Torsades de pointes in a patient with Kearns-Sayre syndrome: a fortunate finding

We present a case of a 33-year-old woman with Kearns-Sayre syndrome (KSS) who had a pacemaker implanted for complete heart block postpartum and was found to have torsades de pointes. KSS is a rare encephalomyopathy associated with varying levels of central nervous system involvement, typically resulting in progressive external opthalmoplegia and retinal degeneration. Onset of cardiac conduction disease can be insidious and is a strong predictor of sudden cardiac death. The mainstay of treatment has been the judicious implantation of pacemakers. However, as highlighted in this case, patients who have an underlying cardiomyopathy may be more appropriately treated with an implantable cardioverter defibrillator.