Napolitano Angela, Venturini Andrea, Ronzoni Mauro, Saggiorato Graziella, Simioni Paolo, Zanon Ezio
First Chair of Internal Medicine, Department of Medicine (DIMED), University of Padua, 35121 Padua, Italy.
Cardiac Surgery Unit, Spedali Civili Hospital, University of Brescia, 25123 Brescia, Italy.
Hematol Rep. 2025 Aug 6;17(4):41. doi: 10.3390/hematolrep17040041.
Hemophilia A presents a considerable challenge in cardiac surgery due to the elevated risk of perioperative bleeding, particularly during procedures involving cardiopulmonary bypass. Standard management typically involves standard half-life (SHL) factor VIII (FVIII) concentrates, which require frequent dosing. Extended half-life (EHL) FVIII products offer theoretical advantages, including prolonged action and reduced infusion frequency, but their use in cardiac surgery remains largely undocumented. We report the case of a 73-year-old male with mild Hemophilia A who underwent successful aortic valve replacement using a 25 mm Carpentier-Edwards Magna Ease biological prosthesis. The patient was managed perioperatively with an anti-hemorrhagic protocol based on EHL recombinant FVIII. The surgery and postoperative course were uneventful, with no bleeding complications or need for transfusion. This case illustrates the potential role of EHL FVIII in safely managing hemophilic patients undergoing major cardiac surgery. Given the lack of existing reports in the literature, further studies are warranted to evaluate the efficacy and safety of EHL FVIII in this setting and to potentially optimize perioperative care protocols for this patient population.
由于围手术期出血风险升高,尤其是在涉及体外循环的手术过程中,甲型血友病给心脏手术带来了相当大的挑战。标准治疗通常使用标准半衰期(SHL)的凝血因子VIII(FVIII)浓缩物,这需要频繁给药。延长半衰期(EHL)的FVIII产品具有理论上的优势,包括作用时间延长和输注频率降低,但其在心脏手术中的应用在很大程度上仍无文献记载。我们报告了一例73岁男性轻度甲型血友病患者的病例,该患者使用25毫米Carpentier-Edwards Magna Ease生物假体成功进行了主动脉瓣置换术。患者在围手术期采用基于EHL重组FVIII的抗出血方案进行管理。手术和术后过程顺利,无出血并发症,也无需输血。该病例说明了EHL FVIII在安全管理接受重大心脏手术的血友病患者中的潜在作用。鉴于文献中缺乏现有报告,有必要进一步研究以评估EHL FVIII在此情况下的疗效和安全性,并可能优化该患者群体的围手术期护理方案。