Outcomes of management of acute coronary syndrome in patients with congenital bleeding disorders: A single center experience and review of the literature

IntroductionEvidence-based guidelines for the management of acute coronary syndrome (ACS) in patients with congenital bleeding disorders are lacking and largely confined to case reports.MethodsOutcomes of acute and long-term management of ACS in patients with mild hemophilia and von Willebrand disease managed at our institution from 2000-2011 were reviewed.ResultsBetween 2000-2011, 8 patients (median age 74 years) experienced 10 ACS events. In the emergency room, 3 of 4 patients received aspirin 325 mg and intravenous unfractionated heparin therapy, with no acute bleeding complications. The 8 patients underwent 10 coronary angiography procedures. Prophylactic factor concentrates were not administered for 6/10 (60%) of the procedures; bleeding complications (groin hematoma) occurred in 1/6 (17%). Two patients receiving bare metal stents and glycoprotein IIb/IIIa inhibitor infusion with factor concentrates experienced no acute hemorrhagic complications. On discharge, aspirin was initiated/continued in 6/10 events; the 2 patients receiving dual anti-platelet therapy for 1 month did not receive factor concentrates and experienced no bleeding complications. During a median follow-up of 8.5 years (1 – 11.5 years), 2 of 5 patients developed minor bleeding complications while on aspirin.ConclusionOur data demonstrate that in patients with mild congenital bleeding disorders, despite not receiving factor concentrates prior to coronary angiography, the acute management of ACS did not result in severe hemorrhagic complications. Short-term dual anti-platelet therapy seemed to be well tolerated. In patients receiving long-term aspirin for secondary prevention for ACS, bleeding complications were mild, however such patients warrant close follow-up.