Objective: To present a case report of a patient with Noonan syndrome who developed life-threatening gastrointestinal bleeding shortly after cardiac surgery that was successfully treated with recombinant factor VIIa.
Design: Case report.
Setting: Pediatric intensive care unit of a children's hospital.
Patient: Ten-month-old with Noonan syndrome and massive gastrointestinal bleeding resulting in severe hypovolemic shock.
Interventions: Recombinant factor VIIa was used in this patient's severe bleeding associated with Noonan syndrome after no other supportive measures were successful.
Measurements and main results: Recombinant Factor VIIa significantly decreased the patient's bleeding and allowed his hypovolemic shock to improve. Ultimately, the patient made a complete recovery.
Conclusions: Noonan syndrome has a constellation of both cardiac and noncardiac malformations including an increased risk of bleeding, and recombinant factor VIIa is an important agent in the treatment of significant bleeding.