Open-heart surgery in sickle-cell haemoglobinopathies: report of 15 cases.

Fifteen cases of open-heart surgery in patients with sickle-cell haemoglobinopathies are reported; 13 had sickle-cell trait, one had SC haemoglobinopathy, and one had β-thalassaemia sickle-cell disease. All patients except one were operated on with moderate hypothermia, aortic cross-clamping, topical hypothermia, and cold cardioplegia. A bloodless priming solution was used in nine patients and five did not receive any blood throughout their hospital stay. Arterial and venous blood gas analysis and a search for sickle cells and haemolysis were carried out during and after cardiopulmonary bypass. The data were compared with the findings in a group of 29 patients without haemoglobinopathy operated on without blood transfusion. Two patients died from low cardiac output, unrelated to the haemoglobinopathy. All other patients recovered uneventfully. Sickling occurred during and after bypass in only one case, and the percentage of sickle cells was considerably lower during and after surgery than before. Haemolysis occurred only once during cardiopulmonary bypass and twice after surgery (the two deaths from low cardiac output). There was no acidosis or hypoxia. There was no difference in the loss of haemoglobin between the 13 survivors and the control group. Our data suggest that adequate oxygenation and avoidance of acidosis and dehydration during surgery are important. On the other hand, we do not believe that preoperative transfusion or exchange transfusion, a blood prime, normothermia, and the avoidance of aortic cross-clamping or topical hypothermia are essential precautions. We believe that transfusion should be used during cardiopulmonary bypass only for severely anaemic patients. The technique used in our cases adds to the safety of the procedure and improves the protection of the myocardium.