ArticleESC heart failure2022
Heart transplantation strategies in arrhythmogenic right ventricular cardiomyopathy: a tertiary ARVC centre experience.
Article in ESC heart failure, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 18 citations in OpenAlex.
- Arrhythmogenic right ventricular cardiomyopathy.European heart journal · 2026Review
- Right Heart Failure in Critical and Chronic Care: Current Concepts, Challenges and Mechanical Support Strategies.Medical sciences (Basel, Switzerland) · 2025Review
- Arrhythmogenic Cardiomyopathy: Expanding Phenotypic Variability in Different Populations Around the World.JACC. Asia · 2025Article
- Heart Transplant and Durable Mechanical Circulatory Support for Specific Less-Common Cardiomyopathies.CJC open · 2025Review
- Successful management of HeartMate 3 in a patient with arrhythmogenic right ventricular cardiomyopathy.Journal of artificial organs : the official journal of the Japanese Society for Artificial Organs · 2024Article
- Describing and Mapping the Research Trend of Scientific Publications on Arrhythmogenic Right Ventricular Cardiomyopathy Across Four Decades: A Bibliometric Analysis.Clinical cardiology · 2024Article
- Management Strategies in Arrhythmogenic Cardiomyopathy across the Spectrum of Ventricular Involvement.Biomedicines · 2023Review
- A unique cuff-sewing method to implant heartmate3 LVAD for arrhythmogenic right ventricular cardiomyopathy, facilitated by 3D-printing heart model.Journal of artificial organs : the official journal of the Japanese Society for Artificial Organs · 2023Article
- Heart transplantation strategies in arrhythmogenic right ventricular cardiomyopathy: a tertiary ARVC centre experience.ESC heart failure · 2022Article
Corrections and comments
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Authors and funding
17 authors at 1 institution in 1 country.
Funding
Abstract
aimsEnd-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
resultsWe 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/m
conclusionsHeart 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.
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