Reducing the risk of atherosclerotic cardiovascular disease in people with haemophilia, the importance of primary prevention.

Revolutionary advances in the treatment of haemophilia has led to a significant improvement in life expectancy. Associated with this has been an increase in age-related diseases especially atherosclerotic cardiovascular disease (CVD). While people with haemophilia (PWH) develop atherosclerosis at rates similar to the general population, rates of atherothrombosis and mortality related to CVD have been much lower, due to their hypocoagulable state. Changing treatment paradigms, aimed at reducing the risk of bleeding by improving haemostasis to levels approaching normality, has meant the protection they are thought to have had may be lost. CVD risk factors are just as common in PWH as in the general population but appear to be under-treated. In particular primary prevention of CVD is vital in all individuals, but particularly in PWH as treatment of established CVD can be difficult. Active identification and management of CVD risk factors, such as obesity, physical inactivity, hypertension and hypercholesterolaemia, is required. In particular, statins have been shown to significantly reduce cardiovascular and all-cause mortality with few adverse events and no increased risk of bleeding in the general population, and their use needs urgent assessment in PWH. Further longitudinal research into preventing CVD in PWH, including accurate CVD risk assessment, is required to optimise prevention and management.