A case of KearnsSayre syndrome complicated with complete AV block / 대한내과학회지

KearnsSayre syndrome (KSS) is a mitochondrial DNA disorder characterized by the onset before age 20 years, progressive external opthalomoplegia, atypical retinal pigmentation and cardiac conduction disturbance. This report describes a 24-year-old woman who experienced syncope due to complete atrioventricular block complicated by KSS. At 12 years old, she was diagnosed KSS. The consecutive change of EKG shows typical progression of cardiac conduction disturbance of KSS. She was successfully treated with implantation of a pacemaker.