Acute coronary syndrome in a hemophilia patient: A clinical challenge
Nakhle A, and Schairer J. Acute coronary syndrome in a hemophilia patient: A clinical challenge. J Hosp Med 2017; 12(s2)
Journal of Hospital Medicine
Case Presentation: A 68 year old male with a history of severe hemophilia (factor VIII <1%) and hypertension presented with symptoms of nausea, vomiting, severe dyspnea, diaphoresis and substernal chest pain for several days prior to admission. On initial presentation, electrocardiogram revealed ST-segment depressions in leads V5 and V6, with a troponin elevation to 24, so he was started on a nitroglycerin infusion and his symptoms of chest pain and dyspnea began to improve. Given his history of frequent bleeding in the setting of hemophilia, patient initially refused treatment with any antiplatelet agents or anticoagulation. The hematology service was consulted for recommendations for anticoagulation in the setting of factor VIII deficiency. Care was coordinated between the cardiology and hematology services, and percutaneous coronary intervention (PCI) and stenting was planned for treatment of his non-ST-segment elevation myocardial infarction (NSTEMI). The patient agreed to the treatment plan and was started on both heparin and factor VIII replacement with a goal of 80% correction prior to PCI. Left heart catheterization revealed severe multi-vessel coronary artery disease. He was deemed a poor surgical candidate by cardiac surgery, so underwent high risk PCI with two drug eluting stents to the ostial left anterior descending artery. Once heparin was discontinued, he remained on dual antiplatelet therapy with continued factor VIII replacement at a goal of 60% correction. He did not have any bleeding complications during his hospital course, and was instructed to follow up for regular factor VIII level checks with hematology after discharge. Discussion: The management of cardiovascular disease in patients with hemophilia is often difficult for clinicians, and there are few studies addressing specific treatment guidelines for these patients. As life expectancy for patients with hemophilia has increased, the incidence of acute coronary syndromes (ACS) in this population has also grown. Despite the fact that studies have shown successful revascularization with PCI in hemophilia patients, one retrospective study showed that in 25% of cases, initial management of ACS was altered due to the fact that the patients had hemophilia, often with delays in treatment. This demonstrates the challenge that clinicians perceive when viewing the balance between bleeding and ischemic risks in patients with this condition. Even so, data suggests that hemophilia patients with ACS should receive the same treatments and interventions as the general population and success rates are similar. Treatment with antiplatelet agents, heparin, and PCI with stenting should be used, however clotting factor replacement should often be given to prevent any associated bleeding risk in patients with severe disease. European guidelines suggest bare-metal stenting may be favored over drug-eluting stents given the shorter duration for antiplatelet agents. Conclusions:
Treatment of ACS in patients with bleeding disorders, particularly hemophilia, is a challenge, however using a multidisciplinary approach involving cardiologists, hematologists, and internists, can provide good cardiac outcomes with minimal adverse events, with rates comparable to the general population.