Balancing anticoagulation and hemostasis in an infant with severe hemophilia A during cardiac transplantation: Review of the literature and development of a surgical protocol

Pediatr Blood Cancer. 2024 Jan;71(1):e30759. doi: 10.1002/pbc.30759. Epub 2023 Nov 7.

Abstract

Hemophilia A is a disorder resulting in a deficiency of clotting factor VIII that can lead to life-threatening bleeding. Evidence-based guidelines for surgical interventions like cardiac surgery are limited. Anticoagulation is necessary for cardiac bypass, thus risk of bleeding in a patient with hemophilia is increased and requires careful attention to maintain hemostasis. We report the first infant with severe hemophilia A and dilated cardiomyopathy who underwent successful cardiac transplantation, and review the literature on previous cardiac transplant cases in congenital hemophilia. To ensure safe and effective management, a multidisciplinary approach was used to develop the surgical protocol for transplant.

Keywords: hemophilia A; hemostasis; pediatric; protocol; transplant.

Publication types

  • Review

MeSH terms

  • Anticoagulants* / therapeutic use
  • Factor VIII / therapeutic use
  • Heart Transplantation*
  • Hemophilia A* / complications
  • Hemophilia A* / drug therapy
  • Hemorrhage
  • Hemostasis*
  • Humans
  • Infant

Substances

  • Anticoagulants
  • Factor VIII