Cardiovascular disease is an emerging problem in the population with haemophilia. These represent a greater risk for patients with haemophilia than the general population because drugs used to treat cardiovascular disease can increase the risk of bleeding, and patients with haemophilia are also at greater risk for surgery.
Hypertension, a risk factor for cardiovascular disease, may be associated with a higher risk of intracranial haemorrhage in persons with haemophilia. In addition, obesity, another risk for cardiovascular disease, is associated with increased joint disease in haemophilia. Therefore, preventing cardiovascular disease and associated risk factors in this patient population is particularly important.
Atherosclerosis, which is the biologic basis for cardiovascular disease, begins in childhood and is associated with established risk factors including obesity, hypertension, abnormal lipid profile, and family history. Overweight and obesity in childhood are prevalent, and there is a high correlation between their presence as a child and as an adult. High blood pressure is also increasing in the general paediatric population. The prevalence of prehypertension and hypertension in children 3 to 18 years old are 3.4% and 3.6% respectively. The prevalence is substantially higher in obese children: >30% in obese boys and 23-30% in obese girls. Similarly, abnormal lipid levels are associated with obesity in children. Therefore, strategies to mitigate risk should be initiated in childhood.
The first step for prevention in the haemophilia population is to determine the prevalence of cardiovascular risk factors. Some of these including overweight, obesity, and hypertension are more prevalent in adults with haemophilia than in the general population.
A recent study identified a significant proportion of children with haemophilia with overweight and obesity including 37% with BMI ≥85% and 4.7% with severe obesity. Additionally, 37% had abnormal weight-to-height ratio (WHtR) consistent with higher cardiometabolic risk, 19% had borderline or high lipids, and 28% met criteria for (pre)hypertension.
Although 93% of subjects participated in physical activity in the past 7 days, most did not meet the recommended minimum of 60 minutes per day. About 16% of participants reported a family history of cardiovascular disease (myocardial infarction or stroke) in a male relative <55 years and/or a female relative <65 years. In these cases, the genetic counsellor can be a valuable support in providing additional information about cardiovascular risk if there is a family history.
The analyses in this study demonstrate that cardiovascular risk factors can be identified within the context of comprehensive haemophilia care. Health care providers should be aware of the definitions of overweight and obesity and hypertension as well as the guidelines for managing these conditions.
- Limjoco J, Thornburg CD. Risk factors for cardiovascular disease in children and young adults with haemophilia. Haemophilia. 2018;00:1–8. https://doi.org/10.1111/hae.13585