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

Abstract

SummaryWe 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.

Open Heart Surgery in Patients With Sickle Cell Hemoglobinopathy

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] Such conditions lead to potassium efflux, causing formation of insoluble globin polymers. These molecules increase the viscosity of blood and lead to vasoocclusive phenomena, which include cell sickling, adherence of sickle cells to the endothelium, and vasoocclusion.…”

“…8 -11 Although the risks accompanying sickle cell pathology before high-risk surgery, including orthopedic and cholecystectomy, have been well stated, 9 the literature contains only small series or case reports on sickle cell patients having cardiac surgery. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] It should be noted that above-mentioned predisposing conditions are more common in patients undergoing cardiac surgery. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Especially during the operation, CPB itself, as well as aortic cross-clamping, low-flow states, topical or whole-body hypothermia, cold cardioplegia, and use of vasoconstrictive agents, may predispose to the crisis state.…”

“…2,3,[5][6][7][8][9][10][11][12][13][14][15][16] On the other hand, CPB itself is also a system that facilitates exchange transfusion, and using blood for priming the circuits can reconcile the aim. 2,6,14,15 Blood transfusion is one of the major issues during any kind of surgery. Generally, autologous transfusion is preferred, but preoperative phlebotomy or the use of cell-saver systems during surgery could reduce the need for homologous blood transfusion.…”

“…This can be avoided by applying "topical-only" cooling or achieving moderate hypothermia, but totally warm CPB is another valid option mentioned frequently in the literature. 2,3,[5][6][7]14,15 In coronary surgery, the endomyocardial capillaries are at particular risk of occlusion by the sickling phenomena when exposed to cold cardioplegia. This can be prevented with the initial institution of warm cardioplegia to wash out the existing blood in coronary arteries, followed by cold or designated cardioplegia according to the protocols of the operating team.…”

Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children

“…Patients with CHD and SCD have tolerated cardiopulmonary bypass in the short term. 9,10 The longest outcome reported before our series involved the 6-month survival of a neonate who had tricuspid atresia and Hb SC disease. 11 Three of our patients had overt cerebrovascular events-unsurprising, given that CHD and SCD are risk factors for pediatric CVA.…”