Management of Acute Coronary Syndrome In Patients with An Inherited Bleeding Disorder: A Single Center Experience.

Evidence based guidelines for management of acute coronary syndrome (ACS) in patients (pt) with congenital hemophilia A & B (HA/HB) and von Willebrand disease (VWD) are lacking and confined to case reports and expert opinion (Schutgens et al, 2009).

To review the management of ACS in patients with HA/HB and VWD followed at the Mayo Comprehensive Hemophilia Center.

All pt with HA/HB and VWD who presented with symptoms of ACS to our institution between 2000 and 2010 were retrospectively identified using the hemophilia center database. Medical notes were reviewed, focusing on types of intervention and bleeding complications.

Ten pt, HA = 3 and VWD=7, experienced 13 ACS or suspected ACS events. Median age at time of event was 74 years (45 – 80 years), with M:F = 7:3. All 13 events presented with chest pain. 6/13 events presented to the emergency department (ED), 4/13 events presented to a Mayo outpatient clinic, 2/13 events occurred in hospitalized pt and 1 event was a direct referral from a non-Mayo outpatient clinic to our Cardiology in-patient service.

ED pt received 325 mg aspirin (HD-ASA) in 5/6 events and intravenous unfractionated heparin (UFH) in 4/6 events, per ACS management guidelines. One ED pt did not receive either ASA or UFH due to atrio-venous malformation (AVM)-related gastrointestinal (GI) bleed (Hg 6 g/dL). Of the inpatients, one received HD-ASA while the other was already on daily low-dose aspirin (LD-ASA); neither received UFH. The pt admitted directly to the Cardiology in-patient service received HD-ASA but not UFH. Of the outpatients, one was started on LD-ASA while another was already on LD-ASA; none received UFH as they were considered low-risk for ACS but warranted further diagnostic evaluation.

Coronary angiography (CA) via a right femoral arterial approach was performed in 12/13 events; of which 5 received prophylactic factor concentrate prior to CA. One pt (no prior prophylaxis) experienced minor bleeding at the puncture site, requiring factor concentrate replacement post-CA. Another patient continued to receive prophylactic factor concentrate for 4 days post-CA. Major bleeding or bleeding requiring red blood cell (RBC) transfusion did not occur.

Following CA, in 2/12 events, pt underwent bare metal stent (BMS) placement and glycoprotein 2b/3a inhibitor infusion, with no bleeding complications. In 4/12 events, pt proceeded to coronary artery bypass grafting (CABG) and received prophylactic factor concentrate; of which one required multiple post-operative RBC transfusions due to ongoing AVM-related GI bleed. The remaining 6 events were managed medically.

The 1 pt who did not undergo CA experienced a post-total knee replacement ACS and was medically managed.

On discharge, in 5/13 events, pt were started on daily ASA (HD-ASA, n=2; LD-ASA, n=3). 2 of these received a month of clopidogrel, in addition to LD-ASA, due to BMS placement and were not placed on prophylactic factor concentrate for the duration of dual anti-platelet agent therapy; there were no bleeding complications. Daily ASA was continued in 4/13 events (HD-ASA, n=1; LD-ASA, n=3), with 1 patient receiving both LD-ASA and a month of warfarin for DVT prophylaxis post-orthopedic surgery. In the remaining 4 events, pt were dismissed without ASA, due to bleeding complications (n=2) or the perceived risk of bleeding outweighed the benefit (n=2).

During long term follow-up, 1 patient had to stop HD-ASA after 2 years due to GI bleed, while another patient stopped LD-ASA due to episodic epistaxis requiring nasal packing.

In this small series of patients, outcomes of management of ACS did not result in significant acute bleeding complications. In addition, long-term treatment with anti-platelet agents appears to be safe and should be strongly considered for all patients with coronary artery disease.

No relevant conflicts of interest to declare.