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Presentation and management of acute coronary syndromes among adult persons with haemophilia: results of an international, retrospective, 10-year survey.
Haemophilia 2015; 21(5):589-97H

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.

Authors+Show Affiliations

Penn Comprehensive Hemophilia and Thrombosis Program, Philadelphia, PA, USA.Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.Division of Hematology and Oncology, Hemophilia and Thrombosis Center, University of North Carolina, Chapel Hill, NC, USA.Munich Hemophilia Centre, Munich, Germany.Children's Hospital of Michigan, Detroit, MI, USA.Royal Free Hospital, London, UK.Department of Haematology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.Queen's University, Kingston, ON, Canada.Tulane University School of Medicine, New Orleans, LA, USA.Faculty of Health Sciences, University of the Witwatersrand and Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg, South Africa.Regional University Hospital Carlos Haya, Malaga, Spain.Children's Hospital of Orange County, Orange, CA, USA.No affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

25689278

Citation

Fogarty, P F., et al. "Presentation and Management of Acute Coronary Syndromes Among Adult Persons With Haemophilia: Results of an International, Retrospective, 10-year Survey." Haemophilia : the Official Journal of the World Federation of Hemophilia, vol. 21, no. 5, 2015, pp. 589-97.
Fogarty PF, Mancuso ME, Kasthuri R, et al. Presentation and management of acute coronary syndromes among adult persons with haemophilia: results of an international, retrospective, 10-year survey. Haemophilia. 2015;21(5):589-97.
Fogarty, P. F., Mancuso, M. E., Kasthuri, R., Bidlingmaier, C., Chitlur, M., Gomez, K., ... Soni, A. (2015). Presentation and management of acute coronary syndromes among adult persons with haemophilia: results of an international, retrospective, 10-year survey. Haemophilia : the Official Journal of the World Federation of Hemophilia, 21(5), pp. 589-97. doi:10.1111/hae.12652.
Fogarty PF, et al. Presentation and Management of Acute Coronary Syndromes Among Adult Persons With Haemophilia: Results of an International, Retrospective, 10-year Survey. Haemophilia. 2015;21(5):589-97. PubMed PMID: 25689278.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Presentation and management of acute coronary syndromes among adult persons with haemophilia: results of an international, retrospective, 10-year survey. AU - Fogarty,P F, AU - Mancuso,M E, AU - Kasthuri,R, AU - Bidlingmaier,C, AU - Chitlur,M, AU - Gomez,K, AU - Holme,P A, AU - James,P, AU - Kruse-Jarres,R, AU - Mahlangu,J, AU - Mingot-Castellano,M E, AU - Soni,A, AU - ,, Y1 - 2015/02/17/ PY - 2015/01/07/accepted PY - 2015/2/18/entrez PY - 2015/2/18/pubmed PY - 2016/6/21/medline KW - acute coronary syndrome KW - antiplatelet therapy KW - cardiovascular disease KW - haemophilia KW - myocardial infarction KW - stent SP - 589 EP - 97 JF - Haemophilia : the official journal of the World Federation of Hemophilia JO - Haemophilia VL - 21 IS - 5 N2 - 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. SN - 1365-2516 UR - https://www.unboundmedicine.com/medline/citation/25689278/Presentation_and_management_of_acute_coronary_syndromes_among_adult_persons_with_haemophilia:_results_of_an_international_retrospective_10_year_survey_ L2 - https://doi.org/10.1111/hae.12652 DB - PRIME DP - Unbound Medicine ER -