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首页> 外文期刊>Haemophilia: the official journal of the World Federation of Hemophilia >Presentation and management of acute coronary syndromes among adult persons with haemophilia: results of an international, retrospective, 10-year survey
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Presentation and management of acute coronary syndromes among adult persons with haemophilia: results of an international, retrospective, 10-year survey

机译：成人血友病急性冠脉综合征的表现和管理：一项国际回顾性十年调查的结果

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摘要

Sparse data are available on presentation and management of acute coronary syndromes (ACS), including unstable angina and non-ST-and ST-elevation myocardial infarction, among persons with haemophilia (PWH). The aim of this study was to determine demographics, bleeding disorder characteristics, cardiovascular risk factors (CRFs), interventions, haemostatic protocol, revascularization outcomes and complications among PWH with ACS. Members of an international consortium comprising >2000 adult PWH retrospectively completed case report forms for episodes of ACS in a >10-year follow-up period (2003-2013). Twenty ACS episodes occurred among 19 patients [rate, 0.8% (95% CI 0.4, 1.2)]. Seven patients (37%) were aged <50 years; 10 (53%) had >= 3 CRFs. In 5/20 episodes (25%), the initial ACS management protocol was altered because of the bleeding disorder. None of the eight patients with severe haemophilia underwent coronary artery bypass grafting (CABG), compared with 54.5% of patients with non-severe disease (P = 0.02). Revascularization with percutaneous coronary intervention (PCI) or CABG was rated successful in 13/13 cases, with no excessive bleeding during initial management. During chronic exposure to antiplatelet agents, secondary haemophilia prophylaxis was more prevalent in patients with severe haemophilia compared with non-severe haemophilia (85.7% vs. 30%, P = 0.05). No ACS-related deaths occurred during initial management, but one patient with severe haemophilia A died of undetermined cause 36 months after the ACS event while on aspirin therapy. ACS occurs even among relatively younger PWH, typically in association with multiple CRFs. Revascularization with PCI/CABG is feasible, and antiplatelet agents plus secondary prophylaxis appears to be well tolerated in selected PWH with ACS.
机译：对于患有血友病（PWH）的人，急性冠状动脉综合征（ACS）的表现和管理（包括不稳定型心绞痛和非ST和ST抬高的心肌梗塞）的数据很少。这项研究的目的是确定人口统计学，出血性疾病特征，心血管危险因素（CRF），干预措施，止血方案，血运重建结果以及ACS患者中PWH的并发症。一个国际协会的成员，在超过10年的随访期内（2003-2013年），回顾性地填写了2000例成年PWH的ACS病例报告表。 19例患者中发生了20例ACS发作[发生率为0.8％（95％CI 0.4，1.2）]。 7名患者（37％）年龄小于50岁; 10个（53％）的CRF> = 3。在5/20发作（25％）中，最初的ACS管理方案因出血性疾病而改变。八名严重血友病患者中没有一名接受冠状动脉搭桥术（CABG），相比之下，非严重疾病患者中有54.5％（P = 0.02）。经皮冠状动脉介入治疗（PCI）或CABG的血运重建在13/13例中被评定为成功，在初始治疗期间无过多出血。在长期暴露于抗血小板药的过程中，与非重度血友病相比，重度血友病患者的继发性血友病预防更为普遍（85.7％vs. 30％，P = 0.05）。在初始治疗期间，没有ACS相关的死亡发生，但是在接受阿司匹林治疗的ACS事件发生36个月后，一名重度A型血友病患者死于原因不明的死亡。 ACS甚至在相对年轻的PWH中发生，通常与多个CRF相关。 PCI / CABG的血运重建术是可行的，抗血小板药物和继发性预防措施在选择ACS的PWH中表现出良好的耐受性。

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