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Case Reports
. 2016 Aug 26;11(1):138.
doi: 10.1186/s13019-016-0534-8.

Open-heart surgery using a centrifugal pump: a case of hereditary spherocytosis

Yuichi Matsuzaki  1 , Hideyuki Tomioka  2 , Masaki Saso  2 , Takashi Azuma  2 , Satoshi Saito  2 , Shigeyuki Aomi  2 , Kenji Yamazaki  2
Affiliations
Case Reports

Open-heart surgery using a centrifugal pump: a case of hereditary spherocytosis

Yuichi Matsuzaki et al. J Cardiothorac Surg. .

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.

Case presentation: 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.

Conclusion: 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.

Keywords: Adult cardiac; Bicuspid aortic valve; Cardiopulmonary bypass; Centrifugal pump; Haptoglobin; Hereditary spherocytosis.

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Figures

Fig. 1
Fig. 1
Subaortic aneurysm showed by TTE
Fig. 2
Fig. 2
Subaortic aneurysm
Fig. 3
Fig. 3
Repair of the Subaortic aneurysm (SAA)
See this image and copyright information in PMC

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