title: Cardiovascular disease in haemophilia patients: a contemporary issue.
abstract: Improvements in treatment options and healthcare provision mean that haemophilia patients now have a life expectancy approaching that of the normal male population. An increased life expectancy, however, also brings an increased risk of developing age-related disorders, the foremost of which is cardiovascular disease. The epitome of age-related morbidity, cardiovascular disease is also a leading cause of mortality in elderly individuals, and presents a particular challenge when it occurs in persons with haemophilia. While the exact incidence of cardiovascular disease in haemophilia is unknown, incidence rates from conditions such as ischaemic heart disease (IHD) have steadily risen over the last 20-30 years, suggesting that cardiac problems are increasingly relevant for these patients. Management of cardiovascular disease in haemophilia warrants close cooperation between cardiologists and haematologists, and evidence-based guidelines are not available. In the absence of such guidelines, antithrombotic treatment is currently based on local clinical experience and adaptation of the general guidelines used in the non-haemophilic population. In this article, we outline the local guidelines used by our two centres in the antithrombotic treatment of IHD, coronary bypass and valve surgery, and atrial fibrillation in patients with haemophilia. Strategies for the management of haemostasis and thrombosis during cardiovascular surgery in haemophilia patients are also briefly reviewed. Finally, we present the cases of three elderly haemophilia patients with cardiovascular and other age-related health problems in whom such treatment strategies were applied.
authors.affiliation: Department of Medicine and Medical Specialities, IRCCS Maggiore Hospital, Milan, Italy. pmmannucci@libero.it
authors.forename: P M
authors.initials: PM
authors.lastname: Mannucci
authors.forename: E P
authors.initials: EP
authors.lastname: Mauser-Bunschoten
mesh_terms: D000368:Aged; D001281:Atrial Fibrillation / Q000188:drug therapy; D001779:Blood Coagulation Factors / Q000627:therapeutic use*; D002318:Cardiovascular Diseases / Q000150:complications* / Q000188:drug therapy* / Q000601:surgery*; D005343:Fibrinolytic Agents / Q000627:therapeutic use*; D006349:Heart Valve Diseases / Q000601:surgery; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy*; D006487:Hemostasis; D006801:Humans; D008017:Life Expectancy; D008297:Male; D008875:Middle Aged; D017410:Practice Guidelines as Topic; D013927:Thrombosis / Q000188:drug therapy; D016896:Treatment Outcome
chemical_list: D001779:Blood Coagulation Factors; D005343:Fibrinolytic Agents