Dilated cardiomyopathy with cardiogenic shock in a child with Kearns-Sayre syndrome

Swati Sehgal, Swati Choudhry, Larisa Debelenko, Thomas L'Ecuyer

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Kearns-Sayre syndrome (KSS) is a mitochondrial myopathy resulting from mitochondrial DNA deletion. This syndrome primarily involves the central nervous system, eyes, skeletal muscles and the heart. The most well-known cardiac complications involve the conduction system; however, there have been case reports describing cardiomyopathy. We describe a case of a child with KSS who presented with decompensated cardiac failure from dilated cardiomyopathy representing cardiomyocyte involvement of KSS. Our patient had a rapidly progressing course, despite maximal medical management, requiring emergent institution of extracorporeal membrane oxygenation and transition to a ventricular assist device. To the best of our knowledge, this is the youngest patient in the literature to have dilated cardiomyopathy in KSS.

Original languageEnglish
Article number213813
JournalBMJ Case Reports
StatePublished - Feb 16 2016


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