[Arrhythmogenic dysplasia of the right ventricle: clinical presentation and long-term course].
The clinical findings and follow up in 10 patients with arrhythmogenic dysplasia of the right ventricle were analyzed. Presentation occurred at a mean age of 35 +/- 12(SD) years and all patients presented with ventricular tachycardia and a left bundle branch block pattern. Physical examination was normal. T waves were inverted in right precordial leads in all and late depolarization were seen in 7. Global or segmental dilatation of the right ventricle was shown by echocardiogram in all patients and by right ventricular angiography in 8. After a mean follow up of 6.6 years (+/- 3), 70% had recurrence of ventricular tachycardia in spite of adequate antiarrhythmic therapy. Mortality was only 10%. Surgery was performed in 4 patients. Two patients had focal right ventricular fibrosis and resection lead to cure of arrhythmia.