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.

How I treat patients with inherited bleeding disorders who need anticoagulant therapy

“…Because dual antiplatelet therapy has a bleeding risk comparable to warfarin, 40 we do not typically use dual antiplatelet therapy without prophylactic factor replacement, especially in patients with more-severe variants of VWD. Although published approaches have recommended maintenance of a trough factor level at .30% in hemophilia (Table 1), 1,34,[41][42][43] we have found that patients tolerate dual antiplatelet therapy with somewhat lower troughs, and by doing so, we avoid the frequent higher peak levels of FVIII that are necessary to achieve troughs of this magnitude, thereby improving patient compliance and reducing the thrombotic risk. Further, data from The Netherlands have shown that spontaneous (although not traumatic or anticoagulant-induced) bleeding is eradicated in mild to moderate hemophilia when the factor level is .15%…”

ST‐Segment Elevation Myocardial Infarction (STEMI) and Pulmonary Embolism in a Hemophilia A Patient Receiving Emicizumab and recombinant Activated Factor VII

“…8 Coagulation factor levels must be monitored closely after the procedure and with appropriate factor replacement, maintain the levels of 80%-100% while on therapeutic doses of anticoagulation and trough levels of at least ≥30% when patients are on DAPT (aspirin + P2Y 12 inhibitors) and ≥5%-10% when on aspirin alone. 8 Coagulation factor levels must be monitored closely after the procedure and with appropriate factor replacement, maintain the levels of 80%-100% while on therapeutic doses of anticoagulation and trough levels of at least ≥30% when patients are on DAPT (aspirin + P2Y 12 inhibitors) and ≥5%-10% when on aspirin alone.…”

Coronary angiography with or without percutaneous coronary intervention in patients with hemophilia—Systematic review

“…Hemophilia A and B affect about 1 in 5,000 to 10,000 and 1 in 40,000 males at birth. Disease severity depends on the plasma concentrations of FVIII or FIX, where severe, moderate, and mild disease are characterized by plasma concentrations <1% activity of normal (<0.01 U/l; usually associated with spontaneous unprovoked bleeding episodes), 1–5% activity of normal (0.01–0.05 U/l), and 5–40% of normal (0.05–0.3 U/l) respectively . Patients with moderate or mild disease typically present with bleeding after trauma or surgery.…”