Purpose

Advanced targeted therapy has resulted in increasing life expectancy and incidence of age-related cardiovascular diseases like atrial fibrillation in patients with haemophilia. Oral anticoagulation constitutes a significant dilemma in this patient category as the risks of stroke and bleeding are difficult to balance. We sought to demonstrate the feasibility of left atrial appendage occlusion (LAAO) in patients with haemophilia and atrial fibrillation.

Methods

All patients with haemophilia treated with LAAO at Aarhus University Hospital, Denmark, were identified from a local prospective database comprising all consecutive LAAO procedures from 2010 up to November 2020. Based on review of the medical records, a retrospective descriptive analysis was performed.

Results

Seven patients with haemophilia A and atrial fibrillation underwent LAAO after multidisciplinary conference. Peri-procedural coagulation management was guided by factor VIII activity and treated with repeated bolus administrations of recombinant factor VIII targeting an activity of 100%. The implantation was successful in all patients with only minor bleeding complications post-procedurally. Based on these experiences, a suggested regime has been formulated.

Conclusions

LAAO is feasible in haemophilia patients with concurrent atrial fibrillation. However, special care including intravenous substitution with coagulation factors must be given in the periprocedural management.

中文翻译：

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血友病合并心房颤动患者的左心耳封堵术

目的

先进的靶向治疗导致血友病患者的预期寿命和与年龄相关的心血管疾病（如心房颤动）的发生率增加。由于中风和出血的风险难以平衡，口服抗凝治疗在这一类患者中构成了一个重要的困境。我们试图证明左心耳封堵术 (LAAO) 在血友病和心房颤动患者中的可行性。

方法

所有在丹麦奥胡斯大学医院接受 LAAO 治疗的血友病患者都是从本地前瞻性数据库中确定的，该数据库包含 2010 年至 2020 年 11 月的所有连续 LAAO 程序。基于对医疗记录的审查，进行了回顾性描述性分析。

结果

七名血友病 A 和房颤患者在多学科会议后接受了 LAAO。围手术期凝血管理以因子 VIII 活性为指导，并通过重复推注重组因子 VIII 进行治疗，目标为 100% 的活性。所有患者的植入均成功，术后仅有轻微出血并发症。根据这些经验，制定了建议制度。

结论

LAAO 在血友病并发房颤患者中是可行的。然而，在围手术期管理中必须给予特殊护理，包括用凝血因子进行静脉替代。