The Second Department of Internal Medicine, University of Toyama, Japan.
Intern Med. 2022 Oct 15;61(20):3063-3067. doi: 10.2169/internalmedicine.8911-21. Epub 2022 Mar 26.
Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) classically present with ventricular arrhythmias and less commonly heart failure. ARVC is an inherited cardiomyopathy and generally based on a variant of desmosomal genes. Recently, the association between myocardial inflammation and ARVC has been a matter of great concern. We encountered a patient with ARVC who had a desmoglein-2 mutation with advanced right ventricular failure accompanying a preserved left ventricular function. Concomitant right ventricular myocarditis was detected four years after the diagnosis of ARVC. ARVC and myocarditis might have a deep pathophysiological association, at least in some cases.
致心律失常性右室心肌病（ARVC）患者通常表现为室性心律失常，较少出现心力衰竭。ARVC 是一种遗传性心肌病，通常基于桥粒蛋白基因的变异。最近，心肌炎症与 ARVC 之间的关联备受关注。我们遇到一位 ARVC 患者，其存在桥粒蛋白-2 突变，表现为晚期右心衰竭，同时左心室功能正常。在 ARVC 诊断四年后，发现合并右心室心肌炎。ARVC 和心肌炎之间至少在某些情况下存在深刻的病理生理学关联。
