A man complaining of palpitations was found to have ventricular tachycardia (VT) with LBBB configuration. From the investigations which followed, he was diagnosed as having arrhythmogenic right ventricular dysplasia (ARVD). The patient has been treated with amiodarone and propafenon for 7 months without VT recurrence. ARVD and Uhl's anomaly, which is its most extreme form, may be familial and represent an important cause of sudden death among young people: Prophylactic antiarrhythmic therapy and sometimes surgical treatment are required in case of refractory VT.
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