Cardiopulmonary bypass for surgical correction of congenital heart disease in children with sickle cell disease: a case series.

We present a series of three children with sickle cell disease aged 3 months, 3 weeks and 18 months, all presenting for cardiac surgery requiring cardiopulmonary bypass. The cardiac lesions were atrioventricular septal defect, transposition of the great arteries and ventricular septal defect, with sickle cell loads of 35%, 11% and 39% respectively at presentation. We calculated that the bypass circuit would provide sufficient volume to decrease sickle cell levels to safe values, so we decided to proceed to bypass without pre-operative exchange transfusion, and modified the bypass technique so as to avoid the likely stimulants of a sickle cell crisis. Haemoglobin S levels after the start of bypass were significantly lower than before bypass, and remained low throughout the case and into the second postoperative day. By adopting this approach, we feel that we achieved a successful outcome with minimal distress to the children and their families.