Mitochondrial encephalomyopathy (Kearns-Sayre syndrome) with complete atrioventricular block: - A case report-

Abstract

A pacemaker was implanted into a 17-year-old man with cardiac failure due to complete atrioventricular block complicated by mitochondrial encephalomyopathy (Kearns-Sayre syndrome). Due to the possible complication of latent myocardial dysfunction, it was decided to implant the dual chamber pacemaker (DDD) and the operation mode was set to DDD 70ppm 1 year after implantation; this alleviated the cardiac failure. In this case, the necessity of preventive pacemaker implantation in the early stage of cardiac failure was recognized.