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Bicuspid Aortic Valve (BAV): Symptoms, Causes, Treatment


A bicuspid aortic valve is a congenital heart defect. About 1% to 2% of the general population is born with BAV, making it the most common congenital heart disease. It occurs more often in boys than girls.

In a healthy heart, the aortic valve has three cusps, also called leaflets or flaps. People with bicuspid aortic valve (BAV) only have two cusps. The valve between the heart and the main artery leading to the body does not form correctly before birth, and the heart must work harder, leading to valve changes and aortic dilation over time.

A bicuspid aortic valve can occur alone or in conjunction with other heart defects, such as coarctation of the aorta or ventricular septal defects. In rare cases, a baby may have a unicuspid aortic valve (only one cusp) or a quadricuspid valve (four).

Normal heart function

In a healthy heart, the left side pumps oxygen-rich blood to the body through the aorta (the large artery bringing blood to the body). The aortic valve sits between the left side of the heart (left ventricle) and the aorta.

As the heart pumps, the three cusps of the valve open to allow blood flow into the aorta; in between pumps, they close tightly to prevent blood from going back into the heart ventricle.

The defect

In a bicuspid aortic valve, the aortic valve only has two cusps and may be thicker or stiffer than a healthy valve. These defects in the valve can cause:

  • Aortopathy: an enlarged aortic root (the first section of the aorta), which can cause a tear in the artery.
  • Aortic valve regurgitation: the valve does not close completely, and blood leaks backward into the ventricle, increasing the workload for the left ventricle; over time, the increased workload can cause damage to the left side of the heart.
  • Aortic valve stenosis: the valve is thick and stiff and cannot open completely, leading to decreased blood flow to the body and an increased workload for the heart.
  • Enlargement of the heart's left ventricle: the part of the heart that pumps oxygen-rich blood to the body becomes overworked and stiff, making it difficult to pump effectively.

BAV symptoms

Most people with aortic valve stenosis do not develop symptoms until they become adults, and some will not develop symptoms at all. In most cases, the valve will degenerate over time, and symptoms start appearing in middle age.

Symptoms may include:

  • Heart palpitations (irregular heartbeats) or skipping a beat
  • Shortness of breath with exertion
  • Persistent, unexplained fevers
  • Chest or back pain
  • Lightheadedness
  • Heart murmur
  • Fainting

BAV causes

Bicuspid aortic valve defects are congenital, passed down from one family member to another.

While the baby is forming in the womb, the heart valve does not develop correctly; two of the three cusps on the aortic valve become fused together, and the valve cannot open and close normally.

Often, symptoms of BAV will never surface, and the heart defect will not be a problem. But certain conditions can make the heart valve more likely to cause problems:

  • Chronic Kidney disease
  • High Blood pressure
  • High cholesterol
  • Smoking

Diagnosis

A heart murmur may be the first BAV symptom your doctor discovers; they may order tests to confirm the heart defect and help plan treatment. Your doctor may order a chest x-ray, CT, MRI, EKG, echocardiogram, or other tests.

BAV treatment

You and your doctor will decide on a treatment plan based on the severity of your condition. You may only need regular check-ups and CT scans to check for aortic valve regurgitation, valve stenosis, or artery enlargement.

There are no medications to treat BAV, but your doctor may want you to take medication to control your blood pressure and cholesterol or improve your heart's efficiency.

Your doctor may recommend a balloon valvuloplasty to stretch a thickened and stiffened valve: Your doctor will place a catheter into your bloodstream through an artery in your groin; guide it up into your heart and through the aortic valve; then inflate a "balloon" to expand the opening of the valve. The balloon is then deflated and removed along with the catheter. Since bicuspid aortic valves tend to narrow again after some time, you may need more than one of these procedures.

About 80% of people with bicuspid aortic valve disease will need a repair or replacement of the aortic valve.

If your doctor recommends a valve replacement, they will discuss the benefits and risks of each type of valve with you and help you decide which one is right for you.

If your aortic root is enlarged and narrowed, your doctor may replace that section of the aorta with a synthetic one, and they may be able to replace the valve during the same surgery.

Approximately 30% of people who need a valve replacement will also need surgery on the aorta or the aortic sinuses (where the arteries bringing blood to the heart branch off the aorta).

You will need life-long care by a cardiologist to monitor your heart for any changes or infections.

Complications

An untreated bicuspid aortic valve can cause heart failure with symptoms of shortness of breath, fatigue, and swelling in the extremities.

Other potential complications include:

  • Aortic aneurysm
  • Infective endocarditis: an infection of the lining of the heart
  • Left ventricular hypertrophy: thickening of the left side of the heart muscle
  • Arrhythmias, such as atrial fibrillation
  • Sudden cardiac death: this is very rare and usually occurs after strenuous exercise

Talk to your doctor if you are pregnant or plan to become pregnant; even if you don't usually have symptoms from your defective valve, you may need to receive treatment during your pregnancy.

BAV management

You cannot prevent BAV, but you can effectively manage it with some simple lifestyle changes.

  • Don't smoke.
  • Eat a heart-healthy diet.
  • Maintain a healthy weight.
  • Manage your blood pressure.
  • Keep your cholesterol under control.
  • Take your medications as prescribed.
  • Avoid chemical stimulants, such as caffeine or drungs.
  • Practice good dental hygiene to lower your risk of endocarditis.
  • Exercise regularly; always check with your doctor before beginning a new exercise.

Your doctor may prescribe antibiotics before dental surgery or blood thinners after a valve replacement.

Key take-aways

Bicuspid aortic valve is a congenital heart defect; the three aortic valve cusps fuse into only two while the fetus develops. The heart must work harder to provide the body with blood.

Treatment depends on the severity of the symptoms and can range from monitoring to surgery.

Call your doctor if your BAV symptoms are getting worse. Call 9-1-1 if you are having chest pain or severe shortness of breath.

Sources:

Mayo Clinic. (2022). Bicuspid aortic valve - Overview - Mayo Clinic

Johns Hopkins. (2022). Bicuspid Aortic Valve | Johns Hopkins Medicine

PennMedicine. (2022). Bicuspid Aortic Valve - Symptoms and Causes (pennmedicine.org)

Nationwide Children's (2021). Bicuspid Aortic Valve (BAV): Causes, Symptoms, Diagnosis and Treatment (nationwidechildrens.org)

Mubarik, Ateeq, et al. (2022). Bicuspid Aortic Valve - StatPearls - NCBI Bookshelf (nih.gov)

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