Understanding Bicuspid Aortic Valve Disease

The aortic valve is located between the left ventricle, which is the main pumping chamber of the heart, and the aorta, the major artery conveying blood throughout the body. The aortic valve normally consists of three cusps that open and close with each heartbeat. Patients with BAVD, which is part of the heart and vascular care medical specialty, have a congenital anomaly where the valve has only two cusps instead of three.

This condition, which a person is born with, often leads to an improperly working valve that either leaks or develops premature buildup of calcium. Many patients with BAVD are told they have had a heart murmur on exam for many years.

A bicuspid valve may often be associated with additional heart conditions:

• Aortic dissection – A tear in the inner layer of the aorta that represents a medical emergency
• Aortic regurgitation (also called aortic insufficiency) – A leaky valve and may lead to a dilated heart
• Aortic stenosis – Calcification which narrows the opening of your aortic valve
• Heart failure – The heart does not pump properly from working too hard and can be related to faulty heart valves
• Thoracic aortic aneurysm – An abnormal dilation in the part of the aorta located in the chest

Having a bicuspid aortic valve is very common and can be seen in 1-2% of the population. It often occurs in families and is detected in up to 15% of first-degree relatives of people with the condition. A bicuspid valve may work normally for many years without causing symptoms or signs of a problem. An early diagnosis is important to prevent the potential serious complications of BAVD and can lead to superior long-term patient outcomes.

Symptoms

Most of the time, BAV is not diagnosed in infants or children because it causes no symptoms. However, the abnormal valve can leak or become narrow over time.

Symptoms of such complications may include:

• Easy fatigue
• Chest pain
• Shortness of breath
• Swelling in the legs or ankles
• Loss of consciousness (fainting) or lightheadedness

Exams and Tests

During an exam, our health care providers will examine you for likely signs of a BAV, including:

• Enlarged heart
• Heart murmur
• Weak pulse in the wrists or ankles

Tests that may be ordered include:

• CT scan – Provides a detailed image of the heart, lungs, aorta and other blood vessels
• Echocardiogram – An ultrasound that views the heart structures and blood flow inside the heart

If the provider suspects complications or additional heart defects, other tests may include:

• Chest X-ray
• Electrocardiogram (ECG) – Measures the electrical activity of the heart
• Cardiac catheterization – A procedure in which a thin tube (catheter) is placed into the heart to see blood flow into the arteries on the surface of the heart and take accurate measurements of blood pressure and oxygen levels
• MRA – A specialized MRI that uses a dye to view the blood vessels of the heart

Treatment

More than 50% of patients with BAVD will require a valve intervention at some point in their lifetime. UF Health cardiac surgery and cardiologists monitor your valve, aorta and heart function regularly with advanced imaging techniques, such as echocardiograms or high-resolution CT scans. If there are changes to your valve or aorta, the BAVD team develops a treatment plan tailored to the individual.

Our surgeons treat BAVD and associated aortic aneurysms with operations, including:

• Aortic valve surgery – An operation in which our surgeons can either repair or replace the bicuspid valve using traditional or minimally invasive heart surgery
• Aorta surgery – An open operation to fix an aortic aneurysm by removing the entire diseased segment and replacing it with a permanent prosthetic graft
• Hybrid aorta surgery – Combines open surgery and catheter procedures to repair complex aortic conditions without a prolonged complex open operation
• Thoracic endovascular aortic repair (TEVAR) – A procedure in which surgeons use a reinforced graft (tube) to treat the diseased segment of the aorta using a catheter inserted through an artery in your leg
• Valve-sparing aortic root replacement – Specialized operations used to repair an aortic aneurysm without replacing the native aortic valve, such as Florida Sleeve technique (developed at UF Health) or David Reimplantation technique
• Transcatheter aortic valve replacement (TAVR) – A procedure available for select individuals where surgeons replace the aortic valve through an artery in the leg and avoid opening the chest