医学
外显率
危险分层
心肌病
心源性猝死
心脏病学
植入式心律转复除颤器
心力衰竭
内科学
猝死
重症监护医学
表型
基因
遗传学
生物
作者
Sohaib Ahmad Basharat,Ingrid Hsiung,Jalaj Garg,Amro Alsaid
标识
DOI:10.1016/j.hfc.2023.03.006
摘要
Arrhythmogenic cardiomyopathy (ACM) is an umbrella term encompassing a wide variety of overlapping hereditary and nonhereditary disorders that can result in malignant ventricular arrhythmias and sudden cardiac death. Cardiac MRI plays a critical role in accurate diagnosis of various ACM entities and is increasingly showing promise in risk stratification that can further guide management particularly in decisions regarding use of implantable cardioverter defibrillator. Genotyping plays an important role in cascade testing but challenges remain due to incomplete penetrance and wide phenotypic variability of ACM as well as the presence of gene-elusive cases.
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