Unusual cause of syncope in young girl: arrhythmogenic right ventricular cardiomyopathy

A 15-year-old girl was admitted because of syncope during handball match. On examination the patient was oriented in every condition; blood pressure was 80/50 mmHg and her pulse around 190 beats/min. Electrocardiogram (Fig. 1a) revealed ventricular tachycardia (VT) with left bundlebranch QRS pattern and superior axis. ECG recorded under stable clinical conditions displayed significant repolarization abnormalities and negative T-waves in almost all precordial leads (Fig. 1b). After i.v. administration of Amiodarone VT converted to sinus rhythm. Bedside transthoracic echocardiography showed a severely hypokinetic dilated right ventricle (RV) and dilation of the right ventricular outflow tract (Fig. 1c–e). Left ventricular systolic function was normal. Cardiac MR (CMR) showed dilation of the RV with extreme thinning of the RV myocardium (Fig. 1f) and signs of myocardial late enhancement (not shown). Initial evaluation of our patient focused on structural (e.g. hypertrophic cardiomyopathy) or primary electrical (long QT syndrome, Brugada syndrome, etc.) causes of syncope [1]. However, symptoms, ECG and imaging procedures suggested diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC). An implantable defibrillator (ICD) was implanted due to prognostic reasons after sustained VT. In follow-up visits our patient has not shown any signs of heart failure. ICD delivered one episode of successful antitachycardia pacing (ATP) and one ICD shock due to VT. Screening (echocardiography, ECG, exercise testing) of parents and siblings has not shown any cardiac abnormalities.