Heart Transplantation Strategies In Arrhythmogenic Right Ventricular Cardiomyopathy: The Johns Hopkins Experience

Abstract

<h3>Background</h3> End stage heart failure necessitating evaluation for heart transplantation is increasingly recognized in arrhythmogenic right ventricular cardiomyopathy (ARVC). These patients present unique challenges in pre- and peri-transplant management given predominantly right ventricle (RV) failure and propensity for ventricular arrhythmias. We utilized a tertiary ARVC referral and heart transplant center experience to describe management of patients with ARVC undergoing heart transplantation. <h3>Methods</h3> We queried the Johns Hopkins ARVC Registry for all patients who underwent heart transplantation and studied the subset undergoing transplantation at Johns Hopkins. Patient demographics, clinical characteristics, and pre-transplant clinical course were obtained from the registry and medical records. <h3>Results</h3> Of the 532 patients in the ARVC Registry, 63 underwent heart transplantation. Nine (3 female) patients had known ARVC prior to transplant and were transplanted at Johns Hopkins Hospital from 2006 to 2020; 5 occurred after the 2018 allocation system revision. Plakophilin-2 (<i>PKP2</i>) pathogenic or likely pathogenic variant was present in 6 (67%) patients; the others were gene elusive. Six patients had a history of catheter ablation for ventricular arrhythmia (5 had at least 3 ablations). All 9 patients had significant right ventricular failure as demonstrated in <b>Figure</b>. Transplant evaluation occurred at a mean age of 42±14 years and was typically initiated within 1 year of developing heart failure. Initial waitlist status was 2 (old system) or 6 (revised system) in 7 (78%) patients; 1 patient underwent transplant at this status, the remaining required upgraded status because of clinical worsening. Bridge to transplant support included inotropes (n=3) and ECMO (n=2). Percutaneous RV mechanical support was unsuccessful in 2 patients due to severe RV dilation. Contraindication to inotropes or left ventricle mechanical support was common due to ventricular arrhythmia and RV predominant cardiomyopathy. <h3>Conclusion</h3> Heart transplantation is a curative treatment for ARVC, but due to frequent ventricular arrhythmia and RV predominant pathology, patients require unique considerations in regards to timing of evaluation, hemodynamic support options and wait listing qualification.