Affiliation:
1. Cardiology Department Baker Heart and Diabetes Institute Melbourne Victoria Australia
Abstract
AbstractIntroductionArrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disease characterised by progressive fibrofatty tissue replacement of the myocardium. Several arrhythmogenic cardiomyopathy (ACM) phenotypes are now recognised, including right‐dominant, biventricular, and left‐dominant variants, which led to the development of the 2020 International criteria (Padua criteria).Raising awareness of these variants is crucial as their clinical entity can be concealed. This is particularly important in the athletic population. Exercise can promote the development of the phenotype and accelerate disease progression, resulting in left ventricular (LV) involvement and systolic dysfunction, which can cause ventricular arrhythmias and sudden death.Case DescriptionA late adolescent male was escorted by paramedics to an emergency department following a medium‐speed motor vehicle accident. A multimodality approach was implemented involving a 12‐lead electrocardiogram (ECG), transthoracic echocardiography (ECHO) and cardiac magnetic resonance (CMR) imaging. An unknown cardiomyopathy was revealed with diagnostic clues suggesting biventricular ACM in a recreational soccer player, when using the Padua criteria.ConclusionACM should be considered in the evaluation of an unexplained cardiomyopathy presenting with possible syncopal events. This case describes the clinical features of an ACM patient, emphasising the utility of ECG, ECHO and CMR in determining biventricular involvement.CMR is a powerful tool for the diagnosis of ACM and the identification of myocardial fibrosis with late gadolinium enhancement (LGE). The use of 12‐lead ECG and 2‐dimensional (2D) strain imaging may also raise suspicion of biventricular phenotypes and predict LV involvement with significant myocardial fibrosis.
Subject
Radiology, Nuclear Medicine and imaging
Cited by
1 articles.
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