[Surgical correction of interventricular communication with pulmonary stenosis in a hemophiliac]

Open heart surgery was performed in a 5 year-old boy with severe hemophilia A and large ventricular septal defect with pulmonary stenosis. High doses of Factor VIIIC superconcentrates delivered as small transfusion volumes allowed the use of extracorporeal circulation with heparin. Transfusions began 5 hours before surgery and were stopped 21 days later. No adverse reaction was observed. Thus major surgery is possible in severe hemophiliacs provided strict rules for replacement therapy are followed.