Bicuspid aortic valve: symptoms, diagnosis, treatment and monitoring

The aortic valve sits between the left pumping chamber of the heart and the aorta, the large artery that carries blood to the rest of the body. The British Heart Foundation explains that heart valves have small flaps, called leaflets or cusps, that open and close with every heartbeat to stop blood flowing backwards.

A normal aortic valve has three leaflets. In a bicuspid aortic valve there are only two. The British Heart Foundation describes this as the most common congenital valve problem, which means it is present from birth rather than something that develops later in life.

Many people have a bicuspid valve for years without knowing. The NHS notes that some people with heart valve disease have no symptoms at all, and the condition may only come to light during a scan or when a doctor hears an unusual sound while listening to the heart.

Many people with a bicuspid aortic valve have no symptoms for years. The British Heart Foundation says some people with heart valve disease have no symptoms, while others have one or more. When a bicuspid valve starts to cause problems, the symptoms are those of heart valve disease in general. The NHS lists shortness of breath, feeling tired or weak, dizziness, palpitations, swollen ankles and feet, and chest pain or discomfort. These symptoms have many possible causes, and having them does not definitely mean valve disease, but the NHS says it is important to have them checked.

A doctor may also hear a heart murmur, a swishing sound, when listening to the heart with a stethoscope. The British Heart Foundation says this can be a sign of a heart valve problem.

• See a GP if you are regularly short of breath, feel tired, weak or dizzy during normal activities, have palpitations that keep coming back, have swollen ankles or feet, or have chest pain that comes and goes (NHS advice).

• Call 999 or go to A&E if you are struggling to breathe, have sudden chest pain that spreads to your arms, back, neck or jaw or lasts more than 15 minutes, or have chest pain with shortness of breath or palpitations (NHS advice).

• The British Heart Foundation also lists chest pain that does not go away at rest, fainting and losing consciousness as reasons to call 999.

• It advises contacting your doctor for an urgent appointment if symptoms are new or getting worse, or if you have a temperature, night sweats or weight loss with no obvious reason, or have been unwell for two to three weeks.

If you have a bicuspid aortic valve without significant disease, the emphasis is on monitoring. The British Heart Foundation says that mild valve disease without symptoms may involve check-ups with an echocardiogram every 3 to 5 years, while moderate or severe disease without symptoms means a check-up and echocardiogram every 6 to 12 months. New or worsening symptoms may mean the disease is progressing and should prompt an appointment.

Treatment is for valve disease that causes symptoms or is severe. The NHS says this can include medicines to lower blood pressure and protect the heart, reduce fluid build-up, slow the heart rate or reduce the risk of blood clots, and that severe disease usually needs surgery to repair the valve or replace it with an artificial valve or one made from human or animal tissue. The British Heart Foundation says the type of surgery depends on the cause of the valve problem, its effect on the heart and your quality of life.

The NHS lists possible complications of heart valve disease as heart failure, atrial fibrillation, stroke and endocarditis. Your care team will tell you what to expect for your own valve.

A GP will listen to your heart and may arrange tests or refer you to a cardiologist, the NHS says. The British Heart Foundation says the most common test for diagnosing heart valve disease is an echocardiogram, an ultrasound scan of the heart, and that other tests may include a chest X-ray, ECG, blood tests, cardiac CT scan, cardiac MRI or angiogram.

The NHS describes the usual echocardiogram as a scan done by a sonographer, with gel on the chest and an ultrasound probe, lasting around 30 to 40 minutes. A transoesophageal echocardiogram, using a thin tube passed down the gullet with the throat numbed by spray, is sometimes used for a more detailed image. Results may be discussed on the day or at a follow-up appointment.

The NHS adds that congenital heart disease is screened for at the 20-week pregnancy scan, but the scan cannot find every condition, and sometimes a problem is not diagnosed until adulthood. This is one way a bicuspid valve may first be noticed later in life.

The NHS explains that heart valve disease happens when a valve either does not open fully (stenosis) or does not close fully and may be leaky (regurgitation). The British Heart Foundation also describes valve prolapse, where the valve becomes floppy and does not close properly. In each case the heart has to work harder to pump blood around the body. The NHS says valve disease is most common in the aortic or mitral valves, on the left side of the heart.

The British Heart Foundation lists being born with an abnormal valve, such as a bicuspid aortic valve, among the main causes of heart valve disease, alongside rheumatic fever, cardiomyopathy, damage from a heart attack, getting older and infection of the heart (endocarditis). Having a bicuspid valve is therefore a reason for monitoring rather than a diagnosis of illness in itself.

When valve disease is found, doctors describe it as mild, moderate or severe. The British Heart Foundation says mild disease is more common, that people with mild disease and no symptoms might not need treatment, and that most have long lives. The NHS lists possible complications of heart valve disease as heart failure, atrial fibrillation, stroke and endocarditis, and says your care team will tell you what to expect for your own valve.

The British Heart Foundation says many people live well with heart valve disease, and recommends safe exercise, a healthy weight, a balanced diet, stopping smoking and cutting down on alcohol. It suggests asking your doctor or nurse specialist about exercise, diet, school or work, holidays and flying, contraception, driving, having children, and antibiotics and dental visits.

On exercise, the British Heart Foundation says you may need to avoid activities where you strain or hold your breath, such as heavy lifting, weight training or swimming, depending on your condition and symptoms. The NHS says your care team may also advise avoiding strenuous exercise.

The NHS recommends that anyone with heart valve disease who is pregnant or planning a pregnancy gets advice from a heart specialist, because pregnancy can make symptoms worse. With planning, the British Heart Foundation says most people with heart valve disease will not have complications in pregnancy.

It is normal to feel anxious after a diagnosis. The British Heart Foundation says you can refer yourself to NHS talking therapies if low mood or anxiety is affecting daily life.

A bicuspid aortic valve is congenital, so it is present from birth. The NHS says congenital heart disease develops while the heart is still being formed in the womb, that sometimes problems are caused by an altered gene passed on from one of the parents, and that often there is no clear reason why someone is born with it. The sources used for this page do not give a family risk figure for bicuspid valves in particular.

The NHS also notes that congenital heart disease is screened for at the 20-week pregnancy scan, but the scan cannot find every condition, and a problem is sometimes not diagnosed until adulthood. If you have a bicuspid valve and are wondering whether relatives should be checked, ask your cardiology team.

Referral to cardiology, echocardiography and valve surgery are all part of NHS care, and hospitals often have a specialist valve service with a nurse specialist as a point of contact. Some people also arrange a private cardiology appointment or scan. If you do, share your previous echocardiogram reports so measurements can be compared over time, and keep your NHS team informed.