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- Title
Presentation and management of acute coronary syndromes among adult persons with haemophilia: results of an international, retrospective, 10-year survey.
- Authors
Fogarty, P. F.; Mancuso, M. E.; Kasthuri, R.; Bidlingmaier, C.; Chitlur, M.; Gomez, K.; Holme, P. A.; James, P.; Kruse‐Jarres, R.; Mahlangu, J.; Mingot‐Castellano, M. E.; Soni, A.
- Abstract
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.
- Subjects
ACUTE coronary syndrome; ANGINA pectoris; MYOCARDIAL infarction; CARDIOVASCULAR diseases risk factors; REVASCULARIZATION (Surgery); HEMOPHILIACS; CORONARY artery bypass risk factors
- Publication
Haemophilia, 2015, Vol 21, Issue 5, p589
- ISSN
1351-8216
- Publication type
Article
- DOI
10.1111/hae.12652