Perioperative considerations in a sickle cell patient undergoing cardiopulmonary bypass

Area of Science:

• Pediatric Cardiology
• Hematology
• Cardiovascular Surgery

Background:

• Sickle cell anaemia poses significant risks during major surgery.
• Pulmonary valvotomy is a necessary cardiac procedure.
• Managing sickle cell disease (SCD) patients requires specialized pre-operative care.

Purpose of the Study:

• To describe the peri-operative management of an 11-year-old child with sickle cell anaemia undergoing pulmonary valvotomy.
• To highlight strategies for mitigating surgical risks in SCD patients.

Main Methods:

• Administered pre-operative hydroxyurea and blood transfusions to optimize hematocrit and reduce sickle haemoglobin (HbS).
• Performed exchange transfusion before cardiopulmonary bypass (CPB) to further lower HbS levels.
• Utilized normothermic CPB to prevent hypothermia.

Main Results:

• Successful surgical pulmonary valvotomy was performed.
• Peri-operative management strategies effectively controlled SCD-related risks.
• Normothermic CPB maintained stable physiological conditions.

Conclusions:

• Comprehensive pre-operative optimization, including transfusions and hydroxyurea, is crucial for SCD patients undergoing cardiac surgery.
• Exchange transfusion prior to CPB is a valuable tool for reducing HbS levels.
• Normothermic CPB is a safe and effective technique for this patient population.