Department of Internal Medicine, Hemophilia Care Center Heidelberg, SRH Kurpfalzkrankenhaus, Heidelberg, Germany.
Haemophilia. 2013 Nov;19(6):833-40. doi: 10.1111/hae.12189. Epub 2013 May 28.
There are no evidence-based guidelines for antithrombotic management in people with haemophilia (PWH) presenting with acute coronary syndrome (ACS). The aim of the study was to review the current European Society of Cardiology guidelines, and to consider how best they should be adapted for PWH. Structured communication techniques based on a Delphi-like methodology were used to achieve expert consensus on key aspects of clinical management. The main final statements are as follows: (i) ACS and myocardial revascularization should be managed promptly by a multidisciplinary team that includes a haemophilia expert, (ii) each comprehensive care centre for adult PWH should have a formal clinical referral pathway with a cardiology centre with an emergency unit and 24 h availability of percutaneous coronary intervention (PCI), (iii) PCI should be performed as soon as possible under adequate clotting factor protection, (iv) bare metal stents are preferred to drug-eluting stents, (v) anticoagulants should only be used in PWH after replacement therapy, (vi) minimum trough levels should not fall below 5-15% in PWH on dual antiplatelet therapy, (vii) the duration of dual antiplatelet therapy after ACS and PCI should be limited to a minimum, (viii) the use of GPIIb-IIIa inhibitors is not recommended in PWH other than in exceptional circumstances, (ix) the use of fibrinolysis may be justified in PWH when primary PCI (within 90 min) is not available ideally under adequate clotting factor management. It is hoped that the results of this initiative will help to guide optimal management of ACS in PWH.
目前尚无针对伴有急性冠脉综合征(ACS)的血友病患者(PWH)的抗血栓治疗管理的循证指南。本研究旨在回顾欧洲心脏病学会的现行指南,并考虑如何最好地将其应用于 PWH。采用基于德尔菲法的结构化沟通技术,就临床管理的关键方面达成专家共识。主要最终声明如下:(i)ACS 和血运重建应通过多学科团队迅速进行,该团队包括血友病专家;(ii)每个成年 PWH 的综合护理中心都应具有正式的临床转诊途径,与具有急诊部和 24 小时经皮冠状动脉介入治疗(PCI)可用性的心脏病中心建立联系;(iii)应在充分的凝血因子保护下尽快进行 PCI;(iv)首选裸金属支架而非药物洗脱支架;(v)只有在替代治疗后,才可在 PWH 中使用抗凝剂;(vi)在 PWH 接受双联抗血小板治疗时,最低谷浓度不应低于 5-15%;(vii)ACS 和 PCI 后双联抗血小板治疗的持续时间应尽可能缩短;(viii)除特殊情况外,不建议在 PWH 中使用 GPIIb-IIIa 抑制剂;(ix)在理想情况下无法进行直接 PCI(90 分钟内)时,纤维蛋白溶解可能适用于 PWH。希望这项工作的结果将有助于指导 PWH 的 ACS 最佳管理。