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. 1982 Jul;37(7):486–491. doi: 10.1136/thx.37.7.486

Open-heart surgery in sickle-cell haemoglobinopathies: report of 15 cases

D Métras 1, A Ouezzin Coulibaly 1, K Ouattara 1, A Longechaud 1, P Millet 1, J Chauvet 1
PMCID: PMC459351  PMID: 7135286

Abstract

Fifteen cases of open-heart surgery in patients with sickle-cell haemoglobinopathies are reported; 13 had sickle-cell trait, one had SC haemoglobinopathy, and one had β-thalassaemia sickle-cell disease. All patients except one were operated on with moderate hypothermia, aortic cross-clamping, topical hypothermia, and cold cardioplegia. A bloodless priming solution was used in nine patients and five did not receive any blood throughout their hospital stay. Arterial and venous blood gas analysis and a search for sickle cells and haemolysis were carried out during and after cardiopulmonary bypass. The data were compared with the findings in a group of 29 patients without haemoglobinopathy operated on without blood transfusion. Two patients died from low cardiac output, unrelated to the haemoglobinopathy. All other patients recovered uneventfully. Sickling occurred during and after bypass in only one case, and the percentage of sickle cells was considerably lower during and after surgery than before. Haemolysis occurred only once during cardiopulmonary bypass and twice after surgery (the two deaths from low cardiac output). There was no acidosis or hypoxia. There was no difference in the loss of haemoglobin between the 13 survivors and the control group. Our data suggest that adequate oxygenation and avoidance of acidosis and dehydration during surgery are important. On the other hand, we do not believe that preoperative transfusion or exchange transfusion, a blood prime, normothermia, and the avoidance of aortic cross-clamping or topical hypothermia are essential precautions. We believe that transfusion should be used during cardiopulmonary bypass only for severely anaemic patients. The technique used in our cases adds to the safety of the procedure and improves the protection of the myocardium.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Cornen L., Allemand M. L'anesthésie du drépanocytaire. Med Trop (Mars) 1978 Mar-Apr;38(2):247–251. [PubMed] [Google Scholar]
  2. Craenen J., Kilman J., Hosier D. M., Weinberger M. Mitral valve replacement in a child with sickle cell anemia. J Thorac Cardiovasc Surg. 1972 May;63(5):797–799. [PubMed] [Google Scholar]
  3. HARRIS L. C., HAGGARD M. E., TRAVS L. B. THE COEXISTENCE OF SICKLE CELL DISEASE AND CONGENITAL HEART DISEASE: A REPORT OF THREE CASES, WITH REPAIR UNDER CARDIOPULMONARY BY-PASS IN TWO. Pediatrics. 1964 Apr;33:562–570. [PubMed] [Google Scholar]
  4. Hudson I., Davidson I. A., McGregor C. G. Mitral valve replacement using cold cardioplegia in a patient with sickle cell trait. Thorax. 1981 Feb;36(2):151–152. doi: 10.1136/thx.36.2.151. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Leachman R. D., Miller W. T., Atias I. M. Sickle cell trait complicated by sickle cell thrombi after open-heart surgery. Am Heart J. 1967 Aug;74(2):268–270. doi: 10.1016/0002-8703(67)90288-8. [DOI] [PubMed] [Google Scholar]
  6. Metras D., Ouezzin-Coulibaly A., Ouattara K., Longechaud A., Chauvet J., Girod X., Darracq R. La chirurgie à coeur ouvert dans les hémoglobinopathies. A propos de dix cas. Arch Mal Coeur Vaiss. 1980 Dec;73(12):1437–1445. [PubMed] [Google Scholar]
  7. Pearson H. A., Schiebler G. L., Krovetz J., Bartley T. D., David J. K. Sickle-cell anemia associated with tetralogy of Fallot. N Engl J Med. 1965 Nov 11;273(20):1079–1083. doi: 10.1056/NEJM196511112732005. [DOI] [PubMed] [Google Scholar]
  8. Szentpetery S., Robertson L., Lower R. R. Complete repair of tetralogy associated with sickle cell anemia and G-6-PD deficiency. J Thorac Cardiovasc Surg. 1976 Aug;72(2):276–279. [PubMed] [Google Scholar]
  9. Wenham P. W., Scott G. L., Wisheart J. D. Red cell survival after aortic valve replacement with Björk-Shiley prosthesis in presence of sickle-cell trait. Br Heart J. 1978 Jun;40(6):703–704. doi: 10.1136/hrt.40.6.703. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Yacoub M. H., Baron J., Et-Etr A., Kittle C. F. Aortic homograft replacement of the mitral valve in sickle cell trait. J Thorac Cardiovasc Surg. 1970 Apr;59(4):568–573. [PubMed] [Google Scholar]
  11. deLeval M. R., Taswell H. F., Bowie E. J., Danielson G. K. Open heart surgery in patients with inherited hemoglobinopathies, red cell dyscrasias, and coagulopathies. Arch Surg. 1974 Nov;109(5):618–622. doi: 10.1001/archsurg.1974.01360050016005. [DOI] [PubMed] [Google Scholar]

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