@article{3c82acc4733f4f79aa170d82b1dfaa67,
title = "Heart transplantation strategies in arrhythmogenic right ventricular cardiomyopathy: a tertiary ARVC centre experience",
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.",
keywords = "Arrhythmogenic cardiomyopathy, Arrhythmogenic right ventricular cardiomyopathy, Genetics, Heart transplantation, Mechanical circulatory support, Right ventricular failure",
author = "Scheel, {Paul J.} and Katherine Giuliano and Crystal Tichnell and Cynthia James and Brittney Murray and Harikrishna Tandri and Debra Carter and Tracey Fehr and Priya Umapathi and Joban Vaishnav and Lewsey, {Sabra C.} and Steven Hsu and Hugh Calkins and Kavita Sharma and Choi, {Chun Woo} and Gilotra, {Nisha A.} and Ahmet Kilic",
note = "Funding Information: This work and the Johns Hopkins ARVD/C Program is supported by the Leonie‐Wild Charitable Foundation (Heidelberg, Germany), the Dr. Francis P. Chiaramonte Private Foundation (Alexandria, VA, USA), the Leyla Erkan Family Fund for ARVD Research, the Dr. Satish, Rupal, and Robin Shah ARVD Fund at Johns Hopkins (Baltimore, MD, USA), the Bogle Foundation, the Healing Hearts Foundation, the Campanella Family, the Patrick J. Harrison Family, the Peter French Memorial Foundation, and the Wilmerding Endowments. The authors also wish to acknowledge a grant from the Fondation Leducq (HC) (Paris, France). Funding Information: This publication was made possible by the Johns Hopkins Institute for Clinical and Translational Research (ICTR) which is funded in part by Grant Number UL1 TR003098 from the National Center for Advancing Translational Sciences (NCATS), a component of the National Institutes of Health (NIH), and NIH Roadmap for Medical Research. Its contents are solely the responsibility of the authors and do not necessarily represent the official view of the Johns Hopkins ICTR, NCATS, or NIH. Funding Information: This work and the Johns Hopkins ARVD/C Program is supported by the Leonie-Wild Charitable Foundation (Heidelberg, Germany), the Dr. Francis P. Chiaramonte Private Foundation (Alexandria, VA, USA), the Leyla Erkan Family Fund for ARVD Research, the Dr. Satish, Rupal, and Robin Shah ARVD Fund at Johns Hopkins (Baltimore, MD, USA), the Bogle Foundation, the Healing Hearts Foundation, the Campanella Family, the Patrick J. Harrison Family, the Peter French Memorial Foundation, and the Wilmerding Endowments. The authors also wish to acknowledge a grant from the Fondation Leducq (HC) (Paris, France). This publication was made possible by the Johns Hopkins Institute for Clinical and Translational Research (ICTR) which is funded in part by Grant Number UL1 TR003098 from the National Center for Advancing Translational Sciences (NCATS), a component of the National Institutes of Health (NIH), and NIH Roadmap for Medical Research. Its contents are solely the responsibility of the authors and do not necessarily represent the official view of the Johns Hopkins ICTR, NCATS, or NIH. Study data were collected and managed using REDCap electronic data capture tools hosted at Johns Hopkins University. REDCap (Research Electronic Data Capture) is a secure, web-based software platform designed to support data capture for research studies, providing (i) an intuitive interface for validated data capture; (ii) audit trails for tracking data manipulation and export procedures; (iii) automated export procedures for seamless data downloads to common statistical packages; and (iv) procedures for data integration and interoperability with external sources. We are grateful to the ARVC/D patients and families who have made this work possible. Funding Information: P.S., K.G., D.C., T.F., S.H., J.V., S.L., P.U., H.T., K.S., C.W.C., and A.K. have no declarations. H.C. has no relevant declarations; H.C. is a consultant for Medtronic Inc, Biosense Webster, and St. Jude Medical/Abbott and receives research support from Medtronic, Biosense Webster, Farapulse, Adagio, and Boston Scientific Corp. C.T. and C.J. receive salary support from this grant (Boston Scientific Corp); C.T. and C.J. have no relevant declarations. B.M. has no relevant declarations. B.M. is a consultant for MyGeneCounsel. N.G. has no relevant declarations. N.G. is on the advisory board and speaking honorarium from scPharmaceuticals and receives research funding from Intra‐cellular Therapies, Inc. Publisher Copyright: {\textcopyright} 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.",
year = "2022",
month = apr,
doi = "10.1002/ehf2.13757",
language = "English (US)",
volume = "9",
pages = "1008--1017",
journal = "ESC Heart Failure",
issn = "2055-5822",
publisher = "The Heart Failure Association of the European Society of Cardiology",
number = "2",
}