Ventricular septal defect closure in a neonate with combined methylmalonicaciduria/homocystinuria

Methylmalonic acidemia with associated homocystinuria is a rare inborn erro r of amino acid metabolism affecting energy supply on the cellular level. I ts effects on recovery from surgically induced organ ischemia are largely u nknown. We report the successful closure of a nonrestrictive ventricular se ptal defect by following a normothermic strategy combined with ample metabo lic substrate supply. (C) 2001 by The Society of Thoracic Surgeons.