Acute Myocardial Infarction-Like Events in Related Patients With a Desmoplakin-Associated Arrhythmogenic Cardiomyopathy

Abstract

Patients with familial arrhythmogenic cardiomyopathy typically present with ventricular arrhythmias or progressive heart failure. This paper characterizes a rare presentation of an underlying genetic cardiomyopathy with clinical manifestations mimicking an acute myocardial infarction in 2 siblings, each with the same mutation in the desmoplakin (DSP) gene. (Level of Difficulty: Advanced.).

Keywords: ACM; ACM, arrhythmogenic cardiomyopathy; CMR, cardiac magnetic resonance; CP, chest pain; DSP, desmoplakin; ECG, electrocardiogram; LGE, late gadolinium enhancement; LV, left ventricle; PVC, premature ventricular contraction; RV, right ventricle; TnI, troponin I; genetic cardiomyopathy; heart failure; myocardial injury; sudden cardiac death; troponin I.