Thomas Shon, Jackubowski Jaclynn, Ismair Kevin, Chang Jeremiah, Kuhnlein Jerome
Corewell Health South, Saint Joseph, MI, USA.
J Community Hosp Intern Med Perspect. 2025 Jan 6;15(1):70-74. doi: 10.55729/2000-9666.1432. eCollection 2025.
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a right ventricular disease caused by desmosomal gene mutations leading to fibro-fatty replacement of the myocardium causing ventricular arrhythmias such as ventricular tachycardia (VT). A 59-year-old female presented with new onset VT manifesting as shortness of breath and chest discomfort. Diagnostic workup revealed right ventricular dilation/dysfunction on echocardiogram, VT with left bundle branch block (LBBB) and diffuse T wave inversions (TWIs) on EKG. She was discharged with a diagnosis of ARVC. Later, she developed VT storm, treated with epi-endocardial ablation for complete resolution of recurrent VT. Our case demonstrates the rare presentation of ARVC as ventricular storm and the various management strategies such as anti-arrhythmics, automated implantable cardioverter-defibrillator (AICD) and ablation procedures.
致心律失常性右室心肌病（ARVC）是一种由桥粒基因突变引起的右室疾病，导致心肌纤维脂肪替代，引发室性心律失常，如室性心动过速（VT）。一名59岁女性因新发室性心动过速就诊，表现为呼吸急促和胸部不适。诊断检查显示，超声心动图提示右室扩张/功能障碍，心电图显示室性心动过速伴左束支传导阻滞（LBBB）及弥漫性T波倒置（TWIs）。她出院时被诊断为ARVC。后来，她发生了室性心动过速风暴，接受了心内膜消融治疗，使复发性室性心动过速完全缓解。我们的病例展示了ARVC罕见地表现为室性风暴，以及诸如抗心律失常药物、植入式自动心脏复律除颤器（AICD）和消融手术等各种治疗策略。
