The Center for Bicuspid Aortic Valve Diseases

The Penn Medicine Advantage

Care for bicuspid aortic valve disease (BAVD) involves lifelong surveillance and specialized treatment. Penn Medicine is the only health system in the Philadelphia region to offer a comprehensive program specifically for patients with BAVD and associated thoracic aortic aneurysm.

At the Center for Bicuspid Aortic Valve Diseases, you’ll find:

• Excellent outcomes: Penn surgeons perform the highest number of valve surgeries in Pennsylvania, leading to the best possible outcomes for patients.
• Innovative treatment: For select patients with BAVD, Penn’s renowned surgeons conduct procedures other hospitals can’t, such as surgical repair of bicuspid aortic valves. 
• Genetic screening: In collaboration with our genetics team, we provide screening for all first-degree relatives (parents and siblings) of patients diagnosed with BAVD.
• Multidisciplinary team: Our dedicated team of cardiac surgeons, cardiologists, radiologists, geneticists and advanced practice providers work together to treat every aspect of BAVD and associated aortic aneurysm.
• Timely care: We conduct comprehensive clinical evaluations and monitoring. We can usually offer you an appointment with one of our specialists within two weeks.

What is Bicuspid Aortic Valve Disease? 

In BAVD, the aortic valve has only two cusps (or flaps) instead of three. This deformity, which is congenital (present at birth) keeps the valve from functioning properly. With age, a bicuspid valve may lead to additional heart conditions including:

• Aortic dissection, a tear in the wall of the aorta
• Aortic regurgitation (also called aortic insufficiency), which is a leaky valve 
• Aortic stenosis, the narrowing of your aortic valve
• Heart failure, where your heart weakens from working too hard to pump blood
• Thoracic aortic aneurysm, an abnormal bulge in the part of the aorta located in the chest

Bicuspid aortic valve occurs in 2 percent of the population. It can run in families and is found in up to 15 percent of first-degree relatives of people with the condition. A bicuspid valve may function for years without causing symptoms or obvious signs of a problem. Early diagnosis can lead to improved long-term outcomes for patients.

Treatment for Bicuspid Aortic Valve Diseases

Approximately 80 percent of patients with BAVD require valve repair or replacement at some point. Penn’s cardiac surgery and imaging experts monitor your disease regularly with advanced imaging techniques. If there are changes to your valve or aorta, the cardiac team creates an individualized treatment plan.

Our academic surgeons treat BAVD and associated aortic aneurysm disease with procedures including:

• Aorta surgery, an open-chest surgery to repair aortic damage by removing the diseased section and replacing it with a graft   
• Aortic valve surgery, where our cardiac surgeons repair or replace the bicuspid valve using either traditional or minimally invasive heart surgery
• Hybrid aorta surgery, which combines open surgery and catheter-based procedures to repair complex aortic conditions  
• Thoracic endovascular aortic repair (TEVAR), where surgeons thread a synthetic reinforced graft (tube) to the damaged part of the aorta through a catheter inserted in an artery in your leg
• Valve-sparing aortic root replacement, a specialized operation used to repair the aortic root without replacing the patient’s native valve