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

Published online by Cambridge University Press:  17 October 2018

Mira Trivedi ,
Amy Goldstein  and
Gaurav Arora
Mira Trivedi*
Affiliation:
Department of Cardiology, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA
Amy Goldstein
Affiliation:
Department of Neurology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Gaurav Arora
Affiliation:
Department of Cardiology, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA
*
Author for correspondence: M. Trivedi, MD, Department of Pediatrics, Children’s Hospital of Pittsburgh of UPMC, 4401 Penn Ave, Pittsburgh, PA 15224, USA. Tel: 937 367 5996; Fax: 412 692 5138; E-mail: [email protected]
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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 syndromepacemakerconductionpediatrics
Type
Brief Report
Information
Cardiology in the Young , Volume 28 , Issue 12 , December 2018 , pp. 1487 - 1488
Copyright
© Cambridge University Press 2018 

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