How we manage cardiovascular disease in patients with hemophilia

Abstract

With the striking advances in hemophilia care that materialized particularly in the last two decades, an increasing number of persons with hemophilia (PWH) have achieved a quality of life and life expectancy very close to that of unaffected individuals. With aging, a growing number of PWH develop age-related co-morbidities, including cancer and cardiovascular disease. The latter (particularly coronary artery disease and atrial fibrillation) represent a new challenge for the hemophilia treatment centers because their management implies a delicate balance between the thrombotic risk and bleeding tendency, that is further enhanced by the concomitant use of antithrombotic agents. Because evidence from clinical trials is lacking, the management of PWH with cardiovascular diseases is mostly based on expert opinions, personal experiences and the adaptation of the evidence stemming from studies on people without hemophilia. In this article, we focus on how to manage coronary artery disease and atrial fibrillation.

Guidance for the antithrombotic management of persons with haemophilia and cardiovascular disease

“…If the decision for oral anticoagulation in PWH has been made, is there a need for monitoring anticoagulation treatment and dose adjustment? The present commentary on the article by Schutgens et al 22 is written by a long-standing insider in haemophilia care, 12,19 who chose to enlist the input of a cardiologist and geriatrician with specific expertise on the aging PWH. Impressed by the clarity of the settings of the guidance blueprint (Table 1), we are convinced that it is a badly needed handbook to be widely consulted by those confronted with the clinical situations epitomised by the comprehensive and well-focussed clinical questions.…”

“…[12][13][14] Early How to Treat articles were based on the experience of large haemophilia centres and focused on how to tailor the management of the coagulation defect to the challenge of antithrombotic therapies prescribed for acute events and secondary prevention. [12][13][14][15][16][17][18][19] Further, the French network of haemophilia centres elected to start and run a prospective registry (COCHE) of incident PWH who needed antithrombotic medications owing to CVD. 20 Moreover, since 2008 the European Haemophilia Safety Surveillance program (EUHASS) is collecting information on adverse events occurring in haemophilia treatment centres and incident episodes of thrombosis are gathered.…”

“…21 However, unmet needs remained pertaining to the antithrombotic management of PWH, because recommendations were largely informed by expert opinions in the absence of high quality data. [12][13][14][15][16][17][18][19] Furthermore, the most recent therapeutic advances, such as emicizumab and gene transfer, 1 were not taken into account.…”

“…Moreover, since 2008 the European Haemophilia Safety Surveillance program (EUHASS) is collecting information on adverse events occurring in haemophilia treatment centres and incident episodes of thrombosis are gathered 21 . However, unmet needs remained pertaining to the antithrombotic management of PWH, because recommendations were largely informed by expert opinions in the absence of high quality data 12–19 . Furthermore, the most recent therapeutic advances, such as emicizumab and gene transfer, 1 were not taken into account.…”

“…Some of us have indeed faced the challenge of managing PWH who developed CVD and provided advice on how to handle the contrasting risks of thrombosis and bleeding, the latter enhanced by antiplatelet and anticoagulant drugs 12–14 . Early How to Treat articles were based on the experience of large haemophilia centres and focused on how to tailor the management of the coagulation defect to the challenge of antithrombotic therapies prescribed for acute events and secondary prevention 12–19 . Further, the French network of haemophilia centres elected to start and run a prospective registry (COCHE) of incident PWH who needed antithrombotic medications owing to CVD 20 .…”