# Cardiac surgery in an infant hemophilia B carrier with moderate hemophilia: a case report

**Authors:** Julia Hölz, Chiara Nobile, Johannes Holzapfel, Victoria Lieftüchter, Nicole De Winkel, Martin Olivieri

PMC · DOI: 10.3389/fcvm.2026.1690797 · Frontiers in Cardiovascular Medicine · 2026-03-18

## TL;DR

A 4-month-old girl with moderate hemophilia B and a heart defect successfully underwent complex cardiac surgery using a long-lasting clotting factor.

## Contribution

First reported case of complex cardiac surgery in an infant hemophilia B carrier using extended half-life factor IX.

## Key findings

- Extended half-life recombinant factor IX (rFIX-FP) enabled stable coagulation during surgery.
- The patient experienced no excessive bleeding and recovered well post-surgery.
- Interdisciplinary collaboration and individualized planning are crucial for such cases.

## Abstract

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 clinical setting.

A 4-month-old girl with Trisomy 21 was found to have moderate hemophilia B and an atrioventricular septal defect requiring surgery.

Genetic testing confirmed hemophilia B. Surgery was performed successfully using extended half-life recombinant factor IX (rFIX-FP) to maintain stable coagulation. The patient experienced no excessive bleeding and recovered well.

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.

## Linked entities

- **Diseases:** hemophilia B (MONDO:0010604), atrioventricular septal defect (MONDO:0020290), Trisomy 21 (MONDO:0008608)

## Full-text entities

- **Genes:** F9 (coagulation factor IX) [NCBI Gene 2158] {aka F9 p22, FIX, HEMB, P19, PTC, THPH8}
- **Diseases:** bleeding (MESH:D006470), hemophilia (MESH:D006467), atrioventricular septal defect (MESH:C562831), Trisomy 21 (MESH:D004314), hemophilia B (MESH:D002836)
- **Chemicals:** FIX (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

14 references — full list in the complete paper: https://tomesphere.com/paper/PMC13039020/full.md

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Source: https://tomesphere.com/paper/PMC13039020