关键词: Arrhythmogenic right ventricular cardiomyopathy Cardiac magnetic resonance Prognosis

Mesh : Humans Arrhythmogenic Right Ventricular Dysplasia / diagnosis physiopathology Retrospective Studies Male Female Adult Magnetic Resonance Imaging, Cine / methods Electrocardiography Prognosis Pakistan / epidemiology Middle Aged Young Adult Death, Sudden, Cardiac / epidemiology etiology prevention & control Prevalence Adolescent

来  源:   DOI:10.1016/j.cpcardiol.2024.102630

Abstract:
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a hereditary condition with a prevalence ranging from 1 in 2000 to 1 in 5000 individuals. ARVC is a significant contributor to sudden cardiac death, particularly in young individuals and athletes, and remains challenging to diagnose definitively. We conducted a single-center retrospective study to evaluate the presentations, electrocardiogram findings, and imaging characteristics of ARVC patients evaluated at our center between 2021 and 2023. Notably, our study is the second investigation of ARVC conducted in Pakistan. We report divergent symptom prevalence as compared to the current literature and have incorporated the Task Force Criteria. Despite limited access to cardiac magnetic resonance (CMR) facilities worldwide, our findings underscore the critical role ofCMR in ARVC diagnosis. Our cohort had a mortality rate of 17 % highlighting the importance of early detection and the need for improved diagnostic facilities for ARVC in the region.
摘要:
致心律失常性右心室心肌病(ARVC)是一种遗传性疾病,患病率从2000年1人到5000人中1人不等。ARVC是导致心源性猝死的重要因素,特别是年轻人和运动员,并且仍然难以明确诊断。我们进行了一项单中心回顾性研究来评估报告,心电图检查结果,2021年至2023年在我们中心评估的ARVC患者的影像学特征。值得注意的是,我们的研究是在巴基斯坦进行的ARVC的第二次调查。与当前文献相比,我们报告了不同的症状患病率,并纳入了工作组标准。尽管全球范围内使用心脏磁共振(CMR)设施的机会有限,我们的发现强调了CMR在ARVC诊断中的关键作用.我们的队列死亡率为17%,突显了早期发现的重要性以及该地区ARVC诊断设施的改进需求。
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