Abstract：
BACKGROUND: As people with haemophilia (PWH) receive better treatment and live longer they are more likely to encounter cardiovascular disease (CVD) and other comorbidities. ESC guidelines for the acute management of patients presenting with acute coronary syndrome (ACS) are based on the non-haemophilia population.
OBJECTIVE: To review the guidelines and propose relevant adaptations for PWHA without inhibitors who are treated with prophylaxis and present with ACS.
METHODS: As part of the ADVANCE Group, 20 European haemophilia experts used a modified Delphi approach to develop and gain consensus on proposed adaptations of the ESC guidelines for PWHA without inhibitors.
RESULTS: Of the 32 Class I recommendations across both guidelines, adaptions were considered necessary and proposed for 15. The adaptions highlight the need to provide sufficient FVIII trough levels at the time of antithrombotic treatment in people with haemophilia A (HA) without inhibitors. Patients receiving emicizumab prophylaxis and requiring oral anticoagulation therapy or combined single antiplatelet plus oral anticoagulation therapy will require additional FVIII replacement therapy.
CONCLUSIONS: In the absence of high-quality clinical evidence, the combined expert opinion used to develop these adaptions to the current ESC guidelines may help to guide clinicians in their treatment decisions when a PWHA presents with ACS.
OBJECTIVE: To review the guidelines and propose relevant adaptations for PWHA without inhibitors who are treated with prophylaxis and present with ACS.
METHODS: As part of the ADVANCE Group, 20 European haemophilia experts used a modified Delphi approach to develop and gain consensus on proposed adaptations of the ESC guidelines for PWHA without inhibitors.
RESULTS: Of the 32 Class I recommendations across both guidelines, adaptions were considered necessary and proposed for 15. The adaptions highlight the need to provide sufficient FVIII trough levels at the time of antithrombotic treatment in people with haemophilia A (HA) without inhibitors. Patients receiving emicizumab prophylaxis and requiring oral anticoagulation therapy or combined single antiplatelet plus oral anticoagulation therapy will require additional FVIII replacement therapy.
CONCLUSIONS: In the absence of high-quality clinical evidence, the combined expert opinion used to develop these adaptions to the current ESC guidelines may help to guide clinicians in their treatment decisions when a PWHA presents with ACS.
摘要：
背景：随着血友病（PWH）患者接受更好的治疗，寿命更长，他们更有可能遇到心血管疾病（CVD）和其他合并症。急性冠状动脉综合征（ACS）患者的急性治疗ESC指南基于非血友病人群。
目的：回顾指南，并提出对无抑制剂的PWHA的相关调整，这些PWHA经预防性治疗并出现ACS。
方法：作为高级小组的一部分，20名欧洲血友病专家使用改良的Delphi方法，就不含抑制剂的PWHA的ESC指南的拟议改编制定并获得共识。
结果：在两项指南的32项I类建议中，改编被认为是必要的，并建议15。这些适应强调了在没有抑制剂的A型血友病（HA）患者中抗血栓治疗时需要提供足够的FVIII波谷水平。接受emicizumab预防并需要口服抗凝治疗或单一抗血小板联合口服抗凝治疗的患者将需要额外的FVIII替代疗法。
结论：在缺乏高质量临床证据的情况下，用于制定适应当前ESC指南的合并专家意见可能有助于指导临床医生在ACS患者出现PWHA时做出治疗决策.
目的：回顾指南，并提出对无抑制剂的PWHA的相关调整，这些PWHA经预防性治疗并出现ACS。
方法：作为高级小组的一部分，20名欧洲血友病专家使用改良的Delphi方法，就不含抑制剂的PWHA的ESC指南的拟议改编制定并获得共识。
结果：在两项指南的32项I类建议中，改编被认为是必要的，并建议15。这些适应强调了在没有抑制剂的A型血友病（HA）患者中抗血栓治疗时需要提供足够的FVIII波谷水平。接受emicizumab预防并需要口服抗凝治疗或单一抗血小板联合口服抗凝治疗的患者将需要额外的FVIII替代疗法。
结论：在缺乏高质量临床证据的情况下，用于制定适应当前ESC指南的合并专家意见可能有助于指导临床医生在ACS患者出现PWHA时做出治疗决策.
