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Cardiol Young. 2018 Dec;28(12):1487-1488. doi: 10.1017/S1047951118001609. Epub 2018 Oct 17.

Prophylactic pacemaker placement at first signs of conduction disease in Kearns-Sayre syndrome.

Author information

1
1Department of Cardiology,Children's Hospital of Pittsburgh of UPMC,Pittsburgh, PA,USA.
2
2Department of Neurology,Children's Hospital of Philadelphia,Philadelphia, PA,USA.

Abstract

Cardiac conduction disease affects patients with Kearns-Sayre syndrome. We report a young asymptomatic patient with Kearns-Sayre syndrome with abnormal conduction on electrocardiogram and Holter monitor, although not advanced atrioventricular block. She underwent prophylactic pacemaker placement, and rapidly developed complete atrioventricular block, which resulted in 100% ventricular pacing. It may be reasonable to consider prophylactic pacemaker implantation in patients with Kearns-Sayre syndrome with evidence of cardiac conduction disease even without overt atrioventricular block given its unpredictable progression to complete atrioventricular block.

KEYWORDS:

Kearns–Sayre syndrome; conduction; pacemaker; pediatrics

PMID:
30326976
DOI:
10.1017/S1047951118001609

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