Ann Thorac Surg 2001;72:1391-1392
© 2001 The Society of Thoracic Surgeons
a Department of Thoracic, Cardiac, and Vascular Surgery, Tübingen University Hospital, Tübingen, Germany
b Pediatric Intensive Care Unit, Tübingen University Hospital, Tübingen, Germany
c Department of Pediatric Cardiology, Tübingen University Hospital, Tübingen, Germany
d Zentrum für Stoffwechseldiagnostik, Reutlingen, Germany
Accepted for publication October 30, 2000.
Address reprint requests to Dr Heinemann, Department of Pediatric Cardiac Surgery, University Hospital, Langenbeckstr 1, D-55131 Mainz, Germany
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.
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