesions

By Stephen McIntyre, MD

Calcium is an integral component of atherosclerotic plaques, and the extent of coronary calcification correlates with success rates, complication rates, acute luminal gain and subsequent restenosis rates.

The pattern of plaque calcification directly affects the complexity, risk and outcome of percutaneous procedures in both the coronary and peripheral interventions. The deposition of calcium in plaque is not uniform. In the vessel wall, it may be eccentric or circumferential, luminal or deep in the wall, relatively focal or quite diffuse. The calcification pattern directly affects the radial compliance, vessel flexibility and luminal resistance to the passage of interventional equipment.

Several devices and strategies have been developed to address coronary calcification, including rotational atherectomy, cutting and sculpting balloons and off-label use of lasercontrast detonation. These devices are sufficient to treat many patterns of coronary calcification but are limited in their efficacy at treating deep circumferential calcium. It is not uncommon to encounter

“non-dilatable” lesions that fail to expand within rated balloon burst pressures despite atherectomy. Overpressurizing or oversizing balloons, aggressive repeat atherectomy or offlabel laser contrast detonation may be successful but carry substantial risk of perforation, rupture, dissection or abrupt vessel closure. The alternative strategy of deploying a stent within the under-expanded segment is a frequent cause of in-stent restenosis and target lesion failure.

Shockwave intravascular lithotripsy (IVL) was recently approved in the United States for use in the coronary circulation. IVL has been used in the peripheral circulation in the United States for several years (the peripheral lithotripsy device was approved by the FDA in 2016) and coronary interventions have been available and approved in Europe since 2018.

IVL is indicated for de novo treatment of severely calcified lesions in the coronary arteries. It is complementary to current atheroablative technologies such as orbital (CSI Technologies) and rotational (Rotablator™, Boston Scientific) atherectomy.

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These rotational ablative technologies are irreplaceable for treating long, diffusely calcified lesions with mixed superficial and deep calcium.

They play a crucial role in napkin ring lesions where other devices fail to cross.

Compared to vessel preparation with balloon angioplasty, however, rotational/orbital atherectomy devices have several limitations.

Foremost is a steep learning curve to proficiency and comfort in the use of atherectomy. The devices are cumbersome to set up and require the operator and assisting technologist to be facile with long wire exchanges.

Catastrophic complications such as coronary perforation or no reflow are more common than with balloon angioplasty. And there are unique complications such as burr stall, burr entrapment and wire transection that can suddenly occur with catastrophic consequences.

Concern for these factors can foster a bias towards underutilization of atheroablative techniques commonly referred to as “Rotablator regret.”

IVL is attractive for its ease of use and safety profile. It preps like a standard balloon and delivers like a stent. The coronary balloons are 12 mm in length and charged to deliver eight 10-second treatments. The IVL balloons are inflated to four atmospheres with a standard saline contrast mixture. Each coronary balloon has two emitters that are electrified and cause instantaneous vaporization of a saline contrast solution.

This results in the rapid expansion and collapse of a bubble, sending sonic pressure waves through the tissue with an effective pressure of 50 atmospheres.

Each lesion treated requires at least one — and often all — the charges in the balloon. Shockwave lithotripsy balloons are, therefore, most effective at fracturing superficial and deep calcified lesions in limited length segments. IVL alters vessel compliance, allowing dilation of lesions at low pressure.

IVL is not approved for use within stents because of concerns for damage to the polymer coat and alteration in drug delivery of current drug-eluting stents.

This new technology is more expensive then atheroablative technologies or traditional balloons but is reimbursed as of July for outpatient procedures and will be for inpatient in November via a new technology add-on payment (NTAP).

IVL is clearly a breakthrough technology allowing interventional cardiologists to successfully treat, with a very safe and easy-to-use technology, coronary lesions that were previously untreatable. Since the device became available in our lab, we have found it to be an extremely useful addition to our armamentarium – within the first two weeks of having it we used it in about a dozen cases.

Severe restenosis of an under-expanded stent within a non-dilatable plaque.

Challenging case requires IVL We recently treated an 84-year-old male at Cleveland Clinic Martin Health with prior bypass surgery who underwent stenting of the circumflex artery in February 2020. On that occasion, he underwent eight orbital atherectomy runs followed by coronary stenting and post dilatation to high pressure with an NC balloon.

Despite the extensive pretreatment, he was noted to have a residual 50-percent napkin ring lesion post stent deployment.

Eight months later, due to recurrent symptoms in the setting of a positive myocardial perfusion study, he returned to the lab and was found to have a focal 95 percent in-stent restenosis in the circumflex artery. It was treated with multiple high-pressure balloon inflations that failed to expand the napkin ring lesion. Two months later he returned to the lab in the setting of a NSTEMI and underwent rotational atherectomy within the circumflex artery followed by high-pressure balloon angioplasty, which again restored a lumen but was unsuccessful in expanding the napkin ring lesion.

The patient returned to the catheterization lab after the delivery of our first coronary IVL balloon and underwent successful lesion expansion at eight atmospheres after delivery of 10 lithotripsy treatments from a single balloon.

Dr. McIntyre mcintys@ccf.org IVL balloon expanded at eight atmospheres following application of lithotripsy.

Final angiogram demonstrates complete stent expansion within the formerly non-dilatable plaque.

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FEATURE ARTICLE

Cardiac Amyloidosis Center of Excellence Cleveland Clinic Florida’s Comprehensive Diagnostic and Management Strategies for an Increasingly Prevalent Cause of Heart Failure

By David Wolinsky, MD

More than 6 million Americans currently suffer from congestive heart failure, and about 500,000 new cases are David Wolinsky, MD Director of the Cardiac diagnosed each Amyloidosis Center of Excellence at Cleveland year. Despite Clinic Florida advances in therapy, five-year mortality ranges from 40 to 50 percent. Though hypertension and coronary artery disease remain the primary cause, cardiac amyloidosis has been found to be the etiology with increasing frequency.

Amyloidosis is a multisystem disorder caused by the buildup of abnormal proteins in multiple organs.

These proteins become misfolded, then bind together to form fibrils that circulate in the bloodstream and are subsequently deposited into the heart. As a result, cardiac failure, serious arrhythmias and cardiac death can occur without early recognition and specific therapies.

In the past three years, Cleveland Clinic Florida has become a leader in early recognition, diagnosis and treatment of this disease.

Multiple proteins are responsible for the development of amyloidosis.

One form is AL amyloidosis, a rapidly progressive bone marrow disorder marked by the production of abnormal light-chain proteins.

Patients with this form of the disease require early diagnosis and referral to a specialized hematology program for treatment, which includes chemotherapy and stem cell transplant. Initial cardiac treatment is supportive, but these patients may ultimately come for evaluation for cardiac transplant.

Finding cardiac amyloidosis — awareness among practitioners is key ATTR amyloidosis, the most common form of the disease, is caused by an abnormal form of a normally occurring protein called transthyretin, which is produced in the liver. The predominant form of the disease – called ATTR-wt – occurs when transthyretin undergoes transformation, unfolding and cardiac deposition. This disease often presents with progressive congestive heart failure and atrial fibrillation in men over age 70. A smaller percentage of these patients

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have the hereditary (hATTRv) form of the disease. In these cases, hereditary amyloidosis is present at a slightly younger age and is common in African Americans. Three to four percent of African Americans carry the gene for the v122i mutation, but not all carriers will develop the disease.

Deposition of amyloid fibrils leads to marked myocardial hypertrophy of both the left and right ventricles.

Though ejection fraction is preserved, diastolic dysfunction leads to HFpEF or heart failure with preserved ejection fraction with shortness of breath. Additionally, because of the small, stiff left ventricular cavity, cardiac output is reduced and exercise capacity becomes progressively decreased.

As the disease progresses ventricular function may worsen and ejection fraction falls, leading to worsening heart failure and worsening quality of life.

Neuropathic symptoms may also be prominent. Non-cardiac symptoms such as bilateral carpal tunnel syndrome, spinal stenosis, peripheral neuropathy, and orthostatic hypotension significantly impair

quality of life. These findings are often present more than ten years prior to the onset of cardiomyopathy but may be attributed to other diseases, delaying the diagnosis of amyloidosis.

Unfortunately, awareness of the disease has been limited and opportunities for early recognition have been missed. Increased awareness leads to early testing, diagnosis and successful treatment of a disease once believed to be uniformly deadly.

Advanced testing to determine presence and type of amyloidosis Most patients with amyloidosis have seen multiple cardiologists before an accurate diagnosis is made. They often have been hospitalized with signs of heart failure and fluid overload only to be readmitted shortly after discharge with recurrent symptoms. Recurrent atrial fibrillation refractory to therapy is common, as is the need for a permanent pacemaker. Cleveland Clinic Florida has developed a comprehensive noninvasive imaging protocol for amyloidosis evaluation.

Echocardiography is an ideal screening test, demonstrating not only left ventricular hypertrophy but also typical abnormalities of the atria and valves. Most important is the performance of strain imaging as part of the standard echo protocol.

This characterizes myocardial dynamics and, in amyloidosis, a characteristic pattern of “apical-sparing” can be noted.

Cardiac MRI is also a useful tool as characteristic patterns of late gadolinium enhancement and the presence of increased extracellular volume may be seen.

The availability of diagnostic radionuclide imaging using bone-avid scintigraphy has dramatically increased the ability to noninvasively confirm the diagnosis of ATTR amyloidosis. Technetium pyrophosphate imaging (PYPscanning), when done properly, can accurately diagnose ATTR amyloidosis with very high sensitivity (95 to 100 percent) and specificity (85 to 100 percent). As PastPresident of the American Society of Nuclear Cardiology and Section Head of Nuclear Cardiology at Cleveland Clinic Florida, I have extensive experience defining proper protocols and interpreting these scans, and am often consulted by many nuclear cardiology labs to improve their lab techniques or provide second opinions on scan interpretation.

Additionally, I have advocated for SPECT acquisition and review for all patients. In all cases, clonal blood testing should be carried out to rule out AL amyloidosis, and simple genetic testing is done to distinguish between hereditary and wild-type (non-hereditary) amyloidosis.

that includes medical history, physical exam, review of outside records and images, and discussion of further diagnostic testing and treatment plans. Traditional heart failure therapies such as ACE-I, ARB, and ARNI are often detrimental to amyloid patients, and the medical regimen is individualized at the first visit. Heart rhythm management options such as AV node ablation or cardiac resynchronization therapy are discussed, and referral to our electrophysiology team is made if needed.

Referrals to specialists for management of peripheral neuropathy, autonomic dysfunction, chronic spine disease, chronic kidney disease, weight loss, constipation and diarrhea are made at the first visit. Hematology referrals

Scintigraphy has reduced the need for myocardial biopsy by 80 to 90 percent; however, when needed, this can easily be carried out at our facility. Tissue is stained with Congo Red or Thioflavin-S for evidence of typical birefringent fibrils. Positive tissue samples are further analyzed at Cleveland Clinic’s main campus in Cleveland for mass spectrometry.

Accurate diagnosis leads to more successful treatment Each new patient referred to our Amyloid Center undergoes an extensive initial evaluation by myself and Amy Deramo, AGAC NP-BC,

Multiple SPECT CT projections clearly demonstrating technetium pyrophosphate consistent with ATTR cardiac amyloidosis.

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are made when there is a question of the presence of AL disease.

Samples for genetic testing are obtained (buccal swab) and sent out if not already done. Because of the complexity of the initial evaluation, all patients have the assistance of Nurse Navigator Andrea Kay, RN, throughout the process.

Beyond instituting traditional heart failure therapy, patients are assessed for disease-modifying therapy at their initial visit. Transthyretin is a normally existing tetramer protein that, in amyloidosis, becomes unstable and separates into four monomers. Disease-modifying therapy is focused on either blocking the production of the unstable proteins (silencers) or preventing dissociation of the tetramer (stabilizer).

For wild-type cardiomyopathy, a stabilizer called tafamidis is often used. Cleveland Clinic Weston Hospital’s pharmacy works with patients to obtain insurance coverage for the drug, a capsule that is taken once a day and has minimal side effects. Long-term use of the drug is associated with improved survival at 18 months and 30 months, and less clinical deterioration at six months, compared to patients not on the drug. For patients with hereditary ATTR who have peripheral neuropathy with or without cardiac involvement, several silencers are approved to block the mRNA from producing transthyretin protein.

Patients with AL amyloidosis may receive care that includes stateof-the-art treatments and access to clinical research protocols through the hematoloy department at Cleveland Clinic Florida at Weston.

Many patients with advanced AL and hereditary ATTR disease continue to deteriorate despite aggressive therapy. Appropriate patients are evaluated for organ transplant including heart, liver, combined heart-liver and combined heartkidney transplant. Our transplant team is experienced in carrying out en-block heart-liver transplantation, with markedly reduced surgical times and outcomes equivalent to singleorgan transplantation.

Future management and research We are involved in clinical research trials and the development of clinical protocols for patients who are not candidates for traditional treatment options, who cannot afford treatment options or who have failed standard therapy. Additionally, we are studying populations who appear to be at risk for cardiac amyloidosis, such as those patients with aortic stenosis undergoing TAVR and SAVR. Most cardiac amyloidosis studies involve patients with an average age of 70 to 75. South Florida is unique in its elderly population, and we are studying the natural history and response to disease-modifying therapy in the octogenarian population. We are working on multiple educational initiatives to increase the clinical recognition and clinical suspicion for cardiac amyloidosis. It is hoped that earlier

Full-body planar scan demonstrating no myocardial uptake of Tc-Pyp. For totally normal planar studies, SPECT imaging may not be needed. This scan also identifies artifacts that could interfere with accurate scan interpretation.

disease recognition combined with the development of new treatment options will improve quality of life and survival in these patients.

Almost 200 patients with cardiac AL or ATTR amyloidosis are being treated at the Cardiac Amyloid Center of Excellence at Cleveland Clinic Florida. We are learning more about this disease each day and we continue to help both our patients and our referring physicians in managing this complex disease.

Dr. Wolinsky wolinsd@ccf.org To refer a patient to the cardiac amyloid clinic, please call 954.659.5290.

Echocardiogram demonstrating left ventricular hypertrophy and typical bright speckled appearance. Though very suggestive of ATTR cardiac amyloidosis, it is not sufficient. Only myocardial biopsy or positive pyrophosphate scan may confirm the diagnosis.

14 CARDIAC CARE - SUMMER/FALL 2021

Patient in his 90s doing well three years after diagnosis of cardiac amyloidosis Robert K. Lifton had gone through life always expecting he would live a very long time.

He counted on it being in his genes – his father had lived to 100 and his mother to 94.

But during a vacation cruise with his wife, Loretta, ending in Stockholm three years ago, Mr. Lifton, who was 90 at the time, got some surprising news. On the last day he started to feel quite ill and ran a high fever. The ship’s doctor suggested he immediately go to a local hospital, where the emergency room doctor informed him that he had heart failure.

Robert K. Lifton

After about four days of tests and treatment in the hospital, he returned home to South Florida where his friend and cardiologist expressed further concern about his condition and suggested he make an appointment with David Wolinsky, MD, Director of the Amyloidosis Center of Excellence at Cleveland Clinic Florida. After a nuclear test and further evaluation, Dr. Wolinsky informed him that he had non-hereditary ATTR, the most common form of cardiac amyloidosis.

“I was in shock because I had never heard of amyloidosis and no one I knew had ever heard of it,” Mr. Lifton says. “I was also frightened because I had an esoteric heart condition that I thought no one knew enough about.”

I was in shock because I had never heard of amyloidosis and no one I knew had ever heard of it. I was also frightened because I had an esoteric heart condition that I thought no one knew enough about.

- Robert K. Lifton

Under Dr. Wolinsky’s care Mr. Lifton stopped his blood pressure medication and began taking green tea extract. Dr. Wolinsky also encouraged him to continue regular strenuous exercise, even though that sounded counterintuitive given his heart condition.

He also started regularly taking tafamidis, a drug that stabilizes the transthyretin protein to prevent further progression of amyloidosis.

“Dr. Wolinsky’s regimen is helping me hold my own. Recent tests indicate that there has been no further deterioration,” Mr. Lifton notes. “And for that I am grateful.” He feels fortunate that his symptoms are primarily related to water retention – not the neuropathy, carpal tunnel, digestive issues or other symptoms that frequently affect people with this condition.

“For my age I still live a pretty full life,” says Mr. Lifton, who has had a varied and successful career as an entrepreneur, community leader and international diplomat, with a broad range of books and articles to his credit. “I feel very fortunate to have learned about my heart condition early and to have a doctor as knowledgeable as Dr. Wolinsky, a leader in his field, provide my care. He is always responsive and helpful.”

Though he walks more slowly and often with a cane, Mr. Lifton still is able to golf occasionally. He is also able to spend the summer months in New York and spend time with his two daughters and four grandchildren.

In the process of learning about the disease, Mr. Lifton recognized that there was much more to be discovered about cardiac amyloidosis and that more people both in and out of the medical community needed to be educated about the disease.

He recruited friends to join his wife and him in providing critical funding to help support Dr. Wolinsky’s research and improve the diagnosis and management of cardiac amyloidosis.

Because of Mr. and Mrs. Lifton’s commitment to the research and education inherent in Cleveland Clinic’s mission, Dr. Wolinsky and his team can continue to impact patient outcomes and provide a better quality of life for cardiology patients.

CLEVELAND CLINIC FLORIDA 15

The Ride of a Lifetime Heart Transplant Gets Physician Cyclist Back on the Trails After Years of Progressive Heart Failure

Jaime Rodriguez, MD, has dedicated his career to taking care of others.

The 64-year-old physician is devoted to his work as a maternalfetal medicine specialist at another healthcare system in South Florida.

But when he was the one who needed help, his colleagues in medicine were there for him.

In 2007 Dr. Rodriguez, a former competitive cyclist, was out on one of his regular early morning bike rides when he went into cardiac arrest and collapsed on the road. He required cardiac resuscitation on the scene and was rushed to Cleveland Clinic Weston Hospital where his cardiologist, Howard Bush, MD, performed a coronary catheterization that revealed a blocking of the stents he had received six years earlier. An intra-aortic balloon pump (IABP) was implanted, and he was rushed to the operating room where he underwent quadruple coronary bypass surgery.

He had suffered a massive heart attack on that ride, which severely damaged his heart.

Dr. Rodriguez, who was 50 at the time, had been dealing with coronary artery disease since he was 43 years old. “I had gone back to cycling and riding my bike at a competitive level to improve my heart health,” he says. But his predisposition to heart disease was strong – both his father and his grandfather had died from it, at 52 and 53 years old, respectively.

Dr. Bush says that when he first met Dr. Rodriguez he was “immediately struck by his commitment to his family and his passion for life.”

“Following his cardiac event in 2007,” he says, “Dr. Rodriguez made it very clear that he was willing to do whatever was necessary to get back to the best health possible and return to all the things that were important to him.”

failure management under the care of Viviana Navas, MD.

By May of 2020, Dr. Rodriguez had been dealing with worsening episodes of arrythmia and had been hospitalized frequently over six months until one night, which he described as a “nightmare,” when he had eight episodes of arrythmia. He’d had enough. His wife called 911 and he was taken to Cleveland Clinic Florida in Weston.

My prognosis was that the damage would worsen with age. I had a feeling I was going to end up needing a transplant – it was always in the back of my mind.

- Jaime Rodriguez, MD An increasingly difficult path to navigate After the biking incident, Dr. Rodriguez’s health remained stable for several years. The damage to his heart, however, slowly progressed to heart failure and malignant arrhythmia, requiring the implantation of a cardioverterdefibrillator and advanced heart

16 CARDIAC CARE - SUMMER/FALL 2021

Dr. Rodriguez’s heart muscle had deteriorated to the point where a transplant was the only solution.

He was admitted to the ICU, where he was intubated for a few days and then required an IABP support to stabilize his condition while he waited for a donor heart that would be compatible for him.

“I had suffered so much that I was welcoming the idea by then,” Dr. Rodriguez says of overcoming his fear of the transplant. “I knew I had no choice – it was this or I wouldn’t make it.”

A new heart makes all the difference A suitable donor heart become available 10 days after he was admitted to the ICU. “It’s hard to describe how it felt to get that news,” Dr. Rodriguez says. “I realized how lucky I was.”

Nicolas Brozzi, MD, and Cedric Sheffield, MD, performed a complex cardiac reoperation to replace his worn-out native heart with a healthy

donor heart. Dr. Rodriguez’s recovery was gradual, but steady, and he was discharged from the hospital within two weeks.

“He was determined to get through this operation and move on with life, back to his family and his work,” says Dr. Brozzi.

On May 23, Dr. Rodriguez celebrated his milestone one-year anniversary of the transplant. It was a year filled with medications, biopsies and follow-up visits with his care team.

But he knows the first year after transplant is the most critical and it is all worth it. He is back to working full-time, walking, running and cycling. He says exercising is easier

now than before and he can do at least as much as, if not more than anyone else his age.

Dr. Rodriguez has become very fond of his care team at Cleveland Clinic Florida in Weston and looks forward to seeing them at his follow-ups.

“The cardiac transplant team provides very personalized care,” he says. “They make you feel special. I am very grateful to the organization and the transplant care doctors – they have become my friends and partners in this ride of a lifetime.”

Dr. Rodriguez (center) recently enjoyed a 10-mile bike ride with Dr. Brozzi (left) and Dr. Bush (right). “To see this family man, health professional and accomplished cyclist back to doing all the things that are so important to him - it is another reminder of why I love what I do and why I am so grateful to be part of the Cleveland Clinic Florida Heart, Thoracic and Vascular Institute,” says Dr. Bush.

The cardiac transplant team provides very personalized care. They make you feel special. I am very grateful to the organization and the transplant care doctors – they have become my friends and partners in this ride of a lifetime.

- Jaime Rodriguez, MD CLEVELAND CLINIC FLORIDA 17

Long-Term Support with a Left Ventricular Assist Device as an Alternative to Cardiac Transplantation

By Mauricio Velez, MD

Cardiac transplantation is a treatment option for heart failure that is subject to strict eligibility criteria resulting in limited access for older patients with common medical co-morbidities such as obesity and diabetes. Cardiac transplant volume has increased in recent years but remains at approximately 3,000 annually in the United States. Extraordinary advances in left ventricular assist device (LVAD) care during the last 25 years offer an opportunity to provide a life-saving treatment to many heart failure patients who are not eligible for cardiac transplantation.

Clinical outcomes for LVAD therapy have improved dramatically over time. Based on the most recent report of the Interagency Registry of Mechanically Assisted Circulatory Support (INTERMACS), mean one-year post-LVAD survival is 83 percent. Recent reports of long-term LVAD support have suggested that survival greater than 50 percent at seven years with a low incidence of disabling complications is possible.

Personally, we have cared for a patient who was supported by the same LVAD for more than 10 years.

Our own outcomes at Cleveland Clinic Florida exceed national statistics with one-year survival after LVAD surgery of more than 91 percent (See LVAD Outcomes).

This performance rivals one-year survival after cardiac transplantation, which is currently estimated at 92 percent. The survival benefit with LVAD support is accompanied by improvements in quality of life and functional status that are noticeable as early as three to six months after implantation. A significant majority of patients experience symptom improvement from New York Heart Association (NYHA) class IIIb-IV (severe symptoms) to NYHA class I-II (mild to no symptoms) after LVAD surgery. Rehospitalization after LVAD remains a significant challenge, with a majority of patients requiring a hospital admission within one year of surgery. Short- and long-term survival are likely to inch closer to that of cardiac transplantation as clinical care and LVAD technology continue to improve.

Florida is the third largest state in the country and is home to 20 million people, based on recent U.S. Census figures. It is estimated that

18 CARDIAC CARE - SUMMER/FALL 2021

2.6 percent of the population in the United States suffers from heart failure, translating to approximately 520,000 people in the state of Florida Population 20 million Suffering from Heart Failure (2.6%) 520,000 Heart Failure with Reduced Ejection Fraction (50%) 260,000 Severe Heart Failure Symptoms (NYHA class III-IV) (20%) 52,000 80 years or younger who may benefit from advanced HF therapies (50%) 26,000

Only 250 transplants per year in Florida It is estimated that about 26,000 Floridians suffer from advanced heart failure and may benefit from cardiac transplantation or long-term support with a left ventricular assist device.

LVAD Outcomes 30-Day Survival 100% 90% 80%

100.0%

94.3% INTERMACS

Cleveland Clinic Florida

100% of patients survive to 1 month at Cleveland Clinic Florida compared to 94.3% nationally

1-Year Survival 100% 90% 80%

91.7%

84.1% INTERMACS

Cleveland Clinic Florida

91.7% of patients survive to 1 year at Cleveland Clinic Florida compared to 84.1% nationally

Stroke at 1 Year 14.5%

20.0% 10.0% 0.0%

0.0% INTERMACS

Cleveland Clinic Florida

0% of patients experience a stroke at 1 year, which, is lower than the national average

Device Failure at 1 Year 4.3%

5.0%

0.0%

0.0%

INTERMACS

Cleveland Clinic Florida

0% of patients at Cleveland Clinic Florida have a pump failure at 1 year, which is lower than the national average

Bleeding 20.0%

16.2% 8.5% 8.3%

10.0% 0.0%

Florida. Of these half-million patients, about 50 percent suffer from heart failure with reduced ejection fraction, with 20 percent of them, an estimated 52,000 people, living with severe NYHA class IIIb-IV heart failure symptoms. About half of these patients, or about 26,000 people in the state of Florida, are younger than 80 years, and a substantial number of them may benefit from advanced heart failure therapies, including cardiac transplant and mechanical circulatory support. Yet only 288 heart transplants were performed in Florida in 2020, based on data from the Scientific Registry of Transplant Recipients (SRTR) and the United Network for Organ Sharing (UNOS). Of these, only 63 (22 percent) were performed in patients older than 65 years. These contrasting figures highlight the fact that cardiac transplantation alone cannot meet the care needs of the large population of advanced heart failure patients in our region. Long-term LVAD therapy can provide a life-saving alternative to a substantial number of patients. Unfortunately, LVAD surgery volume in Florida as a whole has been chronically low, revealing a great need for access to this treatment option for our patients with severe heart failure symptoms.

Our primary goal at Cleveland Clinic Florida is to offer long-term LVAD therapy based on a multidisciplinary team approach focused on shared decision-making, quality and safety to meet our patient expectations.

Early referral improves individual patient outcomes and we strongly encourage it. Therefore, our referring providers are a key component of our program as the main access point to long-term LVAD therapy for most patients. We strive to provide highly competent consultation at all times and we cultivate relationships in which our physician partners in the community trust us to continue the care of their patients with heart failure. We communicate frequently to update our referring providers on major events pertaining to their patients. Our LVAD program at Cleveland Clinic Florida is committed to maintaining the highest quality world-class care to bridge the care gap that exists in advanced heart failure therapies in our communities locally and statewide.

0.0% <3 Mo

>3 Mo

At Cleveland Clinic Florida, there is 0% bleeding within the first 3 months, which increases to 8.3% after 3 months.

Dr. Velez velezm2@ccf.org

Clinical outcomes for patient supported with

INTERMACS Cleveland Clinic at Florida long-term left ventricular assist devices Cleveland Clinic Florida, 2019-2020 implants.

- 12/31/2020 The data for this Based research on wereimplants provided by7/1/2019 The Society of Thoracic Surgeons’ Interagency Registry for Mechanically Assisted Circulatory Support (Intermacs) Database - INTERMACS Data Quality Report (2021-07)

CLEVELAND CLINIC FLORIDA 19

Growing Conduction System Pacing Program at Cleveland Clinic Florida An Alternative that Preserves Normal Physiology

By Jose L. Baez-Escudero, MD, FACC, FHRS

When patients develop conduction abnormalities and bradycardia, they often require implantation of a pacemaker. Traditionally, this involves anatomical placement of a transvenous pacing lead at the right ventricular (RV) apex, with a proven benefit of restoring the rhythm. However, this conventional and widely used technique can have deleterious effects on cardiac function. In fact, long-term RV apical pacing may cause or worsen heart failure in some patients by causing dyssynchronous ventricular activation and has been associated with increased risk of atrial fibrillation, hospitalization for heart failure, pacing-induced cardiomyopathy and death.

Conduction System Pacing (CSP) is a physiological form of pacing that has been shown to improve clinical outcomes compared with conventional RV pacing. It involves careful placement of a pacing lead in the septum of the RV, aiming to stimulate the His-Purkinje network directly (either the His Bundle, or the Left Bundle Branch). This results in synchronized ventricular activation, which translates into improved clinical outcomes. CSP can potentially correct bundle branch block in patients with proximal bundle branch

disease. Moreover, early data suggest that CSP can be used as cardiac resynchronization therapy (CRT), being at least as effective as biventricular CRT (among patients with a low left ventricular ejection fraction).

Approximately four years ago, we started a CSP program at Cleveland Clinic Weston Hospital. The program was initially conceived by experienced implanter Sergio Pinski, MD. Since then, the section of electrophysiology has embraced the technique and quickly has become a regional pioneer for CSP, having performed more than 450 physiologic pacing implants, with a greater than 95 percent success rate.

“CSP offers an elegant solution to potentially avoid pacing-induced deterioration in cardiac function.

Since activation occurs via the normal conduction system, it does not result in ventricular dyssynchrony. It requires the implanting physician to have a thorough understanding of the electrophysiology of normal cardiac conduction and the anatomy of this region in the RV septum,” Dr. Pinski says.

Compared with right ventricular pacing, CSP can dramatically shorten QRS duration and restore normal

20 CARDIAC CARE - SUMMER/FALL 2021

intrinsic activation patterns in some patients with ventricular conduction delays. This has allowed for CSP to be used in our center as a rescue strategy in cases where biventricular pacing failed, or was suboptimal. It has the advantage that it does not require the use of contrast and can often be performed more quickly than left ventricular lead placement.

It has also become the standard type of pacing in patients with heart failure after AV node ablation for uncontrolled atrial fibrillation.

EKG on His-Bundle pacing. Ventricular pacing stimulus artifact followed by a narrow QRS complex identical to the intrinsic QRS indicates selective capture of the bundle His.

Dr. Baez-Escudero baezj@ccf.org

Imaging Coronary Anomalies

By David Lopez, MD Coronary artery anomalies (CAA) constitute a heterogeneous group of coronary artery congenital abnormalities with benign and pathological implications. CAA may present in the form of anomalous coronary origins, anomalous coronary course, abnormal intrinsic coronary anatomy, or anomalous coronary artery termination. Many CAA are benign, such as absence of the left main trunk with separate left anterior descending and circumflex arteries ostia. Others carry increased risk of morbidity and mortality, such as an anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) with an interarterial course.

CAA are typically identified in the evaluation of atypical chest pain, dyspnea, or syncope. Other times, these abnormalities are incidentally found on preoperative angiography or computed tomography angiography.

Once identified, cardiologists need to determine if the CAA is the source of the patient’s symptoms, a pathological entity that will impact the patient’s prognosis or a curious benign variation. Adequate management of CAA relies on multimodality assessment of the coronary anatomy and physiology to identify anatomical features that portend increased risk of sudden death and characterize potential pathophysiological implications such as myocardial ischemia.

The most common clinically relevant CAA include ACAOS with an interaterial course and coronary fistulae. Although these are well recognized entities, management recommendations are always challenging because of a paucity of data from randomized studies. As such, a team approach in which

clinical cardiologists, noninvasive cardiovascular imagers, invasive cardiologists and cardiothoracic surgeons collaborate is recommended in order to make treatment recommendations for each patient.

The goals of the evaluation are to accurately define the coronary anatomy from vessel origin to termination and to determine any potential pathophysiological implications. Is the abnormality contributing to myocardial ischemia?

Are there high-risk features of sudden cardiac death? Is there volume overload? Does this abnormality contribute to the patient’s symptoms?

The noninvasive evaluation of CAA usually will include multiple imaging modalities. ECG-gated coronary computed tomography (CCTA) allows us to accurately define the coronary anatomy from origin to termination.

With the use of this technique, we can map the course of ACAOS (Figure 1), identify the myocardial territory at risk, define luminal patency and gauge the size and course of coronary fistulae (Figure 2). Exercise stress myocardial perfusion imaging (MPI) is ordinarily used to identify presence of myocardial ischemia. Alternatively exercise stress echocardiography can be utilized. Noninvasive testing is complemented with invasive coronary angiography and intravascular ultrasound (IVUS). With the use of IVUS we can measure luminal stenosis and visualize the dynamic range of the vessel area throughout the cardiac cycle. In the case of coronary artery fistula, the shunt fraction can be determined with the use of cardiovascular magnetic resonance imaging or with a complete invasive hemodynamic study. Once the CAA is fully characterized, the

team can make an informed clinical recommendation to either treat medically, perform percutaneous intervention or offer surgical repair.

Dr. Lopez lopezd@ccf.org

*

Figure 1: Non-dominant anomalous right coronary artery from the left sinus of Valsalva (*) with an interarterial course. (Pulmonary artery removed in this 3D reconstruction.)

A

*

Figure 2: 3D reconstruction (A) of a coronary to pulmonary artery fistula via a Vieussens arterial ring (red) with communication to the right (RCA) and left anterior descending (LAD) coronary arteries. Coronal multiplanar reconstruction (B) illustrates fistula jet (*) draining into the pulmonary artery.

CLEVELAND CLINIC FLORIDA 21

New Staff Cleveland Clinic Florida Heart, Vascular and Thoracic Institute Welcomes the Following New Staff Members Raghavendra Makam, MD Dr. Makam recently joined the Cardiovascular Medicine Department at Cleveland Clinic Indian River Hospital. He completed fellowship training in advanced heart failure and transplant cardiology at University of Miami/Jackson Memorial Hospital in 2021, as well as fellowship training in cardiology in 2020 at the University of Massachusetts Medical Center and in cardiac critical care in 2017 at Johns Hopkins Hospital.

Dr. Makam completed his medical education at Bangalore Medical College and Research Institute in Bangalore, India in 2004. In 2006, Dr. Makam received an MPH in biostatistics, healthcare leadership and management from the Johns Hopkins School of Public Health as well as an MBA in 2011 in medical service management from the John Hopkins Carey School of Business. In 2011, he completed a residency in internal medicine at Berkshire Medical Center at the University of Massachusetts. He is boardcertified in cardiovascular medicine, nuclear cardiology and internal medicine.

To reach Dr. Makam, call 772.778.8687

David Snipelisky, MD Dr. Snipelisky is a cardiologist who recently joined the staff at Cleveland Clinic Weston Hospital. He received his bachelor’s degree from the University of Florida in Gainesville.

He received his medical degree from Florida State University College of Medicine in Tallahassee, and completed his internal medicine residency at the Mayo Clinic in Jacksonville, Fla. Dr. Snipelisky completed a cardiology fellowship at the Mayo Clinic in Rochester, Minn. He was awarded the Mayo Brothers Distinguished Fellowship Award – the most prestigious award given to a trainee at the Mayo Clinic.

Dr. Snipelisky then completed an advanced heart failure and transplant cardiology subspecialty fellowship at the Brigham and Women’s Hospital/Harvard Medical School in Boston. He has authored more than 50 peer-reviewed publications and has volunteered internationally on several mission trips.

Dr. Snipelisky is board-certified in internal medicine, echocardiography, cardiovascular disease, and advanced heart failure and transplant cardiology. His clinical interests include advanced heart failure, critical care cardiology, hypertrophic and genetic cardiomyopathies, and mechanical circulatory support.

To reach Dr. Snipelisky, call 954.659.5290

22 CARDIAC CARE - SUMMER/FALL 2021

Jason Benjamin Wheeler, MD, MSCR, MS, CCRE Dr. Wheeler recently joined the Department of Vascular Medicine at Cleveland Clinic Weston Hospital. Dr. Wheeler was born and raised in the South, completing his undergraduate studies in biology, chemistry and medical humanities at the University of South Carolina Honors College. He graduated from the Medical University of South Carolina in 2011 with a doctorate in medicine and a master’s degree in clinical and translational research. Afterward, Dr. Wheeler continued to pursue his interest in academic research at the Medical University of South Carolina in the College of Graduate Studies, completing a master’s degree in laboratory biomedical sciences and a fellowship in clinical and translational research ethics in 2016. He then completed his internal medicine residency and vascular medicine fellowship training at Cleveland Clinic in Ohio.

Dr. Wheeler has written multiple scientific journal articles and book chapters on his basic and translational research in vascular physiology. He is board-certified in internal medicine and vascular medicine and is a certified Registered Physician in Vascular Interpretation. His clinical interests include pulmonary embolism, DVT, anticoagulation management, cancer-associated thrombosis, COVID-related thrombosis, immune-related thrombotic thrombocytopenia, fibromuscular dysplasia, aortic aneurysms, lymphedema, lipedema, obesity, peripheral arterial disease, carotid stenosis, hypercholesterolemia, diabetes, varicose veins, and peripheral venous interventions. Dr. Wheeler is also an avid teacher, having been recognized by multiple societies for patient and student education as a clinical instructor with the Case Western Reserve University School of Medicine in Cleveland.

To reach Dr. Wheeler, call 954.659.5230

To refer a patient to one of our Heart, Vascular and Thoracic Institute specialists, please call 877.463.2010

#1 HOSPITAL IN SOUTH FLORIDA 4 YEARS IN A ROW Cleveland Clinic Florida’s Weston Hospital has once again earned the top spot as the #1 hospital in the Miami-Fort Lauderdale metro area for 2021-2022, according to the newly released annual ranking of Best Hospitals by U.S. News & World Report. It is the fourth consecutive year Cleveland Clinic Weston Hospital has earned the top ranking, the only hospital to be ranked #1 for four straight years in South Florida.

HIGH PERFORMING IN CARDIOLOGY AND HEART SURGERY Cleveland Clinic Florida’s regional hospitals were recognized as high performing for the following cardiac conditions and procedures:

Cleveland Clinic Florida

Cleveland Clinic Florida

Cleveland Clinic Florida

Cleveland Clinic Florida Cleveland Clinic Indian River Hospital

Cleveland Clinic Florida Cleveland Clinic Indian River Hospital

Cleveland Clinic Martin Health

Cleveland Clinic Martin Health

CLEVELAND CLINIC FLORIDA 23

CLEVELAND CLINIC FLORIDA LOCATIONS

Heart, Vascular and Thoracic Institute at Cleveland Clinic Florida

REGIONAL HOSPITAL

Patients from across the United States, Latin America and the Caribbean turn to Cleveland Clinic Florida’s Heart, Vascular and Thoracic Institute for life-saving treatment options. Physicians are subspecialty trained in a number of areas and provide compassionate heart care that is second to none.

Departments & Centers

INDIAN RIVER HOSPITAL

Cardiology Cardiac Amyloidosis Cardiac and Thoracic Surgery Cardiac Electrophysiology and Pacing Cardiac Imaging Cardio-Oncology Heart Transplant and Mechanical Circulatory Support Hypertrophic Cardiomyopathy Structural and Interventional Cardiology Vascular Medicine Vascular Surgery

TRADITION HOSPITAL MARTIN NORTH HOSPITAL MARTIN SOUTH HOSPITAL

WESTON HOSPITAL

About Cleveland Clinic Florida

For Patient Appointments

Cleveland Clinic Florida is a nonprofit, multi-specialty healthcare provider that integrates clinical and hospital care with research and education. The Florida region now includes Cleveland Clinic Indian River Hospital, Cleveland Clinic Martin Health, and Cleveland Clinic Weston Hospital, with five hospitals and numerous outpatient centers in Broward, Palm Beach, Martin, St. Lucie and Indian River counties. Cleveland Clinic Florida ranked #1 in the Miami-Fort Lauderdale metro area and is a top hospital in Florida, according to U.S. News & World Report’s “2021-22 Best Hospitals” rankings. The Florida region is an integral part of Cleveland Clinic in Ohio, where providing outstanding patient care is based upon the principles of cooperation, compassion and innovation.

Physicians at Cleveland Clinic are experts in the treatment of complex conditions that are difficult to diagnose.

Cleveland Clinic Weston Hospital 877.463.2010

For more information about Cleveland Clinic Florida, visit www.clevelandclinicflorida.org.

© 2021 Cleveland Clinic Florida. All Rights Reserved.

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Note: The photo of Brett Faulknier, DO, in the previous publication (top photo, page 11), was taken without a patient. It was solely a representation of a procedure.