Mitral Valve Repair Surgery in a Patient With Sickle Cell Disease

Sickle cell disease (SCD) affects between 70,000 and 100,000 Americans, making it one of the most prevalent genetic disorders in the United States. It is an autosomal, recessive disorder characterized by a single amino acid change in the betaglobin chain of hemoglobin (Hob) leading to its pathological polymerization, red cell rigidity and poor microvascular blood flow. These changes also produce the characteristic sickle shape of the red cells, which lose the pliability required to successfully traverse small capillaries. The use of cardiopulmonary bypass (CPB) during adult cardiac surgery initiates many of the conditions that contribute to the sickling of the hemoglobin S cells. Traditionally during CPB the patients are hypothermic and can become acidotic during intermediate periods of surgery. The experience of adult cardiac surgery with CPB on patients with SCD is limited. The purpose of our manuscript is to review the first case of adult cardiac surgery in a patient with SCD at our institution and also to review the literature on the management of patients with SCD who undergo adult cardiac surgery. J Hematol. 2014;3(1):10-12 doi: http://dx.doi.org/10.14740/jh102w