Abstract
Kearns-Sayre syndrome has associated cardiac findings, predominantly complete heart block, which has been implicated as a mechanism of sudden death in these individuals. A patient with Kearns-Sayre syndrome who had syncope and multiple cardiac arrests due to ventricular tachycardia in the setting of QT prolongation is described. Long QT syndrome is implicated as another possibility for sudden death in Kearns-Sayre syndrome. This potential association is unexplained, but now totals two reports in the small numbers of patients reported.