[Right ventricular dysplasia]

Right ventricular dysplasia (RVD) is characterized by partial or total replacement of part of the right ventricular musculature by fatty and fibrous tissue. In its typical form it presents with ventricular tachycardia, usually in the fourth decade of life. 6 men and 2 women (mean age 55.6 years), referred for evaluation of arrhythmias or other cardiac symptoms, were diagnosed as having RVD on echocardiography after other causes of right ventricular enlargement were excluded. The mean age was 55.6 years, older than originally reported. 5 presented with supraventricular arrhythmias, including atrial flutter, atrial fibrillation, supraventricular tachycardia and sick-sinus syndrome. Only 2 had ventricular tachycardia; in 1 patient no arrhythmia was found. We conclude that RVD includes a wide spectrum of arrhythmias, of which ventricular tachycardia is probably not the most common. The incidence of RVD in the older population may be greater than originally reported, and may include a slowly developing form of the disease. In some cases RVD may be the pathophysiological basis of lone atrial fibrillation and sick-sinus syndrome.