Bicuspid Aortic Valve In ChildrenDr Naqvi is an expert in disorders of the aorta including bicuspid aortic valve in children. She is the Paediatric Aortopathy Lead at the Royal Brompton Hospital in Chelsea, London. She is an active member of the Pan London Aortopathy expert group and organises professional meetings on diseases of the aorta.
Bicuspid Aortic Valve
What is a Bicuspid Aortic Valve?A normal aortic valve has 3 leaflets and appears Y shaped, rather like a Mercedes Benz sign. It guards the entrance to the aorta which is the large blood vessel which carries oxygenated red blood from the heart around the body. A small proportion of the population (1-2%) has a bicuspid aortic valve which has only 2 leaflets. This bicuspid valve usually works normally but it can develop problems during an affected person’s lifetime, namely narrowing (stenosis) or leakage (regurgitation) or both (mixed aortic valve disease). In addition bicuspid aortic valve is also associated with dilatation (enlargement) of the aorta and aneurysm formation through life and it can also be associated with other congenital heart defects.
SymptomsThe majority of children with bicuspid aortic valve will have no symptoms. If they develop severe narrowing of the valve (aortic stenosis) or a severe leak (aortic regurgitation) they can develop the symptoms associated with these problems. This is not common in childhood but potentially shortness of breath, failure to gain weight, chest pain and fainting could occur.
DiagnosisA clinical examination will usually reveal a heart murmur. An echocardiogram will diagnose bicuspid aortic valve. The leaflets of the aortic valve and not always the easiest to image and it is beneficial to use the highest definition echocardiography machines (ideally not a portable machine). Dr Naqvi uses the newest high-tech echocardiography machines. An ECG will also be performed and often a Chest X-ray. If there is enlargement of the aorta a CT Scan or MRI scan may be requested.
A bicuspid aortic valve with no significant narrowing or leakage requires no treatment. Severe narrowing or leakage of a bicuspid valve usually requires surgical treatment. Mild to moderate narrowing (aortic stenosis) is usually monitored relatively frequently in the outpatient clinic. Moderate aortic regurgitation (leaky valve) is often treated with medication such as an ACE inhibitor eg. Captopril or lisinopril or enalapril. Diuretic, which make the work of the heart easier, may also be given.
The outlook for children with a bicuspid valve is excellent in the vast majority of cases. Dr Naqvi has cared for many babies and children with bicuspid aortic valve and has followed them up until adulthood. She says “Having a bicuspid aortic valve does not stop children doing well in sports or from having a happy full life. I know many who are athletes and academic high achievers. The most famous person I have heard of with a bicuspid aortic valve is Arnold Schwarzenegger”.