Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, People's Republic of China.
J Cardiovasc Electrophysiol. 2018 Oct;29(10):1388-1395. doi: 10.1111/jce.13666. Epub 2018 Jul 6.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable myocardium disorder that predominantly affects the ventricle. Little is known about atrial involvement. This study aimed to assess atrial involvement, especially the role of genotype on atrium in ARVC.
The incidence, characterization and predictors of atrial involvement were investigated. Nine known ARVC-causing genes were screened and the correlation between genotype and atrial involvement was assessed.
Right atrium (RA) dilation, left atrium (LA) dilation, and sustained atrial tachyarrhythmias (ATa) were found in 45, 16 and 3 patients, respectively. Gene mutations were identified in 64 (64.0%) patients. Mutation carriers showed more RA dilation than noncarriers (54.7% vs. 27.8%, P = 0.009), and no difference in LA dilation and ATa. Multivariate analysis showed tricuspid regurgitation (OR: 18.867; 95% CI: 1.466-250.000; P = 0.024) increased the risk of RA dilation and decreased left ventricular ejection fraction (LVEF) (OR: 1.134; 95% CI: 1.002-1.272; P = 0.031) correlated with LA dilation, whereas genotype showed no significant effect. At a median follow-up time of 91 months, 7 patients died and 1 patient accepted heart transplantation. New-onset RA dilation, LA dilation, and sustained ATa were found in 8, 7, and 6 patients, respectively. Atrial involvement was not associated with the long-term survival. Despite mutation carriers showing more RA dilation, Kaplan-Meier analysis showed genotype was not associated with atrial involvement.
Atrial involvement was common in ARVC. Tricuspid regurgitation and decreased LVEF increased the risk for atrial dilation. Genotype was not associated with atrial involvement.
致心律失常性右室心肌病（ARVC）是一种主要影响心室的遗传性心肌疾病。关于心房受累的了解甚少。本研究旨在评估心房受累情况，特别是基因型对 ARVC 心房的作用。
研究了心房受累的发生率、特征和预测因素。筛选了 9 种已知的 ARVC 致病基因，并评估了基因型与心房受累之间的相关性。
分别有 45、16 和 3 例患者出现右心房（RA）扩张、左心房（LA）扩张和持续性房性心动过速（ATa）。64 例（64.0%）患者发现基因突变。突变携带者的 RA 扩张发生率高于非携带者（54.7%比 27.8%，P=0.009），而 LA 扩张和 ATa 无差异。多变量分析显示三尖瓣反流（OR：18.867；95%CI：1.466-250.000；P=0.024）增加了 RA 扩张的风险，而左心室射血分数（LVEF）降低（OR：1.134；95%CI：1.002-1.272；P=0.031）与 LA 扩张相关，而基因型则无显著影响。在中位随访 91 个月时，7 例患者死亡，1 例患者接受心脏移植。8、7 和 6 例患者分别出现新发 RA 扩张、LA 扩张和持续性 ATa。心房受累与长期生存率无关。尽管突变携带者的 RA 扩张更为明显，但 Kaplan-Meier 分析显示基因型与心房受累无关。
ARVC 中常见心房受累。三尖瓣反流和 LVEF 降低增加了心房扩张的风险。基因型与心房受累无关。
