Cleveland Clinic Children's doctor examining a child patient.

Bicuspid Aortic Valve

Close-up of a surgeon surrounded by other surgeons in an operating room.

Need Bicuspid Aortic Valve care for an adult?

You may have been worried about your child’s constant low energy — but you probably didn’t expect to discover they have a heart condition.

Learning your child has a congenital (born with it) heart condition like bicuspid aortic valve disease (BAVD) may leave you feeling uncertain and nervous. And it’s likely you have many questions about your child’s treatment options and their future.

Cleveland Clinic Children’s pediatric healthcare providers are here to give you answers and ease your concerns. We treat heart conditions like bicuspid aortic valve disease in children of all ages. You can rest easy knowing your child will get excellent care from an experienced and compassionate team of heart specialists and other providers.

High-volume specialty:

Our pediatric heart surgery team does hundreds of heart valve procedures on children each year. Our pediatric cardiologists, cardiothoracic surgeons, anesthesiologists and nurses are highly experienced. They provide outstanding, compassionate care before, during and after surgery.

Personalized care:

We take a family-centered approach to care, communicating with you every step of the way. We listen and guide you as you make important decisions about your child’s care, including whether surgery is needed. We also connect you with helpful resources. Meet our team.

Collaborative care:

If your child is born with a bicuspid aortic valve, they’ll need ongoing care throughout their life from a team of many specialists. We offer exceptional continued, collaborative care into their adult years.

Minimally invasive options:

We specialize in pediatric heart valve surgery that uses catheters (small tubes) to replace or repair heart valves. If your child needs surgery, this approach leaves smaller scars and is less invasive than open-heart surgery, which helps them recover faster.

Innovation and research:

Cleveland Clinic Children’s research helps find new treatment options and improve the quality of life for children with heart valve conditions. Our pediatric heart surgeons regularly use advanced imaging techniques and 3D and 4D printed models to guide and personalize complex heart valve repair surgeries. They pioneer new surgical approaches and make existing ones better to improve outcomes and reduce recovery time.

National recognition:

U.S. News & World Report consistently ranks Cleveland Clinic Children’s a top hospital in the nation. Newsweek has also named Cleveland Clinic a top hospital in the world.

Virtual visits:

We make it easy for your child to see their providers when needed. Sometimes, they can even do this from the comfort of home. All you need is an internet connection and a smartphone, tablet or computer. Talk with your child’s care team to see if virtual visits are an option.

Bicuspid Aortic Valve Diagnosis at Cleveland Clinic Children’s

Bicuspid aortic valves are the most common form of heart condition someone is born with. About 1 to 2% of people are born with this condition. Babies born with bicuspid aortic valve disease have only two leaflets on their aortic valve instead of three. This can affect the way blood flows from their heart to the rest of their body.

We can sometimes diagnose a bicuspid aortic valve during pregnancy. This often happens if we find other heart problems during prenatal testing. In most cases, though, we’ll usually diagnose the problem after your baby is born — sometimes not even until they’re older.

Your baby with BAVD may not be growing as they should and have trouble eating. If your child with BAVD is older, they may complain of chest pain, feeling short of breath and heartbeats that feel weird. They might also be sluggish and tired all the time.

If we think your child might have bicuspid aortic valve disease, we’ll order an echocardiogram (echo) to get a closer look at their heart. In addition to a bicuspid aortic valve, the echo may show:

Aortic valve stenosis (narrowing or obstruction of their valve).
Aortic valve regurgitation (leaky valve).
Aortic aneurysm or enlargement.
Other types of valve disease, like mitral valve prolapse.
Other types of congenital heart disease, like aortic coarctation.

If we confirm your child has bicuspid aortic valve disease, we’ll provide ongoing checkups and tests to stay on top of any changes in how their heart is working. Your child may not need treatment right away if they don’t have symptoms and their heart works well.

Meet Our Pediatric Bicuspid Aortic Valve Surgery Team

Your child will get the most capable and compassionate care from our team of pediatric heart specialists. Their team may include:

Pediatric cardiologists.
Pediatric cardiothoracic surgeons.
Pediatric anesthesiologists.
Nurses.

Providers Who Treat Bicuspid Aortic Valve

Locations

Our healthcare providers see patients at convenient locations throughout Northeast Ohio and Florida.

Bicuspid Aortic Valve Surgery at Cleveland Clinic Children’s

If your child begins to have symptoms, it could be a sign of aortic valve stenosis, or regurgitation. We may recommend surgery to repair or replace their bicuspid aortic valve to prevent permanent damage to your child’s heart.

Before recommending surgery, we’ll take the time to do some more advanced testing on their heart. This information helps us tailor the safest procedure for your child while focusing on their specific condition. To get a closer look at your child’s heart, your provider may order a:

Valvuloplasty

If your child’s bicuspid aortic valve has caused aortic stenosis, we may recommend balloon valvuloplasty (dilation). This minimally invasive procedure has a quicker recovery time than open heart surgery. Our experienced pediatric cardiac anesthesiologists give your child general anesthesia, so they’re comfortably asleep during the surgery.

Your child’s surgeon will first make a small incision (cut) in your child’s groin and then insert a catheter through a blood vessel. They’ll guide it to the narrowed heart valve where they’ll inflate a small balloon on the catheter’s tip. This balloon will open your child’s heart valve to increase the amount of blood flowing through it. The surgeon will then remove the catheter and close the incision.

Valve repair

If their bicuspid aortic valve isn’t too thick and can be saved, we may recommend repairing the valve. This is often the case if your child has a bicuspid aortic valve with aortic regurgitation. Valve repair is considered only after our 3D and 4D advanced imaging approach. This innovative, industry-leading technique lets our surgeons guide highly personalized valve repair surgeries. If we decide that repairing your child’s valve with surgery is the best option, we’ll give your child general anesthesia, which puts them to sleep and blocks any pain. Then your child’s surgeon will make an incision in their chest to repair and save the valve. They’ll make sure your child’s heart is working well before they close the incision.

Ross procedure

The Ross procedure is another option if your child’s bicuspid aortic valve has caused aortic stenosis or aortic regurgitation. Similarly to valve repair, we’ll give your child general anesthesia. Then your child’s surgeon will make an incision in their chest to remove the defective bicuspid aortic valve and replace it with your child’s pulmonary (lung) valve. The surgeon will then use a donor valve to replace the pulmonary valve. They’ll make sure your child’s heart is working well before they close the incision.

Ozaki procedure

Cleveland Clinic Children’s is one of the few hospitals that offers the Ozaki procedure — an innovative option if your child has a bicuspid aortic valve with aortic stenosis and the leaflets are too thick to save. Your child will be given general anesthesia to put them to sleep during the procedure, just like the other surgeries.

The surgeon will make an incision in your child’s chest and remove the defective bicuspid aortic valve. Then the surgeon will use your child’s own pericardium (tissue sac that surrounds their heart) to reconstruct valve leaflets. They’ll make sure your child’s heart is working well before they close the incision.

Valve replacement

If we can’t do a balloon valvulopasty, Ross procedure or Ozaki procedure, we may recommend replacing your child’s aortic valve. During valve replacement surgery, we’ll remove your child’s bicuspid aortic valve and replace it with a new valve.

Your child’s new aortic valve will either be a biological valve (made from human or animal tissue) or a mechanical one. Before surgery, we’ll talk with you about the pros and cons of each type of valve. We’ll help you make the best choice for your child based on their age, medical history and goals.

Recovery

After your child leaves the hospital, they’ll have several follow-up appointments so our pediatric heart care team can keep an eye on their recovery. Different procedures have different recovery times. We may recommend cardiac rehab to help your child get strong after surgery.

Genetic Screening for Bicuspid Aortic Valve Disease

About 10% of people with bicuspid aortic valve disease have family members who also have the condition. Our team of experts will work with you and your loved ones to screen family members, including siblings, for bicuspid aortic valve disease. If we find the condition early, before symptoms start, we can design a plan for ongoing check-ups and surgery, if needed.

Taking the Next Step

Knowing your child has a lifelong heart condition, like bicuspid aortic valve disease, can be stressful and overwhelming. The good news? It’s treatable. And Cleveland Clinic Children’s is here for your child (and family) at every step, providing the best, most personalized heart care possible. Together, we’ll create a treatment plan that works for your child’s health and lifestyle — one that will protect and repair their heart and improve their quality of life, now and in the future.