CASE REPORT article
Front. Cardiovasc. Med.
Sec. Thrombosis and Haemostasis
Cardiac surgery in an infant hemophilia B carrier with moderate hemophilia – a case report
Julia Hölz 1
Martin Olivieri 1
Johannes Holzapfel 1
Chiara Nobile 1
Victoria Lieftuecher 1
Nicole De Winkel 2
1. LMU Munich University Hospital, Munich, Germany
2. Deutsches Herzzentrum Munchen, Munich, Germany
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Abstract
Abstract Introduction: This is the first reported case of a female infant with moderate hemophilia B undergoing complex cardiac surgery, highlighting challenges in managing bleeding disorders in this rare setting. Patient Concerns and Clinical Findings: A 4-month-old girl with trisomy 21 was found to have moderate hemophilia B and an atrioventricular septal defect requiring surgery. Diagnosis, Interventions, and Outcomes: Genetic testing confirmed hemophilia B. Surgery was performed successfully using extended half-life recombinant factor IX (rFIX-FP) to maintain stable coagulation. The patient had no excessive bleeding and recovered well. Conclusion: This case demonstrates that extended half-life FIX products enable safe and effective management of moderate hemophilia B during complex cardiac surgery in infants. Individualized perioperative planning and interdisciplinary collaboration are essential for optimal outcomes.
Summary
Keywords
cardiac surgery, Hemophilia B, Infant, Perioperative management, Recombinant factor IX, Trisomy 21.
Received
22 August 2025
Accepted
30 January 2026
Copyright
© 2026 Hölz, Olivieri, Holzapfel, Nobile, Lieftuecher and De Winkel. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
*Correspondence: Julia Hölz; Martin Olivieri
Disclaimer
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