Perioperative Management of Pediatric Cardiac Surgery in a Patient with Hemophilia A and Congenital Heart Disease (Tricuspid Atresia IB)

Background: The combination of complex congenital heart disease (CHD) and hemophilia is extremely rare and involves significant risks, especially during pediatric cardiac surgery. Patients with hemophilia are particularly susceptible to bleeding complications and for corrective surgery. The use of cardiopulmonary bypass, which requires increased anticoagulation, further heightens the risk of bleeding, specifically at cardiac and vascular suture lines. We present a case of a child with hemophilia A and univentricular CHD (tricuspid atresia type IB) who underwent two uneventful cardiac surgeries and catheterizations.

Methods: A 20-day-old boy with mild hemophilia A (Factor VIII activity 7%) and complex CHD—tricuspid atresia type IB—presented with low oxygen saturation, poor weight gain, and breathing difficulties during feeding. Due to clinical deterioration, Norwood stage 1 surgery with aortopulmonary shunt implantation was performed on day 26 of life. At 7 months, he underwent a second surgery, which included an atrioseptectomy and a bidirectional Glenn anastomosis. Both surgeries required cardiopulmonary bypass, increasing the risk of clotting disorders and blood loss. Additionally, cardiac catheterizations with arterial and venous access were performed before and after the second surgery.

Results: We successfully managed perioperative care for the cardiac surgeries and catheterizations. The patient received increasing factor VIII substitution immediately before surgery. Intraoperative coagulation was closely monitored and adjusted with transfusions of factor VIII, erythrocytes, platelets, and fibrinogen as needed. Postoperatively, factor VIII levels were tested regularly. Substitution was continued along with initial heparin anticoagulation, which was discontinued after removal of the drainage tubes on postoperative day 3. Therapy with acetylsalicylic acid (ASA) was then initiated. Factor VIII substitution was gradually reduced from every 12–24 hours to once daily before discharge, with additional substitutions available. All procedures were completed without major bleeding or other complications.

Conclusion: To our knowledge, this is the first reported case of successful pediatric cardiac surgeries in a child with hemophilia A and complex CHD, involving both Norwood-type surgery and Glenn anastomosis. Successful management requires multidisciplinary collaboration among pediatric cardiology, hematology, anesthesiology, and cardiac surgery. Key to success is preoperative planning, intraoperative techniques to minimize blood loss, and continuous preventive adjustment of clotting factors during postoperative care.

Publication History

Article published online:
11 February 2025