From the Editor’s Desk

From the Editor's Desk

Aaron R. Morton, MMSc, PA-C, ATC, FAPACVS

Editor-in-Chief

Welcome to the first JAPACVS issue of 2022. 2021 was an incredible year of adaptation, change, discovery and firsts – some of these better than others. The APACVS had its first virtual meeting, followed by its first stand alone in-person hands-on education meeting in Louisville. At the start of 2021, we saw the first round of vaccine completion for healthcare workers which eventually opened to all adults and many children, followed with booster vaccines and the discovery of multiple Sars Cov-2 strains. We navigated through two Covid-19 surges in 2021 only to find we are ending the year on another. On the practice side of things, we saw the implantation of a new total artificial heart by Carmet, a drastic increase in utilization of adult ECMO, increase in lung transplantation following Covid-19 infections, and we likely all faced enough supply and medication shortages or substitutions to last a lifetime.

2021 was a year full of change as well for the JAPACVS, both from a leadership and structural point of view. This issue is my first issue as Editor-in-Chief. I want to extend my personal gratitude as well as that of the APACVS Board to our founding Editor, Mr. David J. Bunnel, MSHS, PA-C, DFAAPA. Under his leadership, through sheer tenacity, commitment and dedication, we saw the journal grow from an idea to an actual publication. Additionally, this year we have undertaken an immense restructuring effort to situate our journal for growth both in our respective specialties as well as internationally. This was initiated with the appointment of Ms. Anitha Chandrasekhar, PA as our new International Associate Editor. Ms. Chandrasekhar will work to incorporate our international Cardiac Surgical, Thoracic Surgical, Vascular Surgical and Critical Care PA colleagues into the JAPACVS. Additionally, Mr. Edward Ranzenbach, PA-C, MPAS, FAPACVS, DFAAPA has been appointed as the Associate Editor for Writer Development. He will work with interested authors who might not have had the opportunity to write before, to develop a manuscript and work through the writing process.

Furthermore, we have developed additional structure for the JAPACVS in the form of Section Editors who will support each of our represented specialties: Cardiac Surgery, Thoracic Surgery, Vascular Surgery and Cardiothoracic and Vascular Critical Care. The Section Editors will assist with specialty area peer review, content development and serve as specialty experts for the JAPACVS Editorial Board. If you are interested in serving as a Section Editor, be on the lookout for an invite in the new year. What an incredible year it has been!

In this issue, we look at continued care following a complex congenital cardiac repair, examine a protocol for enhanced resuscitation following cardiac surgery and discuss strategies to mitigate clinician burn-out. There has been much in both the lay press as well as the academic world in the last few years regarding burn-out. This well written manuscript helps examine the aspects of burn-out and will be the JAPACVS’s first publication on professional issues.

As always, a journal must have submissions to remain viable. If you would like to contribute an article or get involved with the Editorial Board, please feel free to drop me a line. Wishing you and yours health and prosperity in the new year!

Be well,

-AM

Aaron Morton MMSc, PA-C, ATC, FAPACVS JAPACVS Editor-in-Chief

editor@japacvs.org

JAPACVS publishes invited commentary and letters to the editor in response to published articles in which the authors are given the opportunity to respond .

A Case Study of a Complex Single Ventricle Patient and Placement of a Transvenous Transpulmonary Atrial Pacemaker

Mary Pickard, MSPAS, PA-C Amy Benson, DMSc, PA-C* Presbyterian Hospital, Albuquerque, New Mexico

ABSTRACT

The Fontan operation is the final intervention in the palliation of single ventricle pathology. Advances in the operation and post-operative care have dramatically increased life expectancy. However, the complications post operatively and inherent to single ventricles remain a growing area of concern with arrhythmias being the most common complication. The need for pacemakers, particularly endocardial lead placement in adult Fontan patients, are challenging due to complete redirection of venous blood flow to the pulmonary arteries. This case study visits an alternative option to epicardial lead implantation by inserting an endocardial lead via a puncture through the pulmonary artery and highlights the common complications that are seen in the aging Fontan population.

KEY WORDS

Single ventricle, Fontan, transpulmonary, epicardial lead pacemaker, adult congenital heart disease, endocardial lead placement, arrhythmias

CASE

The patient is a 25-year-old male, with complex congenital cardiac history. He was admitted to the hospital secondary o episodes of junctional bradycardia in the 30s, and pauses up to 4.6 seconds, detected on a Holter Monitor. This precipitated symptoms of dizziness, lightheadedness, and near syncope.

Cardiac History

The patient has a complex cardiac history including single ventricle disease (double inlet left ventricle with subaortic stenosis and coarctation of the aorta) who had previously undergone surgical palliation with bidirectional Glenn anastomosis, Damus-Kaye-Stansel (DKS)

*Dr. Benson is now employed as a Senior Medical Science Liaison with The Janssen Pharmaceutical Companies of Johnson & Johnson. This position was obtained after involvement in the development of this manuscript.

procedure due to subaortic stenosis and extracardiac Fontan completion at the age of six (final anatomy seen in Figure 1). Two previous attempts of the Fontan were aborted due to inadvertently entering the heart while performing redo sternotomy. The patient is also noted to have progressive Fontan failure and stenosis with previous stent placement in the Fontan conduit and related liver cirrhosis and evidence of decompensated portal hypertension with varices. Other cardiac history includes paroxysmal atrial flutter and atrial fibrillation, which have been previously cardioverted, and now medically managed with metoprolol and rivaroxaban. Previous attempts to initiate sotalol ended with torsade de pointes and cardiac arrest.

Past Medical History

The patients past medical history includes: Depression, alcohol abuse and polycythemia, likely due to chronic hypoxemia from right to left shunting through his veno-venous collaterals.

Figure 1.

Legend 1. Total Cavo-Pulmonary Connection (TCPC) 2. Extracardiac Fontan Conduit 3. Fenestration 4. Damus-Kaye-Stansel (DKS)

Procedure 5. Pulmonary Artery Ligation 6. Atrial Mass with ASD Enlargement 7. Hypoplastic Right Ventricle 8. Double Inlet Left Ventricle 9. Single left ventricle

TCPC with Extracardiac Conduit and DKS Anastomosis for DILV

Illustration created June 2021 Illustration taken from http://www.chd-diagrams.com.1

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Background

The Fontan procedure was initially performed in 1968 by Dr. Fontan for tricuspid atresia but is now utilized for any functional or anatomical single ventricle pathology including pulmonary atresia with intact ventricular septum, hypoplastic left heart syndrome, unbalanced atrio ventricular (AV) canal defects, double outlet right ventricle (DORV) and double-inlet left ventricle (DILV).2 The original operation has significantly evolved since it was first introduced and is now the last step in a series of staged interventions for functional single ventricles. Most commonly, the initial procedure, as seen in this patient, starts with a Glenn shunt which connects the superior vena cava (SVC) to the right pulmonary artery. This is followed by an extracardiac total cavopulmonary connection (TCPC) which connects the inferior vena cava (IVC) to the right pulmonary artery via a conduit, also known as a Fontan operation.2 Depending on individual anatomy and patient’s cardio-pulmonary blood flow, several other operations can be seen prior to the Fontan operation. These include atrial septectomy or septostomy, pulmonary artery banding, systemic-pulmonary shunts such as Blalock-Taussig shunt, Norwood operation and Damus-Kaye-Stansel (DKS) procedure.2

In this case study, the patient’s primary underlying cardiac anatomy is a double inlet left ventricle; meaning that both left and right atrium drain into an enlarged left ventricle with a hypoplastic right ventricle.3 Furthermore, this patient was born with subaortic stenosis requiring a DKS procedure prior to Fontan palliation. The DKS procedure is utilized for instances in which the systemic arterial outflow tract is obstructed, such as subaortic stenosis. This procedure includes dividing the main pulmonary artery away from the right and left pulmonary arteries and joining it to the side of the ascending aorta in an end to side anastamosis.4 The DKS is also noted to have several modifications. Under these techniques, systemic venous blood flow is completely redirected to the pulmonary arteries which allows for the single ventricle to be utilized as the systemic arterial pump.

Hospital Course

The patient was directly admitted to hospital on the cardiac floor and placed on telemetry. His rivaroxaban was discontinued with plans to implant a pacemaker for his sinus node dysfunction. He was initiated on a heparin infusion for continued thromboembolic prophylaxis, given his various atrial arrhythmias. His metoprolol was discontinued due to sinus node dysfunction. On telemetry, he continued to exhibit episodes of paroxysmal 2:1 atrial flutter with rapid rates in addition to junctional bradycardia and sinus pauses, with associated hemodynamic compromise and symptoms of dizziness and presyncope. Given patient’s extensive cardiac history, cardio-thoracic (CT) surgery was consulted to review the option of epicardial lead placement. A chest CT angiogram (CTA) was ordered in anticipation of this consult and surgical plan for reoperation of the chest. Ultimately, after review of imaging and history, CT surgery felt the patient was too high risk for surgical epicardial lead placement. As stated earlier, the patient had repeated attempts at redo sternotomy that had been aborted due to inadvertent entry into the heart and associated hemorrhage. In addition, on the CTA, multiple bilateral pulmonary emboli (PE) were identified as well as advanced liver cirrhosis and extensive paraoesophageal varices and potential thrombus within the Fontan conduit. Since the epicardial lead placement was no longer an option by CT surgery, electrophysiology (EP) service was consulted and started the patient on Dofetilide 500mg twice daily for arrhythmias with consideration future transvenous placement of pacemaker. A transesophageal

echocardiogram (TEE) was ordered to evaluate the Fontan conduit for possible thrombus and gastroenterology was consulted for esophagogastroduodenoscopy (EGD) to evaluate the paraesophageal varices found by CTA. Given the need for anticoagulation and plans for transesophageal echocardiogram (TEE) to evaluate the potential Fontan thrombus, gastroenterology was consulted for esophagogastroduodenoscopy (EGD) to evaluate for paraesophageal varices found by CTA. The EGD found small esophageal varices, portal hypertensive gastropathy and gastric/duodenal erosions. Gastroenterology felt it was safe to initiate anticoagulation in addition to proton-pump inhibitor (PPI) therapy. After completing two days of Dofetilide, the patient continued to have intermittent junctional bradycardia and second-degree AV block. After review of several options for atrial support pacing, it was deemed the safest option was to attempt at a transvenous pacemaker system via a puncture through the pulmonary artery into the atrial mass. Given the left and right atria are no longer divided by a septum, the atria are considered one and often referred to as an “atrial mass”. The procedure would require a multidisciplinary approach with extensive TEE guidance and cardiopulmonary angiography with pressure assessments.

Procedure

After informed consent was obtained, the patient was taken to the electrophysiology lab and placed under general endotracheal anesthesia by the pediatric cardiac anesthesia team. The right internal jugular vein was accessed, and a pigtail catheter was positioned into the left pulmonary artery. The TEE probe was inserted to visualize the atrial mass. Multiple angiographic images were taken to assess system and pulmonary pressures and an angiogram was taken for visualization of anatomy (see Figure 2). No thrombus was identified in the Fontan conduit by TEE or angiogram visualization. An incision was made below the left clavicle and the left subclavian vein access was achieved and a Glidewire was placed into the left pulmonary artery. A preface sheath and dilator were advanced and placed into the more distal portion of the pulmonary artery. However, the BRKTM transseptal needle was unable to be advanced past the tight curve from the Glenn anastomosis into the left pulmonary artery (see Figure 3). For this reason, attempts for placement of an atrial lead from left subclavian vein were abandoned. The catheters and needle were removed and the left infraclavicular incision was closed. Next, the right internal jugular sheath was accessed, and a 12 French sheath was advanced over a wire into the left pulmonary artery. The preface sheath was retracted onto the dome of the left atrial mass. The BRKTM transseptal needle was then inserted. Transesophageal echocardiographic imaging was utilized to make certain the preface sheath and needle were positioned correctly over the dome of the left atrial mass. Once confirmed, the BRKTM needle was advanced and entered the left atrial mass and followed by preface sheath and dilator. The dilator and needle were then removed and an Amplatz stiff wire was then inserted through the preface sheath down into the systemic ventricle and up the pulmonary vasculature. Three different delivery sheaths were utilized including multiple deployments of the 3830 lead. Limitations of the procedure included poor thresholds and or poor sensing. Ultimately, a site was identified, lead was deployed and found to have good thresholds and excellent sensing (see Figure 4). Adequate slack was left, and the lead was secured at the neck utilizing a sewing sleeve. A device pocket was created below right clavicle and utilizing a tunneling tool, the atrial lead was accessed from the right internal jugular vein down into the pocket. The neck incision was closed,

and the lead was secured with a second sewing sleeve within the pocket and secured to the pulse generator. The device pocket was closed with an occlusive silver dressing applied over skin. The patient tolerated the procedure well without any apparent complications such as pericardial effusions, tamponade, or excessive bleeding issues.

Figure 2.

Right PA Left PA

Atrial Mass

Angiogram showing Superior vena cava filling Glenn Anastomosis into pulmonary arteries

Angiogram taken October 20205

Figure 3.

Left PA

Atrial Mass

Angiogram showing the acute angle from left subclavian through the SVC, Glenn anastomosis into the left pulmonary artery

Angiogram taken October 20205

Final placement of lead in atrial mass from right jugular vein

Right PA Left PA

Atrial Mass

X-ray taken October 20205

Post Procedure Hospital Course

The patient was taken back to his room and a chest radiograph was performed the next morning showing device and lead were well positioned. Interrogation of device showed good function with no change in thresholds and sensing. A transthoracic echocardiogram was performed with no evidence of a pericardial effusion. His Dofetilide was decreased to 250 mcg twice daily and heparin was converted back to rivaroxaban 20mg nightly. He was discharged home post procedure day two.

Follow up

The patient was enrolled into the device clinic. Three weeks post discharge, he returned to clinic for a device check. The patient was feeling better with improved energy, no further dizziness or syncope. Incisions had healed with some moderate bruising over device pocket without hematoma. His device was functioning normally without any atrial tachycardia/atrial fibrillation events and 44.3% atrial pacing.

DISCUSSION

Advances in the Fontan procedure have increased life expectancy significantly with a 30year post surgery life expectancy of greater than 80%.6 A meta-analysis of 5,859 patients who underwent Fontan completion after the year 2000, found the 5, 10, 15-year transplant free survival was 90.7%, 87.2% and 87.5% respectively.7 Despite advances in the Fontan operation and the marked increase in survivability, complications are inevitable. The Fontan procedure is palliative in nature and the innate complications associated with having single ventricle remain present.7,8 The single ventricle pathophysiology is characterized by chronically elevated systemic venous pressures and decreased cardiac output.6 Common complications include

congestive heart failure, arrhythmias, thromboembolic events, protein-losing enteropathy (PLE), plastic bronchitis, and progressive liver fibrosis and renal dysfunction.6,7 In addition, cognitive and behavioral issues are common.6

The most common complication following a Fontan procedure in both short term and long term follow up are arrhythmias, particularly atrial arrhythmias.7,9 The incident of arrhythmias have been reported as high as 44%, followed by thromboembolic event and protein-losing enteropathy.8,9 A significantly high morbidity is associated with development of atrial tachycardia given the progression to heart failure and creation of thrombosis.6 However, treating these arrhythmias, particularly with antiarrhythmic medications, presents a challenge given the related and progressive hepatic and renal dysfunction. In addition to atrial arrythmias, sinus node dysfunction is present in about 9% of Fontan patients and presents its own set of challenges.10 Pacemaker implantation and revision are the most common late reoperation indication in the post Fontan population at 23%.8,10 Currently in Fontan patients, the standard of care and most pragmatic choice for pacemaker insertion is through redo sternotomy or thoracotomy with epicardial lead placement due to the redirection of venous blood flow into the pulmonary arteries. However, epicardial pacemakers are fraught with issues including rapid battery depletion, frequent lead complications, limited sites for lead implant and limited ability to cause synchronous ventricular systole. Therefore, there is a growing need for alternative options, particularly endocardial lead placements in the adult population.11 Atrial access can be achieved via a puncture in the Fontan conduit which is typically made of a plastic or Gore-Tex tubing. However, this option can be difficult to puncture, increase infection and thrombosis rates. The technique described in this case study via the SVC and puncture through the pulmonary artery has proven both feasible and effective.

CONCLUSION

Advances in the Fontan operation have led to a substantial increase in life expectancy. Unfortunately, a TCPC Fontan palliation does not alleviate the inherent complications associated with a single ventricle pathology that is marked by chronically elevated systemic venous pressures and decreased cardiac output. Complications in Fontan circulation all present their own unique challenges and treatment of arrhythmias and sinus node dysfunction is no exception. Traditional epicardial lead pacemakers are fraught with issues and alternative options are limited given the complete redirection of venous blood flow directly to the pulmonary arteries. As emphasized in this case study, a transvenous endocardial lead placement via puncture through the pulmonary artery was both safe and effective. However, the path to this decision was marked by difficulties and highlights the complexity of the aging Fontan population and the need for a multispecialty team approach for best patient practice in the congenital cardiac patients.

KEY POINTS

1. Fontan patients are highly complex requiring a multispecialty team approach. 2. Longer life expectancy in Fontan population is leading to an increase in complications to be addressed. 3. Recurrent atrial arrhythmias and need for pacemakers in Fontan circulation are prevalent and marked by challenges. 4. Transpulmonary transvenous pacemaker is a safe and effective way to obtain pacing in

1. Figure 1. TCPC with extracardiac conduit with fenestration and DKS anastomosis for DILV.

University of Basel. Image created June 20, 2021. Illustration taken from http://www.chddiagrams.com

2. Fredenburg TB, Johnson TR, Cohen MD. The Fontan procedure: Anatomy, complications, and manifestations of failure. Radiographics. 2011;31(2):453-463. Accessed Nov 24, 2020. https://doi.org/10.1148/rg.312105027

3. Double Inlet Left Ventricle DILV. The Royal Children’s Hosptial Melbourne. Accessed November 24, 2020. https://www.rch.org.au/cardiology/heart_defects/

Double_Inlet_Left_Ventricle_DILV/

4. Yang CK, Jang WS, Choi ES, et al. The clinical outcomes of damus-kaye-stansel procedure according to surgical technique. Korean J Thorac Cardiovasc Surg. 2014;47(4):344-349. https://doi.org/10.5090/kjtcs.2014.47.4.344

5. Love, Jon MD. West, Michael MD. Figure 2-4. Taken October 2020. file:///Users/Mary/

Downloads/figure4pacemaker.JPG

6. Rychik, Jack, Atz Andrew M, Celermajer, David S, et al. Evaluation and management of the child and adult with fontan circulation: A scientific statement from the American Heart

Association. Circulation. 2019;140(6):e234-e284. Accessed Nov 24, 2020. https:// doi.org/10.1161/CIR.0000000000000696

7. Schwartz I, McCracken CE, Petit CJ, Sachdeva R. Late outcomes after the Fontan procedure in patients with single ventricle: a meta-analysis. Heart. 2018;104(18):1508-1514. doi:10.1136/heartjnl-2017-312807

8. Kay, Aaron W, Moe, Tabitha, Suter, Blair, et al. Long term consequences of the Fontan procedure and how to manage them. Accessed Nov 24, 2020. https://doi.org/10.1016/ j.pcad.2018.09.005

9. Pundi, Kavitha N, Johnsons, Jonathan N, Dearani, Joseph A, et al. 40-year follow-up after the Fontan operation: Long-term outcomes of 1,052 patients. Journal of the American

College of Cardiology. 2015;66(15):1700-1710. Accessed Nov 24, 2020. https:// doi.org/10.1016/j.jacc.2015.07.065

10. Kverneland LS, Kramer P, Ovroutski S. Five decades of the Fontan operation: A systemic review of international reports on outcomes after univentricular palliation. Congenital Heart

Disease. 2018;(13)2:181-193. Accessed April 2021. https://doi.org/10.1111/chd.12570

11. DeWitt E, Callahan R, Blume E, Marshall A, Mah D. Trans-fontan baffle placement of an endocardial systemic ventricular pacing lead. HeartRhythm Case Rep. 2017;3(2):129-132.

Accessed Nov 24, 2020. https://doi.org/10.1016/j.hrcr.2016.10.002

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