Lakhani Ishan, Zhou Jiandong, Lee Sharen, Li Ka Hou Christien, Leung Keith Sai Kit, Hui Jeremy Man Ho, Lee Yan Hiu Athena, Li Guoliang, Liu Tong, Wong Wing Tak, Wong Ian Chi Kei, Mok Ngai Shing, Mak Chloe Miu, Zhang Qingpeng, Tse Gary
Cardiovascular Analytics Group, Laboratory of Cardiovascular Physiology, Hong Kong, China.
School of Data Science, City University of Hong Kong, Hong Kong, China.
Rev Cardiovasc Med. 2022 Jun 24;23(7):231. doi: 10.31083/j.rcm2307231. eCollection 2022 Jul.
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a hereditary disease characterized by fibrofatty infiltration of the right ventricular myocardium that predisposes affected patients to malignant ventricular arrhythmias, dual-chamber cardiac failure and sudden cardiac death (SCD). The present study aims to investigate the risk of detrimental cardiovascular events in an Asian population of ARVC/D patients, including the incidence of malignant ventricular arrhythmias, new-onset heart failure with reduced ejection fraction (HFrEF), as well as long-term mortality.
This was a territory-wide retrospective cohort study of patients diagnosed with ARVC/D between 1997 and 2019 in Hong Kong. This study consisted of 109 ARVC/D patients (median age: 61 [46-71] years; 58% male). Of these, 51 and 24 patients developed incident VT/VF and new-onset HFrEF, respectively. Five patients underwent cardiac transplantation, and 14 died during follow-up. Multivariate Cox regression identified prolonged QRS duration as a predictor of VT/VF ( 0.05). Female gender, prolonged QTc duration, the presence of epsilon waves and T-wave inversion (TWI) in any lead except aVR/V1 predicted new-onset HFrEF ( 0.05). The presence of epsilon waves, in addition to the parameters of prolonged QRS duration and worsening ejection fraction predicted all-cause mortality ( 0.05). Clinical scores were developed to predict incident VT/VF, new-onset HFrEF and all-cause mortality, and all were significantly improved by machine learning techniques.
Clinical and electrocardiographic parameters are important for assessing prognosis in ARVC/D patients and should in turn be used in tandem to aid risk stratification in the hospital setting.
致心律失常性右室心肌病/发育不良（ARVC/D）是一种遗传性疾病，其特征为右室心肌纤维脂肪浸润，使患病个体易发生恶性室性心律失常、双腔心力衰竭和心源性猝死（SCD）。本研究旨在调查亚洲ARVC/D患者发生不良心血管事件的风险，包括恶性室性心律失常的发生率、射血分数降低的新发心力衰竭（HFrEF）以及长期死亡率。
这是一项对1997年至2019年在香港诊断为ARVC/D的患者进行的全地区回顾性队列研究。本研究纳入了109例ARVC/D患者（中位年龄：61[46 - 71]岁；58%为男性）。其中，分别有51例和24例患者发生了新发室性心动过速/心室颤动（VT/VF）和新发HFrEF。5例患者接受了心脏移植，14例在随访期间死亡。多因素Cox回归分析确定QRS时限延长是VT/VF的预测因素（P<0.05）。女性、QTc时限延长、除aVR/V1外任何导联出现ε波和T波倒置（TWI）可预测新发HFrEF（P<0.05）。除了QRS时限延长和射血分数恶化参数外,ε波的存在可预测全因死亡率（P<0.05）。开发了临床评分来预测新发VT/VF、新发HFrEF和全因死亡率，并且通过机器学习技术所有这些评分均得到显著改善。
临床和心电图参数对于评估ARVC/D患者的预后很重要，进而应联合使用这些参数以辅助医院环境中的风险分层。
