Acute Myocarditis as the Initial Presentation of Desmoplakin Mutation – Broadening the Differential Diagnosis

Introduction

Genetic cardiomyopathy related to desmoplakin (DSP) mutation can start its clinical manifestation with chest pain symptoms, along with transient troponin elevation configuring first manifest as acute myocarditis, sometimes in a relapsing pattern. It is clear that the revised Task Force criteria for genetic cardiomyopathy will miss many cases, particularly those with left-sided dominance. This case reports highlights to understand that genetic cardiomyopathy, particularly with DSP mutation, can present with acute myocarditis and to understand that cardiovascular magnetic resonance (CMR) imaging could raise the possibility of arrhythmogenic cardiomyopathy and predict ventricular tachyarrhythmias despite preserved LVEF.

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