Open Heart Surgery Using a Centrifugal Pump in a Patient Suffering from Hereditary Spherocytosis / 日本心臓血管外科学会雑誌

ABSTRACT

A very rare case of open heart surgery associated with hereditary spherocytosis (HS) is reported. A 10-year-old girl was admitted for repair of an atrial septal defect (ASD). She was found to have HS by the microscopic findings of a blood smear and characteristic osmotic fragility, but splenectomy had not been undertaken preoperatively. She underwent successful radical operation by means of a centrifugal pump, and poloxamer 188 and haptoglobin were used during cardiopulmonary bypass for prevention of hemolysis. No significant hemolysis occurred intra- or postoperatively. Cardioplumonary bypass using a centrifugal pump appeared to be effective in this patient with HS.