THE KEARNS-SAYRE SYNDROME - A RARE INDICA TION FOR PROPHYLACTIC CARDIAC PACING

The authors report a case of Kearns-Sayre syndrome, a rare mitochondri al myopathy, diagnosed in a 19 year old man. Distal conduction defects are constant findings and are a dominant factor in the prognosis of t his condition. Their early appearance and rapid progression pose the p roblem of prophylactic cardiac pacing despite the young age. In the ca se report, pacing was decided at the age of 23 in the absence of sympt oms but with bifascicular block (right bundle branch block and left an terior hemiblock), without electrophysiological investigation. During follow-up, left bundle branch block was observed 3 years later and per manent complete atrioventricular block occurred 7 years after pacemake r implantation.