In 9 patients with arrhythmogenic right ventricular cardiomyopathy undergoing heart transplantation, right ventricular failure was universal, often necessitating unique pre-transplant management strategies such as ECMO due to frequent ventricular arrhythmias.
Observational (n=9)
No
Heart transplantation is a viable treatment for end-stage ARVC, but patients require specialized pre-transplant management due to predominant right ventricular failure and frequent ventricular arrhythmias that limit standard hemodynamic support options.
Abstract Aims 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-transplant and peri-transplant management given their predominantly right ventricular (RV) failure and propensity for ventricular arrhythmias. We sought to utilize a tertiary ARVC referral and heart transplant centre experience to describe management of a series of patients with ARVC undergoing heart transplantation at our centre. Methods and results We queried the Johns Hopkins ARVC Registry for all patients who underwent heart transplantation and further studied the subset undergoing transplantation at the Johns Hopkins Hospital. Patient demographics, clinical characteristics, and pre-transplant clinical course were obtained from the registry and electronic medical records. Of the 532 patients in the ARVC Registry, 63 (12%) underwent heart transplantation. Nine (six male) of these patients both had known ARVC prior to transplant and were transplanted at Johns Hopkins Hospital between 2006 and 2020 at a mean age of 42 ± 14 years old. Pathogenic ARVC genetic variants were identified in six (67%) patients, all of whom had variants in the plakophilin-2 (PKP2) gene. RV failure was universal with median right atrial to pulmonary capillary wedge pressure (RA/PCWP) ratio of 1.4 interquartile range (IQR) 1.2–1.5 and median right ventricular stroke work index (RVSWI) of 0 g·m/m2/beat (IQR 0–0.3). Six had a history of catheter ablation for ventricular arrhythmia with five of the six having at least three ablations. Transplant evaluation was initiated an average of 344 ± 407 days after first developing heart failure symptoms. The most common bridge to transplant support included inotropes (n = 3) and extracorporeal membrane oxygenation (ECMO) (n = 2). Contraindication to inotropes or mechanical support was common due to ventricular arrhythmia and RV predominant cardiomyopathy. Conclusions Heart transplantation is a curative treatment for ARVC, but due to frequent ventricular arrhythmias and RV predominant pathology, patients require unique considerations in regard to timing of evaluation, haemodynamic support options, and wait listing qualification.
Scheel et al. (Fri,) conducted a observational in Arrhythmogenic right ventricular cardiomyopathy (ARVC) (n=9). Heart transplantation and pre-transplant management was evaluated on Pre-transplant clinical course and management strategies. In 9 patients with arrhythmogenic right ventricular cardiomyopathy undergoing heart transplantation, right ventricular failure was universal, often necessitating unique pre-transplant management strategies such as ECMO due to frequent ventricular arrhythmias.