A T-Wave Tight Spot

Abstract

Presyncopal symptoms are common and usually benign in children. Suspicion should be increased when these symptoms occur in relation to exertion as this may represent significant underlying pathology, including an inherited arrhythmia or cardiomyopathy. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an uncommon inherited cardiomyopathy associated with mutations in genes encoding desmosomal proteins. It is characterised by fibro-fatty replacement of myocardial tissue, cardiac failure, ventricular arrhythmias and sudden cardiac death. Diagnostic criteria include resting ECG abnormalities and demonstration of abnormal myocardial tissue and impaired ventricular function by means of diagnostic imaging; however, detection can be very difficult in the early stages and echocardiography may not be sufficiently sensitive to identify subtle abnormalities. We describe the case of a child presenting with exertional-related presyncope and an abnormal resting 12-lead ECG in whom the diagnosis was missed until he presented with a broad complex tachycardia.