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Bicuspid Aortic Valve

Bicuspid aortic valve—also known as bicuspid aortic valve disease or BAV heart disease—is a congenital heart abnormality that people are born with. People with bicuspid aortic valve are born with two leaflets instead of the usual three. This structure makes bicuspid valves more prone to early dysfunction, including aortic valve regurgitation (backflow of blood) and aortic valve stenosis (narrowing of the valve). If left untreated, these issues can lead to serious complications, such as heart failure.

What is a bicuspid aortic valve?

In a healthy heart, there are three flaps of tissue called "leaflets" or "cusps" that close the valve between the heart and the body's largest artery, called the aorta. In a heart with a bicuspid aortic valve, there are only two cusps instead of the usual three.

Bicuspid aortic valve is the most common congenital heart condition, affecting about 1-2% of the general population.
 

Bicuspid vs. tricuspid aortic valve

Normally, the aortic valve is tricuspid (has three leaflets), but in some cases, it forms with only two leaflets (bicuspid) at birth. Without the third leaflet, the valve may not function properly, leading to potential issues such as:

  • Bicuspid aortic valve regurgitation: The valve doesn't seal completely, allowing blood to flow back into the heart.
  • Bicuspid aortic valve stenosis: The valve doesn't open fully, restricting blood flow from the heart.
  • Heart murmur: Problems with the valve can cause an unusual sound when your heart beats. Your doctor may hear it with a stethoscope.
  • Endocarditis: The valve leaflets can become infected, posing additional health risks.

Causes of bicuspid aortic valve

The causes of congenital bicuspid aortic valve are unknown. However, it has a hereditary component, even if bicuspid aortic valve genetics are still somewhat unclear.

Much is still unknown about the genetics of BAV. It's unclear if the bicuspid aortic valve is inherited from the mother or father, but we know that a bicuspid aortic valve is hereditary. Men are twice as likely as women to be born with the condition.

Current bicuspid aortic valve family screening guidelines recommend screening first-degree relatives, but some studies have suggested that it may be wise to screen second-degree relatives as well.

A genetic disorder called Turner syndrome affects women who are missing one of their X chromosomes. When this happens, they can have a variety of developmental problems, including a bicuspid aortic valve.

Bicuspid aortic valve symptoms

A bicuspid aortic valve may work well for years before it causes symptoms, so it's uncommon to see bicuspid aortic valve symptoms in children. Instead, bicuspid aortic valve symptoms usually appear in middle age, once the condition has progressed, as the valve is predisposed to early failure. When you develop symptoms, you may experience:

The symptoms of BAV heart disease are common to many conditions, especially heart conditions, so consult your doctor to determine the cause of your symptoms.

Bicuspid aortic valve diagnosis

Doctors usually diagnose bicuspid aortic valve with an echocardiogram, an imaging test that uses sound waves to create real-time images of the heart and show blood flow. It allows the doctor to see how the aortic valve is functioning. They may also perform other imaging tests, such as a CT scan or chest X-ray to examine the anatomy of the heart or an electrocardiogram (EKG/ECG) to evaluate the heart's rhythm.

Bicuspid aortic valve treatment

Some people may benefit from medication, but if symptoms are severe, your doctor will usually recommend bicuspid aortic valve surgery, such as bicuspid aortic valve replacement or bicuspid aortic valve repair.

Depending on the nature and severity of the disease, you may receive either minimally invasive or open-heart surgery. The average age of bicuspid aortic valve replacement is about 60 years old. With proper care, including valve surgery when necessary, the life expectancy of a person with a bicuspid aortic valve is the same as that of the general population.

FAQs about bicuspid aortic valve

Some people remain asymptomatic or without serious complications for much of their life. However, some patients develop complications, so getting appropriate care is vital.

BAV is a congenital heart defect, meaning it is present at birth.

Yes. BAV is a form of congenital heart disease. 

BAV is the most common congenital heart condition, affecting about 1-2% of the general population.

Hypertension (high blood pressure) is a common side effect of a bicuspid aortic valve.

No, heart valves generally don't heal on their own. While many people don't experience symptoms for much of their lives, there's no sure way to fix a bicuspid aortic valve except with surgery.