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Repair of ventricular septal defect and aortic regurgitation associated with severe hemophilia B
The Annals of Thoracic Surgery
|July 1, 1986
Insights
This study details managing a child with severe factor IX deficiency during open-heart surgery. Prothrombin complex concentrate and fresh frozen plasma effectively normalized factor IX levels, ensuring safe surgical outcomes.
Area of Science:
- Pediatric Cardiology
- Hematology
- Surgical Management
Background:
- Severe factor IX deficiency (hemophilia B) poses significant bleeding risks, especially during major surgery.
- Open-heart surgery with extracorporeal circulation demands meticulous hemostatic control.
- Effective management strategies for factor IX deficiency in pediatric surgical patients are crucial.
Observation:
- A pediatric patient with severe factor IX deficiency required open-heart surgery.
- Extracorporeal circulation was employed during the surgical procedure.
- Prothrombin complex concentrate (PCC) and fresh frozen plasma (FFP) were administered.
Findings:
- Factor IX levels were normalized pre-operatively and post-cardiopulmonary bypass using PCC and FFP.
- Postoperative monitoring for 20 days revealed sustained factor IX activity above 50%.
- Partial thromboplastin time (PTT) and factor IX serum levels remained within acceptable ranges.
Implications:
- PCC and FFP are effective in managing severe factor IX deficiency during complex pediatric cardiac surgery.
- This approach ensures adequate hemostasis and reduces perioperative bleeding complications.
- Successful surgical outcomes are achievable in pediatric patients with hemophilia B undergoing major cardiac procedures.
Abstract:
A child with severe factor IX deficiency who underwent an open-heart operation using extracorporeal circulation is described. The factor IX level was normalized immediately before operation and at the end of cardiopulmonary bypass by infusing prothrombin complex concentrate and fresh frozen plasma. Partial thromboplastin time and factor IX serum levels were monitored for 20 days postoperatively and showed factor IX activity higher than 50%.