Novel Peri-Operative Strategy for Sickle Cell Disease with Tetralogy of Fallot

Area of Science:

• Cardiology
• Hematology
• Anesthesiology

Background:

• Peri-operative management of patients with cyanotic congenital heart disease (CCHD) and sickle cell disease (SCD) presents unique challenges.
• Sickle cell disease increases the risk of vaso-occlusive crises, particularly during the physiological stress of surgery.

Observation:

• A case of Tetralogy of Fallot with homozygous SCD, a history of multiple blood transfusions, and recurrent sickle cell crises is presented.
• The patient underwent successful intracardiac repair.

Findings:

• A multi-modal strategy involving pre-operative blood transfusion, intraoperative exchange transfusion, and normothermic cardiopulmonary bypass (CPB) significantly reduced Hemoglobin S levels from 75% pre-operatively to 21.8% post-operatively.
• This approach effectively mitigated the risk of sickling crises during the peri-operative period.

Implications:

• Pre-operative optimization and meticulous intraoperative management are crucial for successful surgical outcomes in patients with co-existing CCHD and SCD.
• This case demonstrates a viable strategy for managing complex hematological and cardiac conditions simultaneously.