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Familial ventricular tachycardia with mild ventricular dysfunction: a 15 year follow up of two African American brothers with arrhythmogenic right ventricular dysplasia
  2. G CRESPO,

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Two African American brothers with arrhythmogenic right ventricular dysplasia (ARVD) were followed over a period of 15 years after their initial presentation with symptomaticventricular tachycardia (VT) while engaging in athletic activities. The younger patient was 13 years old when he experienced for the first time a fluttering in his chest during a game of football. The ECG revealed VT of left bundle branch block morphology (top). His resting ECG showed T wave inversions in precordial leads V1–V4. Furthermore epsilon waves are clearly visible as indicated by arrows (bottom). Non-compliance with medical treatment saw him return to the emergency room on more than 40 separate occasions for DC or chemical cardioversion for emergent control of VT. Radiofrequency ablation and amiodarone allowed the patient to enjoy a six month remission, but he soon became symptomatic after abandoning his medication. Serial echocardiography chronicled gradual right ventricular dilatation, although congestive heart failure was clinically absent.  The patient's older sibling was 26 years old when he presented with a prolonged episode of palpitations precipitated by a game of basketball. An emergency room ECG revealed VT of left bundle branch type. An angiography revealed a normal coronary anatomy. The initial electrophysiology study disclosed inducible VT responsive to procainamide, which was absent after the patient began treatment with this agent. With the exception of one brief period of non-compliance, this subject has remained asymptomatic. Echocardiography studies over a 10 year interval were unremarkable. To our knowledge, this is the first reported case of ARVD in African Americans.