Bicuspid Aortic Valve Disease
Key takeaways
Bicuspid aortic valve disease is a congenital heart disease where the aortic valve has two cusps or “flaps” instead of three.
Many people have no symptoms for years, but the condition can lead to valve problems or aortic complications over time.
Bicuspid aortic valve disease is often inherited, and family members may need screening.
More than 50% of patients will require treatment at some point in their lifetime.
Ongoing monitoring and care from a specialized aortic center can help prevent serious complications.
Patients with bicuspid aortic valve disease require steady monitoring and expert-level heart care. UF Health is the only system in Florida to offer a dedicated program through the UF Health Aortic Disease Center focused exclusively on patients with bicuspid aortic valve disease.
Understanding bicuspid aortic valve disease
The aortic valve is located between the left ventricle, the heart's main pumping chamber, and the aorta, the major artery that carries blood throughout the body. The aortic valve normally consists of three cusps or “flaps” that open and close with each heartbeat. Patients with bicuspid aortic valve disease have a congenital heart condition where the valve has only two cusps instead of three.
This condition, which is a congenital heart disease, often leads to an improperly functioning valve that either leaks (aortic valve regurgitation) or develops premature calcium buildup (aortic stenosis). Many patients with bicuspid aortic valve disease are told they have had a heart murmur on exam for many years.
Having a bicuspid aortic valve is relatively common, affecting about 1–2% of the population. It often runs in families and is found in up to 15% of first-degree relatives (parents, siblings and children).
A bicuspid valve may function normally for many years or even decades without symptoms. However, early diagnosis is important to prevent serious complications as well as improve long-term outcomes.
Related conditions and complications
Bicuspid aortic valve disease is often associated with other heart and aortic conditions, including:
Aortic dissection – A tear in the inner layer of the aorta (medical emergency)
Aortic regurgitation – A leaky valve that may lead to an enlarged heart
Aortic valve stenosis – Narrowing of the valve due to calcium buildup
Thoracic aortic aneurysm – Enlargement of the aorta in the chest
Heart failure – When the heart cannot pump effectively
Bicuspid aortic valve symptoms
Most people with bicuspid aortic valve disease do not have symptoms early in life. Over time, symptoms may develop as the valve narrows or becomes leaky.
Typical symptoms include:
Fatigue that comes on easily
Chest pain
Shortness of breath
Swelling in the legs or ankles
Fainting or lightheadedness
Exams and tests for bicuspid aortic valve disease
During an exam, providers may look for signs of bicuspid aortic valve disease, including:
Enlarged heart
Heart murmur
Weak pulse in the wrists or ankles
Imaging and diagnostic tests
Tests commonly used to diagnose and monitor bicuspid aortic valve disease include:
Echocardiogram – Ultrasound to evaluate heart structure and blood flow
CT scan – High-resolution imaging of the heart and aorta
Additional tests may include:
Chest X-ray
Electrocardiogram (ECG)
Cardiac catheterization
Magnetic magnetic resonance imaging (MRA)
Treatment of bicuspid aortic valve disease
More than half of patients with bicuspid aortic valve disease will require a valve intervention at some point in their lifetime. UF Health cardiac surgeons and cardiologists monitor the valve, aorta and heart function regularly using advanced imaging.
If changes are noticed, our care team at the UF Health Aortic Disease Center will develop an individualized treatment plan.
Treatment options may include:
Aortic valve surgery – Repair or replacement of the aorta using traditional or minimally invasive techniques
Aorta surgery – Removal and replacement of a diseased section of the aorta
Hybrid aorta surgery – Combines open and catheter-based approaches
Thoracic endovascular aortic repair (TEVAR) – Minimally invasive repair using a stent graft
Valve-sparing aortic root replacement – Repairs the aorta while preserving the natural valve (including the Florida Sleeve and David procedures)
Transcatheter aortic valve replacement (TAVR) – Minimally invasive valve replacement for select patients
Specialized bicuspid aortic valve care at UF Health
At the UF Health Aortic Disease Center, you will experience:
Excellent outcomes: UF Health surgeons perform a high volume of complex valve and aortic operations every year
Novel treatment options: Including advanced valve repair and valve-sparing techniques not widely available
Multidisciplinary care: Collaboration between cardiac surgeons, vascular surgeons, cardiologists, radiologists, geneticists and state-of-the-art imaging providers
Genetic counseling: Screening and guidance for patients and their families
Prompt access to care: Appointments with specialists typically available within 1–2 weeks
Frequently asked questions
What causes bicuspid aortic valve disease?
Bicuspid aortic valve disease is a congenital condition, meaning it is present at birth. It often runs in families.
Is bicuspid aortic valve disease serious?
It can be. Many people live without symptoms for years, but the condition can lead to valve problems or aortic complications if not monitored.
Will I need surgery?
Not always, but more than half of patients will need a procedure at some point. Regular monitoring helps determine the right timing.
Should family members be screened?
Yes. Because bicuspid aortic valve disease can run in families, first-degree relatives may benefit from screening.
What is the difference between bicuspid aortic valve disease and an aortic aneurysm?
Bicuspid aortic valve disease is a valve condition, while an aortic aneurysm is a weakening or enlargement of the aorta. However, people with bicuspid aortic valve disease are at higher risk of developing aortic aneurysms.
This entry was written by Gabrielle Massari, Marketing Content Writer, and reviewed for accuracy by Eric Jeng, MD. Generative AI was leveraged as part of the content creation process.