Abnormalities of the heart and vessels
Eighty per cent of people with WBS have cardiovascular problems. Some WBS patients have a particular narrowing of the aorta just above the origin of the aorta from the heart (supravalvular aortic stenosis (wikipedia)). The heart itself shows no structural defects and is normally constructed. However, a thickening of the heart wall may occur if narrowing on the aorta causes the pressure at which the heart has to pump to become too high. The majority of supravalvular aortic stenoses are benign. Surgical intervention is only necessary in a small minority of children.
Vascular abnormalities can also occur at other sites. For example, we see constrictions at the level of the pulmonary, hepatic and renal vessels.
Other possible abnormalities of the heart and vessels are:
- People with WBS have an increased risk of heart valve problems
- About half of people with WBS have elevated blood pressure. The risk of hypertension increases with age. Among adolescents and adults with WBS, about half suffer from hypertension
- From puberty onwards, there is an increased risk of developing a stroke, as narrowing can occur in several arteries that supply blood to the brain
- We hear from parents that children with WBS find it more difficult to maintain their body temperature in cold environments, such as when swimming.
Children with WBS usually do not experience physical limitations due to the existing heart defect.
