Revascularization strategies and in-hospital management in acute coronary syndromes complicated by hemophilia A or hemophilia B

Abstract

: Among adult patients with hemophilia A and hemophilia B the emergent management of acute coronary syndromes (ACSs) is challenging, and exposure to antithrombotic agents and/or revascularization procedures may confer an enhanced risk of bleeding. We sought to identify clinical characteristics and in-hospital outcomes among ACS patients with hemophilia A/hemophilia B, compared with matched noncoagulopathic ACS controls. Case discharges from the Nationwide Inpatient Sample, Healthcare Cost and Utilization Project, Agency for Healthcare Research and Quality (1998-2011) had International Classification of Diseases, 9th Revision codes for hemophilia A/hemophilia B and ACS. Control discharges were matched to cases by year of discharge and hospital. Discharges in both groups were assessed for cardiovascular risk factors, type of ACS, use of coronary artery bypass grafting, percutaneous coronary intervention (PCI), bare-metal stent and/or drug-eluting stent, bleeding, and death. In total, 237 cases and 148 848 matched controls were identified. Among cases, HIV/Hepatitis C positivity was more common and obesity/hyperlipidemia less common. ST-elevation myocardial infarction (STEMI) occurred less frequently among hemophilia A cases than controls. hemophilia A and hemophilia B cases were more likely to be managed medically. Cases treated with coronary stent placement were more likely to receive a bare-metal stent than controls. Among PCI, bleeding was more common among hemophilia A cases. The death rates were comparable between groups. ACS-hemophilia A/hemophilia B cases were more often treated noninvasively compared with controls, suggesting an avoidance of PCI/coronary artery bypass grafting in this population, and bleeding (among hemophilia A) was more common. These findings support further study of the management of ACS and in-hospital outcomes among individuals with hemophilia.

Challenges of antithrombotic therapy in the management of cardiovascular disease in patients with inherited bleeding disorders: A single‐centre experience

“…In 54 published cases, major peri‐procedural bleeding episodes occurred in 6% of patients while bleeding during follow‐up occurred in 20% of patients 21 . Furthermore, bleeding was higher in patients with haemophilia A compared to haemophilia B 22 . Consistent with our management of the patient with vWD, prior reports have suggested maintaining vWF activity >50 IU/dL and FVIII >80 IU/dL during the procedure and 48 h thereafter 23 .…”

Current concepts in the management of stable ischemic heart disease and acute coronary syndrome in patients with hemophilia

“…The doses that have been reported set targets of FVIII and FIX levels ranged from 30% to 100%. Periprocedural target peaks aim for about 80% and troughs aim for about 30% (16,17,22). Several cases (ranging from mild to severe disease) have been reported in which no hemostatic support was used during exposure to aspirin with or without heparin, and no excessive bleeding was reported (15,16).…”

“…Fogarty et al noted that 15 out of 20 cases did not alter the initial management of ACS in PWH and when it was modified it usually was withholding or delaying aspirin or lowering the dose of low molecular weight heparin (LMWH) (16). Hemostatic support before and following antithrombotic therapy included FVIII or FIX boluses or boluses followed by infusions with troughs ranging from ≥30% to ≥50%, and/or FVIII or FIX peaks ranging from ≥70% to ≥100% (16,17,22). A more general recommendation prior to PCI is to keep peak levels of clotting factor infused at concentrate above 80% and continued for 48 hours after the procedure (14).…”

Successful use of emicizumab in a patient with refractory acquired hemophilia A and acute coronary syndrome requiring percutaneous coronary intervention

“…Several methods should be employed in this population to reduce procedural‐related bleeding. The use of radial access over femoral access has proven to decrease procedural bleeding by 30% in the general population and has increasingly become the access of choice for PCI . The use of a BMS over DES is also recommended to limit the duration of DAPT .…”