Kosin Med J.  2017 Jun;32(1):133-138. 10.7180/kmj.2017.32.1.133.

Kearns-sayre Syndrome Treated with Permanent Pacemaker Insertion for Complete Atrioventricular Block

• Park EH
• Her SH
• Ha MA
• Kim HS
• Jang JH

• KMID: 2384843
• DOI: http://doi.org/10.7180/kmj.2017.32.1.133

Abstract

Kearns-Sayre syndrome (KSS) is a rare multisystem mitochondrial disorder associated with progressive external ophthalmoplegia, atypical pigmentary degeneration of the retina, and complete heart block. KSS can lead to a risk of sudden death because of the potential progression of conduction abnormalities such as right or left bundle branch block or complete atrioventricular (AV) block. Here we describe the case of a KSS patient with type I diabetes who experienced syncope in the presence of complete AV block, confirmed by muscular biopsy.

Keyword

Complete AV block; Kearns-Sayre syndrome; Permanent pacemaker

MeSH Terms

Atrioventricular Block*
Biopsy
Bundle-Branch Block
Death, Sudden
Heart Block
Humans
Kearns-Sayre Syndrome*
Mitochondrial Diseases
Ophthalmoplegia, Chronic Progressive External
Retina
Syncope

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