Presentation and management of acute coronary syndromes among adult persons with haemophilia: results of an international, retrospective, 10‐year survey

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.

Coronary angiography with or without percutaneous coronary intervention in patients with hemophilia—Systematic review

“…The process of search and article selection is shown in Figure . Information for the present analysis was extracted from 29 articles (case reports and small case series) .…”

“…However, at least two patients (including the patient presented in the case report) experienced severe bleeding episodes months after PCI. Another patient with mild hemophilia A experienced excessive gastrointestinal bleeding during monotherapy with ASA requiring discontinuation [9].…”

Arterial and Venous Thrombosis in Haemophilia Patients: Experiences from a Danish Haemophilia Centre

“…However, recent guidelines and expert opinions [10,11,12,13,14,17] advocate that acute coronary disease in PWH should generally be managed as in non-PWH, provided that factor replacement is given, and they consider ASA therapy (with factor levels >5%) and dual anti-platelet therapy (with factor levels >15-30%) to be generally safe. This is supported by 2 recently published case series on 24 PWH with CVD [18,19], who found that revascularization and anti-platelet treatment were generally well-tolerated. The 2 cases of PCI and 4 cases of CABG reported in our population were all successful and without bleeding complications.…”

Kirurgiske inngrep hos pasienter med alvorlig blødersykdom 1997–2014

“…Leger med liten erfaring med pasienter med blødersykdom vil med stor sannsynlighet komme i kontakt med disse pasientene, som nå i større grad rammes av aldersrelaterte sykdommer på samme måte som resten av befolkningen. Videre forskning som gir grunnlag for utvikling av egne retningslinjer for behandling av pasienter med alvorlig blødersykdom, vil vaere viktig i fremtiden (13,14). Godt samarbeid mellom hematologer og leger i andre spesialiteter vil vaere viktig for å kunne gi bløderne best mulig behandling.…”