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Abstract
Kearns-Sayre (K-S) syndrome is a multisystemic disorder initially described as chronic progressive external ophthalmoplegia associated with pigmentary degeneration of the retina (retinitis pigmentosa) and complete heart block. 1 Apparently, this entity is caused by mitochondrial abnormalities that affect neuromuscular and cardiac conduction systems. 2 The sequence in which this disorder affects the different organs varies, but usually cardiac involvement and atrioventricular (AV] block develop late.2 We report the clinical and pathologic findings of the heart and its conduction system in 2 patients with K-S syndrome.
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