ARRHYTHMOGENIC RIGHT-VENTRICULAR DYSPLASIA - A REVIEW

Arrhythmogenic right ventricular (RV) dysplasia is a predominant disor der of the RV, characterized by progressive replacement of myocardium by fibroadipose tissue and arrhythmic manifestations. It remains incom pletely known despite the fact that it may cause sudden death, often e xertional, in young and healthy individuals. The etiology is speculati ve and the disorder has autosomal dominant genetic predisposition. Com monly, the physical examination is unremarkable and the commonest abno rmality on routine ECG is inverted T-waves on precordial leads. Morpho logic abnormality of dysplastic RV can be detected by echocardiogram, radionuclear angiogram or contrast RV angiogram. They may miss cases w ith less involvement where gated MRI scans may be useful. Patients wit h syncope or symptomatic ventricular tachycardia have worse prognosis. Pharmacologic antiarrhythmic therapy is sometimes disappointing and v arious surgical and endocardial ablation procedures have been tried wi th varying success.