• Victor A Ferraris, Leonard I Boral, Alice J Cohen, Susan S Smyth, and Gilbert C White.
• From the *Division of Cardiovascular and Thoracic Surgery, University of Kentucky Chandler Medical Center, Lexington, KY; †Department of Pathology and Laboratory Medicine, University of Kentucky Chandler Medical Center, Lexington, KY; ‡Division of Hematology and Oncology, Newark Beth Israel Medical Center, Newark, NJ; §Division of Cardiovascular Medicine, University of Kentucky Gill Heart Institute, Lexington, KY; and ║Blood Research Institute, BloodCenter of Wisconsin, Milwaukee, WI.
• Cardiol Rev. 2015 Mar 1; 23 (2): 53-68.

AbstractWith 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.

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