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European Heart Journal 1989 10(3):281-282;
Copyright © 1989 by the European Society of Cardiology.
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© 1989 The European Society of Cardiology

Indications for pacemaker implantation in the Kearns-Sayre syndrome

P. E. POLAK, F. ZULSTRA and J. R. T. C. ROELANDT

The Catheterization Laboratory Thoraxcenter, Academic Hospital Rotterdam Dijkzigt, Erasmus University Rotterdam the Netherlands

Received 22 April 1988; revised 28 July 1988; .

Address for correspondence: Peter E. Polak, M.D., Catheterization Laboratory, Thoraxcenter, Erasmus University, P.O. Box 1738, 3000 DR Rotterdam, the Netherlands.

Abstract

The Kearns-Sayre syndrome is a rare condition, characterized by progressive external ophthalmoplegia, retinal pigmentary degeneration and progressive impairment of cardiac conduction, which mainly determines the prognosis. Two young patients (aged 13 and 18 years) without symptoms of cardiac disease presented with an electrocardiogram showing sinus rhythm, a normal atrio-ventricular conduction time, right bundle branch block and a left anterior fascicular block.Electrophysiologic investigation showed prolongation of His-ventricular interval at rest, which further increased during atrial pacing. Because of the potential progression of the conduction abnormalities and threatening sudden death, we decided to implant a pacemaker in both patients. Ten months later one patient had become pacemaker-dependent. Prophylactic pacemaker therapy is advisable in patients suffering from the Kearns-Sayre syndrome, who have bifascicular block on theprecordial electrocardiogram.

Key Words: Kearns-Sayre syndrome • pacemaker therapy


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