Consensus review of the treatment of cardiovascular disease in people with hemophilia A and B

Abstract

With advances in care, increasing numbers of people with hemophilia (PWH) achieve near-normal life expectancies and present with typical age-related cardiovascular conditions. Evidence-based guidelines for medical or surgical management of cardiovascular conditions in individuals with hemophilia are limited. Published recommendations exist for the management of some common cardiovascular conditions (eg, ischemic heart disease, atrial fibrillation), but identifying optimal strategies for anticoagulant or antithrombotic therapy constitutes the primary challenge of managing nonoperative cardiovascular disease (CVD) in PWH. In general, as long as factor concentrates or other hemostatic therapies maintain adequate hemostasis, the recommended medical and surgical management of CVD in PWH parallels that in individuals without hemophilia. The presence of factor inhibitors complicates hemophilia management. Published outcomes of CVD treatment in PWH are similar to those in the general population. Specific knowledge about factor replacement, factor inhibitors, and disease-specific treatment distinguishes the cardiovascular care of PWH from similar care of individuals without this rare bleeding disorder. Furthermore, a multidisciplinary approach incorporating a hematologist with an onsite coagulation laboratory, ideally associated with a hemophilia treatment center, is integral to the management of CVD in PWH.

Figure 1.

Recommended algorithm for management of atrial fibrillation in people with hemophilia without inhibitors. The primary determinant of management of AF in PWH is the baseline FVIII/FIX level (ie, hemophilia severity). People with severe hemophilia are considered candidates for thromboprophylaxis provided they are receiving factor concentrate prophylaxis. The decision to initiate antithrombotic therapy is further based on the individual projected risk for stroke, in this case determined by the CHADS₂ scoring system. A score of ≥2 is considered indicative of a high risk for stroke. (See text for a description of the CHADS₂ score calculation.) CFC indicates clotting factor concentrate; CHADS₂, congestive heart failure, hypertension, age, diabetes mellitus, stroke; FVIII, factor VIII; FIX, factor IX; PWH, people with hemophilia. Republished with permission of Blood: Journal of the American Society of Hematology, from “How I treat age-related morbidities in elderly persons with hemophilia,” Mannucci, PM, et al.,114, 26 © 2009; permission conveyed through Copyright Clearance Center, Inc.

Figure 2.

Suggested algorithm for management of ischemic heart disease in people with hemophilia without inhibitors.^,, ACS indicates acute coronary syndrome; ASA, aspirin; BMS, bare metal stent; CV, cardiovascular; d/c, discontinue; DES, drug-eluting stent; GP, glycoprotein; IHD, ischemic heart disease; LMWH, low-molecular-weight heparin; PCI, percutaneous coronary intervention; PPX, prophylaxis; Rx, therapy; STEMI, S-T segment elevation myocardial infarction; UFH, unfractionated heparin.