表现为单形性室性心动过速的致心律失常性右室心肌病
Arrhythmogenic Right Ventricular Cardiomyopathy Presenting as Monomorphic Ventricular Tachycardia.
作者信息
Hussain Fahad, Cyr Andrew, Shusterman Vlad Shknevskiy, Blumfield Amit, Patel Priyen, Li Shuojohn, Ahmad Syed, Willner Jonathan, Azari Bani, Shah Samit
机构信息
Department of Internal Medicine, Northwell Health, Manhasset, New York, USA.
Department of Internal Medicine, Northwell Health, Manhasset, New York, USA.
出版信息
JACC Case Rep. 2025 Aug 27;30(25):104941. doi: 10.1016/j.jaccas.2025.104941.
INTRODUCTION
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an autosomal dominant genetic cardiomyopathy characterized by the replacement of right ventricular myocardium with fibrous and adipose tissue, leading to arrhythmias, heart failure, and an increased risk of sudden cardiac death.
CASE SUMMARY
A 25-year-old woman without any medical history presented with palpitations after exercise and was found to be in sustained monomorphic ventricular tachycardia. Imaging and presentation met the 2010 modified Task Force Criteria for a diagnosis of ARVC.
DISCUSSION
ARVC can be a devastating cause of heart failure and sudden cardiac death in young adults. Once the diagnosis is confirmed, treatment including exercise restriction, antiarrhythmics, goal-directed medical therapy, ablation, and implantable cardioverter-defibrillator placement can lead to marked improvement in prognosis.
TAKE-HOME MESSAGE: Clinicians should consider ARVC in young patients presenting with ventricular tachyarrhythmias and use the 2010 modified Task Force Criteria to guide initial diagnosis before confirming with genetic testing.
引言
致心律失常性右室心肌病（ARVC）是一种常染色体显性遗传性心肌病，其特征是右心室心肌被纤维组织和脂肪组织替代，导致心律失常、心力衰竭以及心脏性猝死风险增加。
病例摘要
一名25岁无任何病史的女性在运动后出现心悸，被发现为持续性单形性室性心动过速。影像学检查和临床表现符合2010年修订的工作组ARVC诊断标准。
讨论
ARVC可能是年轻成人心力衰竭和心脏性猝死的一个毁灭性病因。一旦确诊，包括限制运动、抗心律失常药物、目标导向的药物治疗、消融以及植入式心律转复除颤器植入等治疗可显著改善预后。
要点
临床医生在面对出现室性快速心律失常的年轻患者时应考虑ARVC，并在进行基因检测确诊之前，使用2010年修订的工作组标准来指导初步诊断。
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