ARRHYTHMOGENIC RIGHT-VENTRICULAR DISEASE

In patients with arrhythmogenic right ventricular disease (ARVD), life threatening ventricular tachyarrhythmias and sudden cardiac death most ly occur in adolescence, or in young adults before the age of 40. In t he right ventricle, progressive fibrolipomatous replacement of the ven tricular myocardium is pathognomonic. In severe cases progressive cong estive right heart failure can develop although mild forms are extreme ly difficult to recognized. In most cases the disease can be diagnosed only by elaborate investigation. Right ventricular cineangiography, m yocardial biopsy, MRI, and electrophysiological investigation are the most important diagnostic procedures. If the disease is diagnosed in a n early stage the risk of life-threatening arrhythmias can be reduced by carefully selected antiarrhythmic therapy.