Revascularization Strategies and In-Hospital Outcomes In Acute Coronary Syndromes (ACS) Complicated By Von Willebrand Disease (VWD)

Information is limited regarding in-hospital management of ACS, which typically requires invasive procedures and/or exposure to antithrombotic agents, when complicated by the most common inherited bleeding disorder, VWD. We sought to identify clinical characteristics and in-hospital outcomes among ACS patients with VWD, compared to noncoagulopathic ACS controls.

The study included discharges from the Nationwide Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality (2004-2010). Case discharges had ICD-9 codes for both VWD and ACS; discharges with ICD-9 codes indicating an alternative bleeding disorder were excluded. Control discharges had ICD-9 codes for ACS and were matched to case discharges using state, year of discharge and hospital type (urban teaching, urban non-teaching, and rural). IDC-9 codes were used to identify presence of cardiovascular risk factors; use of coronary artery bypass grafting (CABG), percutaneous coronary intervention (PCI), bare-metal stent (BMS) and/or drug-eluting stent (DES); bleeding or bruising; and transfusions of packed red blood cells (pRBCs). Unadjusted and adjusted odds ratios were obtained from conditional univariate and multivariate logistic regression analyses.

264 cases and 706,124 matched controls were identified. The median age was 61.5y (28-95y) and 67.0y (18-112y), respectively. 59.5% of cases were female, compared to 39.4% of controls (p<0.001). Obesity, diabetes, and hyperlipidemia occurred at a similar rates in the two groups, whereas HCV and HIV infection were more common among cases than in controls (1.1% versus 0.3% and 0.8% versus 0.1%, respectively; p=0.014 and p=0.003). Compared to controls, a significantly higher proportion of cases did not undergo PCI or CABG and were managed medically (56.9% and 48.4%, respectively; p=0.006)(Table). Cases were less likely to undergo PCI (31.4%) than controls (40.5%) (p=0.03), whereas a similar proportion of both groups underwent CABG (11.7% and 11.1%, respectively)(Table). At least one intracoronary stent was deployed in almost all cases and controls undergoing PCI (Table), but use of only BMS, which does not require as long a period of post-insertion dual antiplatelet therapy as DES, was twice as common among cases than controls (46.2% v. 22.0%, respectively; p<0.001). Reported bleeding among PCI or CABG was higher in cases compared to controls (7.2% vs 3.3% and 12.9% vs 4.0%, respectively; p=0.0472 and p=0.045). However, the use of pRBC transfusion associated with PCI or CABG was comparable (Table). The death rate was similar in both groups (4.2% and 3.6%) (Table). There were no in-hospital deaths involving cases undergoing PCI or CABG.

Among discharges associated with ACS complicated by VWD, the majority are female. Median age at hospital presentation of ACS is lower and rates of cardiovascular risk factors appear to be comparable to the noncoagulopathic ACS population. PCI and DES are less frequently offered to patients with ACS complicated by VWD, possibly in consideration of the underlying bleeding disorder and the desire to avoid exposure to extended-duration dual antiplatelet therapy. Similar rates of pRBC transfusion in the setting of PCI or CABG, however, suggest no increase in clinically important in-hospital bleeding due to VWD. These data suggest that CABG or PCI as definitive management of ACS are safe in selected patients with VWD. Further studies are needed to determine long-term outcomes and whether PCI and DES should be made more widely available to ACS patients with VWD.

Fogarty:Bayer Healthcare: Honoraria; Baxter Healthcare: Consultancy, Research Funding; Biogen IDEC: Consultancy, Honoraria, Research Funding; CSL Behring: Research Funding; Grifols: Consultancy; NovoNordisk: Consultancy, Research Funding; Pfizer: Consultancy, Research Funding.