Perioperative Management of a Case of Homozygous Hemoglobin C Disease During Cardiac Surgery with Cardiopulmonary Bypass

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Résumé

Background: Hemoglobin C disease is a rare hemoglobinopathy mainly observed in black people and considered less severe than
sickle cell disease. However, its homozygous phenotype predisposes patients to hemolysis and complications during
cardiopulmonary bypass, driven by increased blood viscosity, erythrocyte fragility under shear stress, and fluctuations in
oxygenation. Case Presentation: We describe the case of a 49-year-old male with homozygous Hemoglobin C Disease admitted
for severe aortic regurgitation at the University Hospital of Tengandogo, Burkina Faso. The patient underwent aortic valve
replacement under cardiopulmonary bypass. Due to the risk of hemolysis and hemoglobin crystallization, a tailored strategy was
implemented: partial exchange transfusion with packed red blood cells and fresh frozen plasma, strict control of temperature,
oxygenation and acid-base balance, avoidance of vacuum-assisted venous drainage. The intraoperative course was uneventful,
with stable hemodynamics and no evidence of hemolysis. Postoperatively, the patient was extubated within six hours and required
no additional transfusion. He was discharged from the intensive care unit after 48 hours. Conclusion: This case highlights the
importance of individualized perioperative management in patients with Hemoglobin C Disease undergoing cardiac surgery.
Understanding the pathophysiological implications of this hemoglobinopathy is essential to mitigate risks associated with
cardiopulmonary bypass.

Mots-clés

Hemoglobin C Disease, Cardiopulmonary Bypass, Aortic Valve Replacement, Perioperative Management, Hemoglobinopathy, Burkina Faso