Perioperative Endocarditis Management in a Patient with Homozygous Sickle Cell Disease
21 August 2018
16 November 2018
08 January 2019 (online)
Background Homozygous sickle cell disease (SCD) compounded with bacterial endocarditis makes open-heart surgery a multidisciplinary challenge.
Case description A 45-year-old African male patient with homozygous SCD presented with right heart decompensation, tricuspid regurgitation, and endocarditis of the aortic valve. Blood coulters were positive for coagulase-negative staphylococci. An emergent double valve replacement was successfully performed involving a multidisciplinary team.
Conclusion Homozygous SCD is associated with an increased risk of preoperative vaso-occlusive complications. Surgery with cardiopulmonary bypass can be performed, if hypothermia, hypoxia, acidosis, or low-flows are being avoided. Due to the lack of data, the adequate approach is still intuitive and requires standardization.
- 1 Firth PG. Anaesthesia for peculiar cells--a century of sickle cell disease. Br J Anaesth 2005; 95 (03) 287-299
- 2 Steinberg MH. Management of sickle cell disease. N Engl J Med 1999; 340 (13) 1021-1030
- 3 Hemming AE. Pro: exchange transfusion is required for sickle cell trait patients undergoing cardiopulmonary bypass. J Cardiothorac Vasc Anesth 2004; 18 (05) 663-665
- 4 Yousafzai SM, Ugurlucan M, Al Radhwan OA, Al Otaibi AL, Canver CC. Open heart surgery in patients with sickle cell hemoglobinopathy. Circulation 2010; 121 (01) 14-19
- 5 Sachithanandan A, Nanjaiah P, Wright CJ, Rooney SJ. Mitral and tricuspid valve surgery in homozygous sickle cell disease: perioperative considerations for a successful outcome. J Card Surg 2008; 23 (02) 167-168
- 6 Wanko SO, Telen MJ. Transfusion management in sickle cell disease. Hematol Oncol Clin North Am 2005; 19 (05) 803-826 , v–vi
- 7 Crawford TC, Carter MV, Patel RK. , et al. Management of sickle cell disease in patients undergoing cardiac surgery. J Card Surg 2017; 32 (02) 80-84
- 8 Fox MA, Abbott TR. Hypothermic cardiopulmonary bypass in a patient with sickle-cell trait. Anaesthesia 1984; 39 (11) 1121-1123
- 9 Head CA, Brugnara C, Martinez-Ruiz R. , et al. Low concentrations of nitric oxide increase oxygen affinity of sickle erythrocytes in vitro and in vivo. J Clin Invest 1997; 100 (05) 1193-1198