Sattar Yasar, Abdullah Hafez Mohammad, Neisani Samani Elham, Myla Madhura, Ullah Waqas
Internal Medicine, Icahn School of Medicine at Mount Sinai, New York, USA.
Internal Medicine, University of South Dakota Sanford School of Medicine, Sioux Falls, USA.
Cureus. 2019 Aug 13;11(8):e5381. doi: 10.7759/cureus.5381.
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition caused by the replacement of the normal right ventricular myocardium with fibrofatty tissue. ARVC/D can present with a variety of clinical conditions including right ventricular dysfunction, ventricular tachyarrhythmias, sudden cardiac arrest, and sudden cardiac death (SCD). Since the first report of ARVC/D in 1982, many advances have been made in the diagnosis, genetic findings for pathology, and treatment. The 2010 International Task Force diagnostic criteria distinguish between major and minor criteria and focus on gross structural changes, microscopic changes, repolarization defects, conduction defects, arrhythmias, and family history. Implantable cardiac defibrillators and catheter ablation of the endocardium and epicardium with electromagnetic mapping have emerged as successful tools in the treatment and prevention of ventricular tachyarrhythmias and SCD. This review discusses the pathophysiology, genetics, diagnosis, and treatment advances in ARVC/D.
致心律失常性右室心肌病/发育不良（ARVC/D）是一种因正常右室心肌被纤维脂肪组织替代而引起的病症。ARVC/D可表现为多种临床情况，包括右室功能障碍、室性快速心律失常、心脏骤停和心源性猝死（SCD）。自1982年首次报道ARVC/D以来，在诊断、病理遗传学发现及治疗方面已取得诸多进展。2010年国际工作组诊断标准区分了主要标准和次要标准，并着重关注大体结构改变、微观改变、复极异常、传导异常、心律失常及家族史。植入式心脏除颤器以及采用电磁标测进行的心内膜和心外膜导管消融术已成为治疗和预防室性快速心律失常及SCD的成功手段。本综述讨论了ARVC/D的病理生理学、遗传学、诊断及治疗进展。
