Korean J Med.  2006 May;70(5):564-568.

A case of KearnsSayre syndrome complicated with complete AV block

Affiliations
  • 1Department of Internal Medicine and Research Institute, Yonsei Universitiy College of Medicine, Seoul, Korea. cby6908@yumc.yonsei.ac.kr

Abstract

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.

Keyword

Kearns-Sayre syndrome; Complete atrioventricular block; Cardiac pacemaker

MeSH Terms

Atrioventricular Block*
Child
DNA, Mitochondrial
Electrocardiography
Female
Humans
Kearns-Sayre Syndrome
Pigmentation
Retinaldehyde
Syncope
Young Adult
DNA, Mitochondrial
Retinaldehyde
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