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Ventricular septal defect closure in a neonate with combined methylmalonic aciduria/homocystinuria
M K Heinemann1, M Tomaske, F K Trefz
1Department of Thoracic, Cardiac, and Vascular Surgery, Tübingen University Hospital, Germany. heinemann@uni-mainz.de
The Annals of Thoracic Surgery
|October 18, 2001
Abstract:
Methylmalonic acidemia with associated homocystinuria is a rare inborn error of amino acid metabolism affecting energy supply on the cellular level. Its effects on recovery from surgically induced organ ischemia are largely unknown. We report the successful closure of a nonrestrictive ventricular septal defect by following a normothermic strategy combined with ample metabolic substrate supply.