title: Open-heart surgery using a centrifugal pump: a case of hereditary spherocytosis.
abstract: BACKGROUND
  Hereditary spherocytosis is a genetic, frequently familial hemolytic blood disease characterized by varying degrees of hemolytic anemia, splenomegaly, and jaundice. There are few reports on adult open-heart surgery for patients with hereditary spherocytosis.
  
  
  METHODS
  We report a rare case of an adult open-heart surgery associated with hereditary spherocytosis. A 63-year-old man was admitted for congestive heart failure due to bicuspid aortic valve, aortic valve regurgitation, and sinus of subaortic aneurysm. The family history, the microscopic findings of the blood smear, and the characteristic osmotic fragility confirmed the diagnosis of hereditary spherocytosis. Furthermore, splenectomy had not been undertaken preoperatively. The patient underwent a successful operation by means of a centrifugal pump. Haptoglobin was used during the cardiopulmonary bypass, and a biological valve was selected to prevent hemolysis. No significant hemolysis occurred intraoperatively or postoperatively.
  
  
  CONCLUSIONS
  There are no previous reports of patients with hereditary spherocytosis, and bicuspid aortic valve. We have successfully performed an adult open-heart surgery using a centrifugal pump in an adult patient suffering from hereditary spherocytosis and bicuspid aortic valve.
authors: The Heart Institute Japan, Department of Cardiovascular Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjyuku-ku, Tokyo, 162-8666, Japan. bokumatsuzaki@hotmail.co.jp.
authors: Yuichi
authors: http://orcid.org/0000-0002-5931-2020
authors: Y
authors: Matsuzaki
authors: The Heart Institute Japan, Department of Cardiovascular Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjyuku-ku, Tokyo, 162-8666, Japan.
authors: Hideyuki
authors: H
authors: Tomioka
authors: The Heart Institute Japan, Department of Cardiovascular Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjyuku-ku, Tokyo, 162-8666, Japan.
authors: Masaki
authors: M
authors: Saso
authors: The Heart Institute Japan, Department of Cardiovascular Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjyuku-ku, Tokyo, 162-8666, Japan.
authors: Takashi
authors: T
authors: Azuma
authors: The Heart Institute Japan, Department of Cardiovascular Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjyuku-ku, Tokyo, 162-8666, Japan.
authors: Satoshi
authors: S
authors: Saito
authors: The Heart Institute Japan, Department of Cardiovascular Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjyuku-ku, Tokyo, 162-8666, Japan.
authors: Shigeyuki
authors: S
authors: Aomi
authors: The Heart Institute Japan, Department of Cardiovascular Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjyuku-ku, Tokyo, 162-8666, Japan.
authors: Kenji
authors: K
authors: Yamazaki
mesh_terms: D001014:Aortic Aneurysm / Q000150:complications / Q000601:surgery; D001021:Aortic Valve / Q000002:abnormalities* / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000150:complications / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D006348:Cardiac Surgical Procedures / Q000379:methods*; D002315:Cardiopulmonary Bypass / Q000295:instrumentation*; D006333:Heart Failure / Q000209:etiology / Q000601:surgery*; D006349:Heart Valve Diseases / Q000150:complications / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012850:Sinus of Valsalva / Q000601:surgery; D013103:Spherocytosis, Hereditary / Q000150:complications*
keywords: Adult cardiac; Bicuspid aortic valve; Cardiopulmonary bypass; Centrifugal pump; Haptoglobin; Hereditary spherocytosis