Key result
An 18-year-old male presenting with syncope during a basketball match was diagnosed with advanced arrhythmogenic right ventricular cardiomyopathy with biventricular involvement and successfully treated with a subcutaneous defibrillator.
Case Report (n=1)
No
This case highlights the importance of multimodality imaging in diagnosing advanced ARVC with biventricular involvement in a young athlete presenting with syncope, and the unmasking of an epsilon wave post-defibrillation.
Supports S-ICD feasibility in young ARVC with syncope; hypothesis-generating and should not yet change practice.
We reported a case of a young adult male aged 18 years admitted in our institution for syncope during a basketball match. No previous symptoms were reported. Electrocardiogram (ECG) showed T-wave inversion in the anterior leads and an incomplete right bundle branch block. Surprisingly, a complete echocardiographic evaluation demonstrated the presence of severe right ventricular enlargement with significant wall motion abnormalities, apical aneurysm and reduced systolic function. Cardiac Magnetic Resonance was pathognomonic for a fibro-fatty replacement of both ventricles. We decided for a subcutaneous defibrillator implantation and, after inducing a ventricular fibrillation to test the device status, epsilon wave appeared on the ECG. This clinical scenario depicted an advanced arrhythmogenic right ventricular cardiomyopathy at its first clinical manifestation.
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Brandimarte et al. (2019) conducted a case report in Arrhythmogenic right ventricular cardiomyopathy (n=1). Subcutaneous defibrillator (ICD) implantation was evaluated. An 18-year-old male presenting with syncope during a basketball match was diagnosed with advanced arrhythmogenic right ventricular cardiomyopathy with biventricular involvement and successfully treated with a subcutaneous defibrillator.
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