What is a bicuspid aortic valve?

A bicuspid aortic valve (BAV) is a heart defect that is present at birth (congenital), which affects the aortic valve.

The aortic valve is a valve in the heart where the left ventricle (lower chamber of the heart) meets the aorta (the body’s largest artery, which carries blood out of the heart to the rest of the body). Its purpose is to open when the heart contracts to allow blood to flow out, then closing to prevent backflow. It usually consists of three cusps or flaps. However, a small proportion of the population are born with an aortic valve that only has two cusps. This is called a bicuspid aortic valve.

What is the prognosis?

Most babies with a bicuspid aortic valve do not display any symptoms and the condition may go undiagnosed until adulthood or not at all. However, if the BAV causes complications or if other heart abnormalities are present, the child may tire easily, lose appetite, or even suffer heart failure.

Symptoms of a bicuspid aortic valve

It can be hard to know that a child has a bicuspid aortic valve, as it often causes no symptoms. Many cases go undiagnosed. During routine tests and exams, a healthcare provider may notice signs such as a heart murmur, enlargement of the heart, or weakness in the child’s wrists and ankles, all of which can indicate the presence of BAV.

BAV can mean that the aortic valve isn’t completely effective. This can lead to aortic regurgitation (allowing blood to flow back into the heart) or aortic stenosis (the valve becomes stiff and is more difficult to open, forcing the heart to pump harder). It can also cause the opening of the valve to narrow.

Signs of complications due to a BAV include:

• Palpitations
• Fatigue
• Chest pain
• Shortness of breath
• Fainting
• Pale skin

Medical tests to diagnose a bicuspid aortic valve

The most common tests are MRIs and echocardiograms, which allow the doctor to view the internal structures of the heart, including the aortic valve. Further tests may be ordered if the doctor suspects other heart defects or complications.

What are the causes of a bicuspid aortic valve?

Bicuspid aortic valve is the most common congenital heart defect. It often runs in families, suggesting that genetics play a part. It is also more common in males than in females. BAV usually begins in the first few weeks of pregnancy, when the baby’s heart is developing.

Treatments for a bicuspid aortic valve

Bicuspid aortic valves may not require treatment if it is causing no symptoms or if the symptoms are only mild. In these cases, the condition is monitored to make sure it isn’t getting worse.

In more serious cases, surgery may be needed to replace the aortic valve. If the valve is narrowed, it may be opened up via cardiac catheterisation (inserting a tube through the blood vessels to the heart and inflating a small balloon to push the valve open). If the aorta has also been damaged by the abnormal blood pressure caused by the BAV, it may also need treatment.

Which type of specialist treats bicuspid aortic valves?

Cardiologists diagnose and treat heart conditions such as bicuspid aortic valves. In the case of babies and children, they will be referred to a paediatric cardiologist.