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Electrophysiological findings in patients with kearns sayre syndrome report of 2 cases

, : Electrophysiological findings in patients with kearns sayre syndrome report of 2 cases. Zeitschrift fuer Kardiologie 74(5): 281-286

The Kearns-Sayre Syndrome is identified by the triad of progressive external ophthalmoplegia, atypical pigmentary retinopathy and conduction disturbances. Clinical manifestations may include mental retardation, sensorineural deafness, cerebellar ataxia and facial and peripheral muscle weakness. Morphologic alterations in skeletal muscle may be characterized by ragged-red fibers. Two patients with Kearns-Sayre Syndrome underwent electrophysiological examination. The 1st patient had a 1st and 2nd degree AV [atrioventricular] block (Mobitz type II), right bundle branch block and left axis deviation. The His-bundle electrogram showed a prolonged HV interval as a hint at an intraventricular conduction delay. The signal-averaging technique and endocardial mapping revealed ventricular after-depolarizations. The 2nd patient had an unsuspicious ECG, recurrent atrial tachycardias, normal atrial and ventricular conduction and after-depolarizations in endocardial mapping. Two mo. later he showed a 2nd degree AV block combined with clinical symptoms. Pacemakers were implanted in both patients. Beside disturbances of the conduction system in both patients signs of electrical instability of the myocardium were found. In this way the Kearns-Sayre syndrome may be seen as a form of cardiomyopathy.

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