From the Division of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore, MD (N.A.G., A.B., C.A.J., B.M., C.T., C.S.O., D.P.J., S.D.R., H.C., R.J.T.); Division of Cardiology, University Medical Center Utrecht, The Netherlands (A.S.J.t.R.); and Division of Cardiology, Department of Medicine, University at Buffalo, State University of New York (A.S.).
Circ Heart Fail. 2017 Sep;10(9). doi: 10.1161/CIRCHEARTFAILURE.116.003819.
Heart failure (HF) prevalence in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) varies depending on study cohort and is not well characterized. This study sought to determine prevalence and predictors of HF in ARVC/D.
Clinical HF, defined as at least 1 HF sign or symptom, was retrospectively adjudicated for 289 patients meeting ARVC/D Task Force Criteria. HF was present in 142 patients (49%): 113 had isolated RV involvement and 29 had evidence of LV dysfunction. Average age of HF onset was 40±14 years. Most commonly reported symptoms were exertional dyspnea (78%) and fatigue (73%). Only 40% (n=57/142) had signs of volume overload. Left-sided HF signs were rare. Patients with clinical HF before ARVC/D diagnosis (n=31) were older (=0.005) and met fewer Task Force Criteria (=0.013) than those who developed HF after ARVC/D presentation. Female sex (odds ratio, 2.2; 95% confidence interval, 1.21-4.01; =0.01) and lateral precordial T-wave inversions (odds ratio, 9.87; 95% confidence interval, 1.07-91.1; =0.043) were associated with increased odds of HF. Additionally, patients with symptomatic LV dysfunction had higher odds of lateral precordial T-wave inversions (odds ratio, 18.4; 95% confidence interval, 2.92-116.18; =0.002). Patients with HF were more likely to undergo heart transplantation (15/142 versus 1/147; <0.001) or die during study follow-up period (7 versus 0; =0.007).
HF symptoms, especially exertional dyspnea, are common in ARVC/D; yet, classic left-sided signs are typically absent and less than half have evidence of volume overload. Given the unique predominately right-sided phenotype, a large portion of patients with HF may be under-recognized.
心律失常性右室心肌病/发育不良（ARVC/D）的心力衰竭（HF）患病率因研究队列而异，且尚未得到充分描述。本研究旨在确定 ARVC/D 中 HF 的患病率和预测因素。
临床 HF 定义为至少有 1 项 HF 体征或症状，对符合 ARVC/D 工作组标准的 289 例患者进行了回顾性判定。142 例患者（49%）存在 HF：113 例为单纯 RV 受累，29 例存在 LV 功能障碍证据。HF 发病的平均年龄为 40±14 岁。最常报告的症状是劳力性呼吸困难（78%）和疲劳（73%）。仅有 40%（n=57/142）存在容量超负荷的体征。左侧 HF 体征罕见。在 ARVC/D 诊断前发生临床 HF（n=31）的患者比在 ARVC/D 表现后发生 HF 的患者年龄更大（=0.005），且符合的工作组标准更少（=0.013）。女性（比值比，2.2；95%置信区间，1.21-4.01；=0.01）和外侧胸前区 T 波倒置（比值比，9.87；95%置信区间，1.07-91.1；=0.043）与 HF 发生的可能性增加相关。此外，有症状的 LV 功能障碍患者更有可能出现外侧胸前区 T 波倒置（比值比，18.4；95%置信区间，2.92-116.18；=0.002）。HF 患者更有可能在研究随访期间接受心脏移植（15/142 例 vs. 1/147 例；<0.001）或死亡（7 例 vs. 0 例；=0.007）。
ARVC/D 中 HF 症状，尤其是劳力性呼吸困难，较为常见；然而，典型的左侧体征通常不存在，且不到一半的患者存在容量超负荷的证据。鉴于其独特的主要右侧表型，很大一部分 HF 患者可能未被充分识别。
