Bicuspid aortic valve disease is a heart condition you're born with. It occurs in 1-2% of people.
A bicuspid aortic valve has two flaps, called leaflets, instead of the normal three. This can lead to problems with blood flow from the heart to the aorta, the body's main artery.
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The aortic valve is a one-way valve that connects the heart and the aorta. Blood flows from the heart to the aorta, the main artery that carries oxygen-rich blood away from the heart to the rest of the body.
A normal aortic valve has three flaps, called leaflets. The flaps open and close to allow blood to flow into the body.
A bicuspid aortic valve only has two flaps. Over time, these flaps, or leaflets wear out more quickly than normal leaflets. This can result in either aortic stenosis (a condition in which the valve becomes thickened and calcified and does not open normally), or aortic insufficiency (a condition in which the aortic valve does not fully close, allowing blood to leak backward).
About 1-2 percent of all people in the U.S. are born with a bicuspid valve. It's far more common in males than in females, with twice as many men having the disease.
The exact cause of bicuspid aortic valve disease is unknown. Bicuspid aortic valve can be inherited. This means that it can have a genetic cause and be passed down in families, but it is not always genetic. It occurs in up to 25% of first-degree relatives of people with a bicuspid valve.
In the early stages of pregnancy, as the aortic valve is developing, two of the three leaflets, or flaps, fuse together. This results in two leaflets instead of three.
People who have a parent with bicuspid aortic valve are more likely to develop the condition. Males are more likely to develop the condition than females.
In some cases, people with a bicuspid aortic valve aren't aware they have it. A doctor may find the problem when they listen to the heartbeat and hear a murmur, or an abnormal sound caused by problems with blood flow.
In rare and severe cases, a child born with the condition may need surgery soon after birth.
In adulthood, bicuspid aortic valves can lead to aortic stenosis, aortic insufficiency, or a combination of both:
Both aortic insufficiency and aortic stenosis cause the heart to work harder to push blood to the rest of the body. Both aortic stenosis and aortic insufficiency can strain and weaken the heart, leading to symptoms such as shortness of breath, chest pain, lightheadedness, and leg swelling.
People with a bicuspid aortic valve are also known to be at risk for developing ascending aortic aneurysms. The aorta is the largest artery in the body, and it carries blood from the heart to every area and organ in the body. An aneurysm happens when the vessel enlarges, causing the walls to become thinner and weaker. Development of an ascending aortic aneurysm occurs in about 30% of patients with a bicuspid aortic valve. It is suggested that this is due to underlying genetic factors as well as differences in blood flows across the aortic valve.
Bicuspid valves are known to deteriorate and develop disease sooner than normal, tri-leaflet aortic valves. People with a bicuspid valve may develop symptoms of aortic valve disease in their middle-age, as early as in their 30’s and 40’s. Once you are diagnosed with a bicuspid valve, it is important to follow closely with a cardiologist and have regular echocardiograms. When watched carefully, and treated with surgery at the appropriate time, patients with bicuspid aortic valves have normal life expectancy. If not monitored and not treated, bicuspid aortic valve can lead to severe aortic valve disease causing heart failure and even death.
Bicuspid aortic valve is congenital and often genetic condition, so there is no way you can prevent it. However, with routine follow-up care and a heart-healthy lifestyle, you may be able to reduce your risk of developing serious complications.
In most cases of bicuspid aortic valve disease, the valve works normally until middle age. Most people do not develop symptoms until the valve is significantly diseased, and some people never develop symptoms. When the bicuspid valve develops aortic insufficiency or aortic stenosis it can cause symptoms such as:
A heart murmur. Your doctor can detect this by listening to your heart.
If you have a family history of bicuspid aortic valve, you should talk to your doctor. You should also talk to your doctor if you begin experiencing bicuspid aortic valve symptoms.
Although people are born with a bicuspid aortic valve, it often goes undiagnosed until adulthood. One exception is in cases when a newborn has a severe valve defect and needs treatment right away.
During your physical exam, your doctor will:
A doctor may order further tests if they hear a heart murmur or if you are having symptoms.
To diagnose bicuspid aortic valve or associated aortic valve disease , your doctor may order tests such as:
Many people with bicuspid aortic valves lead normal, active lives. When diagnosed early, routine check-ups and early treatment can prevent serious complications.
Most people with bicuspid aortic valves need surgery in adulthood. Some children with severe aortic stenosis at birth, may need surgery right away.
About 20 percent of people who are born with a bicuspid aortic valve will not need surgery.
After you are diagnosed with a bicuspid aortic valve, your doctor will monitor your condition to watch for the development of valve disease and aortic aneurysm.
Based on how complex your condition is, you may need valve repair or valve replacement surgery. Your doctor can advise you which type is right for you. Surgical treatment options include:
If you have regurgitation — that is, blood flowing back to the heart — you may need valve repair surgery.
Your surgeon makes and incision in the middle of the chest and repairs the valve so that the flaps close properly. This is open heart surgery and requires the heart-lung machine.
The David procedure is a special type of valve repair for people who have an aortic root aneurysm and a valve that can be fixed.
With this technique, your surgeon:
During this procedure, your surgeon:
Some people who can't have open-heart surgery may be a good fit for TAVR. This minimally invasive technique accesses the heart through a catheter inserted in an artery in the leg or groin. The surgeon inserts a new valve within the existing damaged valve.
Typically, patients with a bicuspid valve cannot have TAVR because of the abnormal shape. At UPMC, our team of TAVR experts will determine if you are a candidate. Most patients with bicuspid aortic valve require traditional aortic valve replacement through open heart surgery.
Aortic valve surgery is an effective treatment option for bicuspid aortic valve disease that is causing symptoms or complications.
UPMC provides expert care for people living with bicuspid aortic valve disease. We offer the latest valve repair and replacement surgeries to treat your heart problem. In addition to caring for an incredibly high volume of patients with bicuspid aortic valve disease, we have outstanding outcomes.
Last reviewed by a UPMC medical professional on 2024-10-01.
