Bicuspid Aortic Valve & Congenital Aortic Valve Disease
Information for teenagers and adults with a congenital aortic valve condition
The aortic valve sits between the main pumping chamber of the heart (the left ventricle) and the aorta, the major artery that carries blood to the body.
Normally, the aortic valve has three leaflets, or cusps, which open and close with each heartbeat. Some people are born with an aortic valve that has developed differently.
The most common form is a bicuspid aortic valve (BAV), where the valve has two functional leaflets rather than three. Bicuspid aortic valve is one of the most common congenital heart conditions, affecting approximately 1–2% of the population.
Other people may be born with a valve that is unusually narrow or shaped differently, causing congenital aortic stenosis. Some people have undergone a balloon procedure, surgical valvotomy or valve surgery during childhood and continue to require follow-up as adults.
Many people with congenital aortic valve disease remain well for decades. However, the valve and the aorta can change over time, so lifelong surveillance is often important.
What can happen to a bicuspid or abnormal aortic valve?
There are two main problems that can develop.
Aortic stenosis
Aortic stenosis means that the valve has become narrowed and does not open fully.
The left ventricle must generate higher pressure to pump blood through the narrowed valve. Over time, this can cause the heart muscle to become thicker.
Aortic stenosis can range from mild to severe and may gradually progress over many years.
Aortic regurgitation
Aortic regurgitation means that the valve does not close completely, allowing some blood to leak backwards from the aorta into the left ventricle.
If the leak is significant, the left ventricle has to pump a larger volume of blood with each heartbeat. Over time, this can cause the ventricle to enlarge.
Some people have a combination of both aortic stenosis and aortic regurgitation.
What about the aorta?
Bicuspid aortic valve can also be associated with enlargement of the aorta, particularly the section closest to the heart.
This is called aortic dilatation or aortopathy.
For this reason, follow-up for bicuspid aortic valve involves looking not only at the valve but also at the size of the aorta.
The aorta may remain stable for many years. In some people, however, it gradually enlarges and requires closer surveillance. If it becomes significantly enlarged, surgery may eventually be recommended to prevent complications.
The decision about when an enlarged aorta should be treated depends on several factors, including its size, the rate at which it is changing, body size, family history, the underlying valve condition and whether heart surgery is required for another reason.
Does everyone with a bicuspid aortic valve need treatment?
No.
Many people with a bicuspid aortic valve have a valve that functions well and an aorta that is normal or only mildly enlarged. In this situation, regular monitoring may be all that is required.
Treatment is generally considered when:
Aortic stenosis becomes severe
Aortic regurgitation becomes severe and begins to affect the size or function of the heart
Symptoms develop because of significant valve disease
The aorta becomes significantly enlarged
There is another important complication related to the valve or aorta
Your cardiologist will monitor for these changes and advise if treatment becomes necessary.
How is congenital aortic valve disease treated?
Treatment depends on your age, valve anatomy, previous procedures and whether the main problem is stenosis, regurgitation or both.
Balloon valvuloplasty
In children and younger people with congenital aortic stenosis, a balloon valvuloplasty may sometimes be used.
A catheter is passed through a blood vessel to the heart and a balloon is inflated across the valve to improve its opening.
This can relieve obstruction but does not make the valve normal. Some people develop aortic regurgitation afterwards, and the valve can become narrowed again later in life.
Aortic valve repair
In selected patients, particularly younger people with suitable valve anatomy, it may be possible to repair the person’s own aortic valve rather than replace it.
Repair techniques vary considerably and depend on the precise structure of the valve and aorta. Not every bicuspid or congenital aortic valve is suitable for repair.
Aortic valve replacement
When the valve can no longer function adequately, it may need to be replaced.
There are different types of replacement valves.
A mechanical valve is very durable but usually requires lifelong anticoagulation with warfarin.
A tissue valve does not usually require lifelong anticoagulation solely because of the valve, but it has a limited lifespan and may eventually require another procedure.
The best choice is different for each person and depends on factors including age, lifestyle, pregnancy plans, other medical conditions and the likelihood of future procedures.
In some patients, catheter-based aortic valve replacement (TAVI/TAVR) may be an option. Its role depends on age, anatomy, previous surgery and other individual factors and is different from its use in older people with acquired aortic valve disease.
What is the Ross procedure?
The Ross procedure is an alternative type of aortic valve replacement that may be considered in selected children, teenagers and younger adults.
Instead of inserting an artificial valve, the person’s own pulmonary valve is removed and used to replace the diseased aortic valve. A donor valve or conduit is then placed in the pulmonary position.
One advantage is that the replacement aortic valve is living tissue and can provide excellent blood flow without requiring lifelong anticoagulation solely because of the valve. This can be particularly attractive for some young and active people and for some women considering future pregnancy.
However, the Ross procedure is a complex operation that effectively turns a one-valve condition into a two-valve operation. Both the new aortic root (the pulmonary autograft) and the pulmonary conduit require long-term surveillance and may require further intervention later in life.
Outcomes are strongly influenced by appropriate patient selection and surgical expertise. For this reason, the Ross procedure is generally considered in specialist centres with experience in the technique.
For some younger patients requiring aortic valve replacement, it is worth discussing whether the Ross procedure is an appropriate option alongside mechanical valve replacement, tissue valve replacement and, where possible, valve repair.
How to stay well
Keep up with your heart checks
Bicuspid aortic valve and other congenital aortic valve conditions can change gradually over time, even when you feel completely well.
The frequency of follow-up depends on the severity of your valve disease and the size of your aorta.
Some people with mild disease may only need assessment every few years, while people with significant valve disease or aortic enlargement may require review every 6–12 months.
Know your aortic measurements
If your aorta is enlarged, it can be useful to know which part of the aorta is enlarged and its approximate size.
Your cardiologist will monitor whether this measurement remains stable or changes over time.
Blood pressure matters
High blood pressure places additional stress on the heart and aorta.
Have your blood pressure checked regularly and treat high blood pressure appropriately. Maintaining a healthy weight, exercising regularly, reducing excess salt intake and avoiding smoking are also important for long-term cardiovascular health.
Stay active
Regular exercise is encouraged for most people with bicuspid aortic valve disease.
If your valve functions normally and your aorta is not significantly enlarged, there may be few or no exercise restrictions.
Recommendations may change if you have:
Moderate or severe aortic stenosis
Significant aortic regurgitation
An enlarged aorta
Reduced heart function
Symptoms during exercise
People with significant aortic enlargement may be advised to avoid very heavy lifting or intense straining.
If you participate in competitive sport, heavy resistance training or very high-intensity exercise, discuss this specifically with your cardiologist.
Look after your teeth
Good dental hygiene is an important part of looking after your heart.
People with abnormal or replacement heart valves have an increased lifetime risk of infective endocarditis, an infection involving the heart valves.
Regular dental care and maintaining healthy teeth and gums are important.
Having a bicuspid aortic valve alone does not usually mean you need antibiotics before dental procedures. Antibiotic prophylaxis is recommended for certain higher-risk groups, including some people with prosthetic heart valves or a previous episode of infective endocarditis.
Your cardiologist or dentist can tell you whether this applies to you.
Don’t smoke or vape
Smoking contributes to cardiovascular and vascular disease and should be avoided.
Does bicuspid aortic valve run in families?
Bicuspid aortic valve and associated aortic enlargement can occur in families.
Because of this, screening of first-degree relatives — parents, brothers, sisters and children — with an echocardiogram is often recommended.
Your cardiologist can advise whether family screening is appropriate in your situation.
Pregnancy
Most women with a bicuspid aortic valve and mild valve disease with a normal-sized aorta can have a successful pregnancy.
Pregnancy increases the workload on the heart and also produces changes in the aorta. The risk therefore depends on:
The severity of aortic stenosis or regurgitation
Heart function
The size of the aorta
Previous aortic or valve surgery
Other associated heart conditions
Women with significant aortic valve disease or aortic enlargement should have pre-pregnancy assessment with a cardiologist experienced in congenital heart disease and pregnancy.
If you have a mechanical valve, pregnancy requires particularly careful planning because of the complexities of anticoagulation.
Symptoms to tell your doctor about
Contact your doctor or cardiology team if you develop:
New shortness of breath during exercise
A noticeable reduction in exercise capacity
Chest discomfort during exertion
Dizziness or feeling faint during exercise
New fainting
Frequent or prolonged palpitations
New swelling of your ankles
A significant change in your usual symptoms
Symptoms are particularly important in people with moderate or severe aortic stenosis and should not simply be attributed to getting older or becoming less fit.
Emergency — get help immediately if
Seek urgent medical attention or call 000 if you develop:
Sudden severe chest, back or abdominal pain
Fainting during exercise
Severe or rapidly worsening shortness of breath
Chest pain associated with breathlessness, sweating or feeling very unwell
Sudden weakness, difficulty speaking or other symptoms suggesting a stroke
Sudden severe chest or back pain is particularly important in someone known to have significant enlargement of the aorta.
Your regular check-ups
Depending on your condition, follow-up may include:
Clinical review
Your cardiologist will ask about exercise capacity, chest discomfort, breathlessness, dizziness and palpitations and check your blood pressure and heart.
ECG
An electrocardiogram records the electrical activity and rhythm of your heart.
Echocardiogram
An ultrasound of the heart assesses how well the aortic valve opens and closes, the size and function of the left ventricle and parts of the aorta.
Cardiac MRI or CT
MRI or CT may be used to obtain more detailed measurements of the aorta when it cannot be fully assessed by echocardiography. Cardiac MRI can also accurately assess heart size, function and the amount of aortic regurgitation.
Exercise testing
Exercise testing can sometimes help determine how significant valve disease is and whether it is affecting your exercise capacity.
Questions for your doctor
Do I have a bicuspid aortic valve or another type of congenital aortic valve disease?
Does my valve have stenosis, regurgitation or both?
Is my aorta enlarged, and what does it currently measure?
How often should my valve and aorta be checked?
Do I have any restrictions on exercise or heavy lifting?
Should my parents, siblings or children have an echocardiogram?
If I eventually need valve surgery, what options would be suitable for me?
Would valve repair or a Ross procedure ever be an option for me?
Is there anything I need to consider before pregnancy?
This information is intended as a general guide for teenagers and adults with bicuspid aortic valve or congenital aortic valve disease. Individual conditions vary, and your cardiologist can provide advice specific to your valve, aorta, previous treatment and current health.
