Aortic valve replacement and coronary revascularization in paroxysmal nocturnal hemoglobinuria

Cardiac surgery in patients with paroxysmal nocturnal hemoglobinuria (PNH), which is an acquired hemolytic anemia associated with thrombocytopenia and an abnormal susceptibility to venous thromboses, requires special perioperative measures. PNH is based on a clonal defect of hematopoietic stem cells characterized by deficiency in glycosyl-phosphatidylinositol-anchored surface proteins. The major mechanism of hemolysis consists of unregulated complement activation. In cardiac surgery, PNH-induced granulocytopenia increases the risk of postoperative infection. PNH-induced complement activation is further exaggerated by extracorporeal circulation in cardiac surgery leading to putative hemolytic crisis. Here, we report on a patient who developed PNH after severe aplastic anemia undergoing aortic valve replacement and coronary revascularization using extracorporeal circulation and discuss the special perioperative management and the relevant literature on this issue. Special emphasis should be given to optimal preoperative patient preparation including G-CSF administration and red blood pack transfusions, perioperative platelet substitution, fluid management, and antibiotic prophylaxis.