Perioperative management of sickle cell disease in paediatric cardiac surgery

K Bhatt1, S Cherian, R Agarwal

  • 1Department of Cardiac Anaesthesiology, Frontier Lifeline, Chennai, Mogappair, India.

Insights

This study details managing sickle cell disease in a child needing heart surgery. Partial exchange transfusion effectively lowered hemoglobin S levels during cardiopulmonary bypass, ensuring patient safety.

Area of Science:

  • Hematology
  • Pediatric Cardiology
  • Cardiovascular Surgery

Background:

  • Sickle cell disease (SCD) poses risks during cardiopulmonary bypass (CPB) due to potential red blood cell sickling.
  • Partial exchange transfusion is a strategy to reduce circulating hemoglobin S (HbS) levels.
  • Surgical intervention for congenital heart defects in SCD patients requires careful management to mitigate risks.

Observation:

  • A pediatric patient with SCD required surgical closure of a ventricular septal defect.
  • A staged approach involving preoperative and intraoperative partial exchange transfusion was implemented.
  • The patient's HbS level was reduced from 76% to 37% during the CPB procedure.

Findings:

  • Combined preoperative and intraoperative exchange transfusions effectively lowered HbS levels.
  • This phased transfusion strategy potentially minimizes adverse hemodynamic effects compared to a single-stage exchange.
  • The CPB was managed at normothermia, avoiding cold cardioplegia and utilizing fibrillatory arrest.

Implications:

  • This management strategy offers a viable approach for SCD patients undergoing CPB.
  • Phased exchange transfusion may improve safety and outcomes in pediatric cardiac surgery for SCD.
  • Further research can explore optimizing transfusion protocols in this high-risk population.

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