Division of Cardiology, Department of Medicine, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Division of Cardiac Surgery, Department of Surgery, Johns Hopkins School of Medicine, Baltimore, MD, USA.
ESC Heart Fail. 2022 Apr;9(2):1008-1017. doi: 10.1002/ehf2.13757. Epub 2021 Dec 24.
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.
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/m /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.
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.
心律失常性右室心肌病（ARVC）患者因终末期心力衰竭需要评估进行心脏移植的情况日益增多。由于这些患者主要为右心室（RV）衰竭并易发生室性心律失常，因此在移植前和移植期间的管理方面存在独特的挑战。我们试图利用三级 ARVC 转诊和心脏移植中心的经验来描述我们中心一系列 ARVC 患者接受心脏移植的管理情况。
我们从约翰霍普金斯 ARVC 注册处查询了所有接受心脏移植的患者，并进一步研究了在约翰霍普金斯医院接受移植的亚组患者。从注册处和电子病历中获取患者的人口统计学、临床特征和移植前临床过程数据。在 ARVC 注册处的 532 名患者中，有 63 名（12%）接受了心脏移植。其中 9 名（6 名男性）患者在移植前已知患有 ARVC，并于 2006 年至 2020 年在约翰霍普金斯医院接受移植，平均年龄为 42±14 岁。6 名患者（67%）发现致病性 ARVC 基因突变，所有患者均存在桥粒蛋白-2（PKP2）基因突变。RV 衰竭普遍存在，中位右心房至肺毛细血管楔压（RA/PCWP）比值为 1.4[四分位距（IQR）1.2-1.5]，中位右心室每搏做功指数（RVSWI）为 0g·m/m /beat（IQR 0-0.3）。6 名患者因室性心律失常接受了导管消融治疗，其中 5 名患者至少进行了 3 次消融。首次出现心力衰竭症状后，平均 344±407 天开始移植评估。最常见的桥接移植支持包括正性肌力药物（n=3）和体外膜肺氧合（ECMO）（n=2）。由于室性心律失常和 RV 为主的心肌病，正性肌力药物或机械支持的禁忌证很常见。
心脏移植是 ARVC 的一种治愈性治疗方法，但由于频繁发生室性心律失常和 RV 为主的病理学改变，在评估时机、血流动力学支持选择和等待列表资格方面，患者需要独特的考虑。
