心律不整性右心室心肌病變/發育不全是一種罕見疾病，主要是以心室性心律不整，突發性心因性死亡來表現而較少用右心衰竭的方式來表現。根據近期文獻報告，不到百分之十是以右心衰竭來表現。本文報告一例42歲女性，因長期咳嗽，喘及體重減輕就醫。經檢查之後，意外發現此病患是一位以非典型症狀，右心室肥大合併右心衰竭呈現之右心室變異個案。是故當病人以右心衰竭的症狀來就醫時，急診的醫師應將此疾病列入鑑別診斷。

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a rare disease with the predominant presenting symptoms of ventricular arrhythmias, sudden cardiac death or uncommonly, right ventricular heart failure. Right ventricular heart failure is uncommon as a presenting symptom, accounting for fewer than 10% of live index cases in a recent series. Here, we present a case that serves to illustrate an unexpected presentation of ARVC/D with the manifestation of right ventricular failure. This patient died of multiple organ failure after 25 days of hospitalization with the support of extracorporeal membrane oxygenation. Emergency physician should always take ARVC/D into consideration when facing of life-threatening right ventricular heart failure, early consultation and support using extracorporeal membrane oxygenation ECMO for unstable hemodynamic status should be mandatory.