Adipose dysplasia of the right ventricle: is endomyocardial biopsy useful?

Abstract

Right ventricular dysplasia (RVD) is a cardiac anomaly characterized by replacement of right ventricular myocardium by adipose and fibrous tissue that was first described at autopsy, and is now being recognized pre-mortem. Sudden death and ventricular arrhythmias are the most common clinical manifestation of the condition. This paper reviews the literature on myocardial biopsies performed in patients with idiopathic arrhythmias who had pathologic findings consistent with RVD, and the results of biopsies from patients suspected of having RVD. The significance of sampling error in myocardial biopsies, possible familial associations, and other conditions associated with myocardial adipose infiltration are also discussed. Based on the review, the possibility is raised that RVD may represent the end result of an unusual healing process, whereby genetically susceptible individuals recover from a variety of RV insults by deposition of adipose tissue. These fatty scars may then be the focus for arrhythmias which remain the major clinical manifestation of the syndrome.