Arrhythmogenic right ventricular dysplasia: a review.
Arrhythmogenic right ventricular (RV) dysplasia is a predominant disorder of the RV, characterized by progressive replacement of myocardium by fibroadipose tissue and arrhythmic manifestations. It remains incompletely known despite the fact that it may cause sudden death, often exertional, in young and healthy individuals. The etiology is speculative and the disorder has autosomal dominant genetic predisposition. Commonly, the physical examination is unremarkable and the commonest abnormality on routine ECG is inverted T-waves on precordial leads. Morphologic abnormality of dysplastic RV can be detected by echocardiogram, radionuclear angiogram or contrast RV angiogram. They may miss cases with less involvement where gated MRI scans may be useful. Patients with syncope or symptomatic ventricular tachycardia have worse prognosis. Pharmacologic antiarrhythmic therapy is sometimes disappointing and various surgical and endocardial ablation procedures have been tried with varying success.
Dalal
P
P
Department of Cardiology, Beth Israel Medical Center, New York, N.Y. 10003.
Fujisic
K
K
Hupart
P
P
Schwietzer
P
P
D001145:Arrhythmias, Cardiac / Q000209:etiology*; D004452:Echocardiography; D004562:Electrocardiography; D006801:Humans; D017380:Hypertrophy, Right Ventricular / Q000150:complications* / Q000473:pathology; D018497:Ventricular Dysfunction, Right / Q000150:complications* / Q000175:diagnosis