Cardiac surgery in a patient with paroxysmal nocturnal hemoglobinuria

Abstract

A 44-year-old woman, who had been diagnosed with paroxysmal nocturnal hemoglobinuria, presented with chest pain. Angiography revealed critical distal left main stenosis and proximal stenoses of the left anterior descending and circumflex arteries. Prophylactic antibiotics, infusion of blood products and mannitol intraoperatively, and antithrombotic therapy postoperatively ensured a good outcome of coronary artery bypass graft surgery under cardiopulmonary bypass.

Cardiopulmonary bypass in a patient with classic paroxysmal nocturnal hemoglobinuria during treatment with eculizumab

“…Lastly, patients with PNH frequently have associated bone marrow failure, increasing the risk of infections and bleeding. Strategies minimizing the risk of hemolysis include leukocyte‐depleted red blood cell (RBC) transfusions to reduce the percentage of GPI‐deficient erythrocytes, and avoiding complement‐containing blood products (3–8). Nevertheless, surgery in untreated patients with PNH was generally discouraged because of associated risks and low life expectancy.…”