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Pulmonary valvulotomy in Factor XI deficiency
The Thoracic and Cardiovascular Surgeon
|June 1, 1983
Insights
This case study presents the first report of Factor XI deficiency in an Indian child undergoing heart surgery. Successful management involved plasma infusions to maintain Factor XI levels, preventing excessive bleeding.
Area of Science:
- Pediatric Hematology
- Congenital Bleeding Disorders
Background:
- Factor XI deficiency is a rare inherited bleeding disorder.
- Limited data exists on managing this condition in pediatric patients from India.
Observation:
- A pediatric patient from India with Factor XI deficiency required open-heart surgery.
- The patient underwent pulmonary valvulotomy.
Findings:
- Plasma infusion was used to maintain Factor XI levels at approximately 40% of normal.
- This management strategy prevented excessive or delayed hemorrhage during and after surgery.
Implications:
- This case provides a successful management approach for Factor XI deficiency in children undergoing major surgery.
- Highlights the importance of perioperative Factor XI level monitoring and replacement therapy.
Abstract:
This is the first reported case in the English literature of Factor XI deficiency in a child from India. He underwent open-heart surgery and pulmonary valvulotomy without excessive or delayed hemorrhage using plasma infusion to sustain Factor XI levels at about 40% of normal before, and for 10 days after surgery. The management of this child is presented as an approach to patients with Factor XI deficiency who require major surgery.