Kearns-sayre Syndrome Treated with Permanent Pacemaker Insertion for Complete Atrioventricular Block.
10.7180/kmj.2017.32.1.133
- Author:
Eun Hye PARK
1
;
Sung Ho HER
;
Myung A HA
;
Hyo Suk KIM
;
Jae Hyuk JANG
Author Information
1. Division of Cardiology, College of Medicine, the Catholic University of Korea, Seoul, Korea. hhhsungho@naver.com
- Publication Type:Case Report
- Keywords:
Complete AV block;
Kearns-Sayre syndrome;
Permanent pacemaker
- MeSH:
Atrioventricular Block*;
Biopsy;
Bundle-Branch Block;
Death, Sudden;
Heart Block;
Humans;
Kearns-Sayre Syndrome*;
Mitochondrial Diseases;
Ophthalmoplegia, Chronic Progressive External;
Retina;
Syncope
- From:Kosin Medical Journal
2017;32(1):133-138
- CountryRepublic of Korea
- Language:English
-
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.
