Anesthetic and Cardiopulmonary Bypass Considerations for Cardiac Surgery in Unique Pediatric Patient Populations: Sickle Cell Disease and Cold Agglutinin Disease

Abstract

Physiological disturbances induced by cardiopulmonary bypass (CPB) and hypothermia during cardiac surgery are particularly pronounced in certain unique patient populations, such as patients with sickle cell disease (SCD) and cold agglutinin disease. Red blood cells containing hemoglobin S (HbS) are at increased risk of sickling under conditions encountered during cardiac surgery, leading to SCD-related complications such as vaso-occlusive events. While a target level of HbS has not been determined for patients with SCD undergoing CPB, a safe practice includes increasing the Hb level to 10 g/dL and reducing the proportion of HbS to approximately 30%. This can be accomplished through simple or exchange transfusion prior to surgery or via the modification of the CPB circuit prime. There is no clear consensus on the formulation or the delivery temperature of the cardioplegia solution necessary to prevent sickling and microvascular occlusion. The presence of cold agglutinins is another entity requiring extra vigilance for the conduct of CPB, where hypothermia can lead to activation of cold agglutinins inducing massive hemagglutination, hemolysis, microvascular thrombosis, and possibly intracoronary thrombosis. Determination of thermal amplitude is important to provide a safe reference range of temperature during surgery. High-volume plasmapheresis may be warranted to reduce cold agglutinin titers. Both warm blood cardioplegia and cold crystalloid cardioplegia above the thermal amplitude have been utilized with success.