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Bicuspid Aortic Valve

Bicuspid Aortic Valve

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What is bicuspid aortic valve?

The heart has four chambers and four valves. Blood passes through a valve before leaving each chamber of the heart. Heart valves are made of flaps of tissue called leaflets. These leaflets work like one-way doors, opening to let blood move forward and closing to keep blood from flowing backward. 

The heart’s lower chambers are called ventricles. The left ventricle pumps oxygen-rich blood through the aortic valve and into the aorta, the largest artery in the body. Blood travels from the aorta to blood vessels throughout the body, delivering oxygen and nutrients. 

The aortic valve is located between the left ventricle and the aorta. A healthy aortic valve has three thin leaflets. A bicuspid aortic valve has two leaflets. These leaflets may be thick and can cause the aortic valve to be too narrow.

If the bicuspid aortic valve is too narrow (called aortic stenosis), it can block blood flowing out of the heart. This causes the heart to work harder to pump enough blood to the body. Aortic stenosis can range from mild to severe.

If the leaflets of the aortic valve don’t close all the way, some blood can leak back into the left ventricle. This is called aortic regurgitation, (also known as aortic valve regurgitation or aortic valve insufficiency). Aortic regurgitation can range from mild to severe.

Sometimes, the walls of the aorta can become thin or too wide (dilated). This condition needs to be monitored by your cardiologist.   

Signs and symptoms of bicuspid aortic valve

If your child has mild aortic stenosis or mild aortic regurgitation, they may not experience symptoms, especially in infants or young children. If your child has progressive aortic stenosis or regurgitation, they may experience some or all of these symptoms:

  • Fatigue
  • A heart murmur, which is an abnormal heart sound when a doctor listens with a stethoscope

Sometimes, symptoms may include:

  • Chest pain
  • Fainting
  • Arrhythmias, or abnormal heart rhythm
  • Inability to exercise without tiredness

Testing and diagnosis of bicuspid aortic valve

In the Cardiac Center at Children’s Hospital of Philadelphia (CHOP), a bicuspid aortic valve is usually diagnosed after a child has been referred by their pediatrician. Diagnosis may require some or all of these tests:

  • Pulse oximetry, which is a non-invasive way to monitor the oxygen content of the blood
  • Chest x-ray
  • Electrocardiogram (EKG or ECG), which is a record of the electrical activity of the heart
  • Echocardiogram (also called echo or ultrasound), which is when sound waves create an image of the heart
  • Exercise stress test
  • Cardiac MRI, which is a 3D image that shows the heart's abnormalities

Sometimes, cardiac catheterization will be required. A thin, flexible tube (catheter) is inserted through a vein or artery in the leg and into the heart to provide detailed information about the structure and function of the heart and lungs.

Treatment for bicuspid aortic valve

Treatment for a bicuspid aortic valve depends on how severe the narrowing (stenosis) or leakiness (regurgitation) is. If mild, treatment isn't usually necessary. Your child will need regular checkups with their cardiologist. They may also be prescribed medication if stenosis or regurgitation gets worse.

More severe cases of aortic stenosis or regurgitation may require surgery to repair. 

Valvuloplasty

Depending on your child’s age, needs and valve structure, doctors may try to fix the valve or improve how it works using a surgical procedure called valvuloplasty.

  • Aortic Valve Repair in Children and Teens

    Jonathan Chen, MD: Just like each child is different, so is each heart valve. I'm Jonathan Chen, a surgeon at the Pediatric Heart Valve Center at Children's Hospital of Philadelphia. My team is one of the most experienced in the country in valve repair and replacement. This video will help you understand the kinds of repairs we perform.

    To better understand aortic valve repair, let's first take a look at the anatomy of the heart. This is your heart. It has four chambers and four valves. Blood passes through a valve before entering or leaving each chamber of the heart. The heart's lower chambers are called ventricles. The left ventricle pumps oxygen-rich blood through the aortic valve and into the aorta, the largest artery in the body.

    Blood travels from the aorta to blood vessels throughout the body, delivering oxygen and nutrients.

    Heart valves are made of flaps of tissue called leaflets. These leaflets work like one way doors, opening to let blood move forward, and closing to keep blood from flowing backward. Watch how the leaflets move from another angle, opening to let blood move forward and closing to keep blood from flowing backward.

    To work properly, all three leaflets must meet when they close. If the valve doesn't completely close, blood leaks back into the heart. This is called regurgitation. Aortic regurgitation develops when the aortic valve leaflets become retracted, prolapsed, or distorted in some other way. A retracted valve is drawn in and the leaflets don't meet when they close. To repair a retracted valve, a surgeon usually performs a valve extension.

    This means the surgeon takes a small amount of the strong tissue covering the heart, called the pericardium, and uses it to lengthen the leaflets. This does not affect the function of the pericardium. A prolapse valve has leaflets that droop. A surgeon will adjust or remove parts of the valve until all the leaflets meet.

    In some cases, a surgeon may replace entire leaflets with tissue from the pericardium. This is called the Ozaki Procedure. Another cause of valve damage is infection. Infection may cause a perforation or small hole in the valve leaflets. A surgeon will use tissue from the pericardium to patch the hole.

    Aortic valve repair is highly individualized. At the Valve Center at Children's Hospital of Philadelphia, our team uses cutting edge imaging to view each patient's heart before surgery. This allows us to develop a personalized surgical plan based on your child's unique anatomy. We work together and with you, to make the best decisions for your child before, during, and after surgery.

Transcript Transcript

Artificial valve surgery 

Another treatment option is replacing the valve with a mechanical (artificial) one. If this happens, your child will need to take blood-thinning medicine for life to help prevent blood clots.

  • Mechanical Valve Repair in Children and Teens

    Jonathan Chen, MD: A mechanical valve can be used to replace a damaged or diseased aortic or mitral valve. I'm Jonathan Chen, a surgeon in the Pediatric Heart Valve Center at Children's Hospital of Philadelphia. My team is one of the most experienced in the country in valve repair and replacement. To better understand mechanical valve replacement, let's first take a look at the anatomy of the heart. This is your heart. It has four chambers and four valves.

    The upper chambers are the left atrium and the right atrium. The lower chambers are the left ventricle and the right ventricle. Before entering or leaving each chamber of the heart, blood passes through a valve. The mitral valve is between the chambers on the left side of the heart. The left atrium receives oxygen-rich blood from the lungs and pumps it through the mitral valve to the left ventricle.

    The left ventricle then pumps the blood through the aortic valve and into the aorta, the largest artery in the body. Blood travels from the aorta to blood vessels throughout the body, delivering oxygen and nutrients. Heart valves are made of flaps of tissue called leaflets. The leaflets work like one way doors, opening to let blood move forward, and closing to keep blood from flowing backward. Watch how the leaflets move from another angle, opening to let blood move forward and closing to keep blood from flowing backward.

    The leaflets are attached to a strong tissue called the annulus. The annulus helps maintain the shape of the valve. If a valve is diseased or damaged, it can be surgically removed and replaced with a mechanical valve. A mechanical valve is made of metal and plastic. A thick cloth surrounds the valve. Just like your child's heart valves, the leaflets in the mechanical valve open and close to keep blood flowing through your heart. The mechanical leaflets make a soft clicking noise as they open and close. You can hear it, if you have a mechanical valve. Patients adjust to the sound and are eventually able to ignore it. You might have heard of adults who have bioprosthetic valves, made of tissue from pigs or cows.

    These valves have a high risk of becoming hard over time, in a process called calcification, causing these valves to last less than three to five years in children. During a mechanical valve replacement, your surgeon will remove the diseased or damaged valve and choose the right size replacement valve for your child.

    The surgeon will suture the mechanical valve to the annulus using the sewing ring or cloth rim of the valve. At the Valve Center at Children's Hospital of Philadelphia, our team uses cutting edge imaging to view each patient's heart before surgery. This allows us to develop a highly personalized surgical plan based on your child's unique anatomy.

    In babies and toddlers, a mechanical valve will need to be upsized as your child grows. While a teenager may only need one valve operation, a baby or toddler will likely need two or three re-operations over time. A mechanical valve has an increased risk of infection. It's important to tell your doctor if your child has a serious cut or lesion because they may need antibiotics to prevent infection from traveling to the heart.

    It is possible for scar tissue, called pannus, to grow around the mechanical valve. Too much scar tissue can make it hard for the leaflets to move, requiring a replacement valve. Patients with mechanical valves must take daily blood thinners to prevent clotting on the valve. If a blood clot detaches from the valve and travels to another area of the body, this can be life-threatening. Blood thinning medication needs careful management.

    Dosage must be monitored regularly, which can be done at home. When your child is facing heart valve surgery, we know there are difficult decisions to make. Our experts work together and with you, to make the best decisions for your child before, during, and after surgery.

Transcript Transcript

Ross procedure 

A third option is the Ross Procedure. In this surgery, the doctor replaces the aortic valve with the child’s own pulmonary valve, then replaces the pulmonary valve with one from a donor. This allows the new aortic valve to grow with the child and doesn’t require blood thinners.

  • Ross Procedure for Damaged Aortic Valve in Children and Teens

    Jonathan Chen, MD: The Ross procedure is a surgical technique that can be used to replace a damaged or diseased aortic valve. I'm Jonathan Chen, a surgeon in the Pediatric Heart Valve Center at Children's Hospital of Philadelphia.

    My team is one of the most experienced in the country in valve repair and replacement, and we regularly perform the Ross procedure. To better understand the procedure, let's first take a look at the anatomy of your heart. This is your heart. It has four chambers and four valves. Blood passes through a valve before leaving each chamber of the heart. The valves prevent the backward flow of blood.

    To understand the Ross procedure, you'll need to know about the aortic valve and the pulmonary valve. The heart's lower chambers are called ventricles. The left ventricle pumps oxygen-rich blood through the aortic valve and into the aorta, the largest artery in the body. Blood travels from the aorta to blood vessels throughout the body, delivering oxygen and nutrients. Blood that has traveled through your body and is now low in oxygen goes into the right side of the heart.

    The right ventricle pumps the blood through the pulmonary valve, into the pulmonary artery and to the lungs for more oxygen.

    If the aortic valve is diseased or damaged, it can be surgically removed and replaced with the body's own healthy pulmonary valve. This is known as the Ross procedure. Here's how it works. First, the surgeon must detach the coronary arteries from the aorta. The coronary arteries are the blood vessels that feed oxygenated blood to the heart.

    Then the surgeon will remove the diseased or damaged aortic valve, as well as the aorta surrounding the valve. The healthy pulmonary valve, surrounded by a cylinder of pulmonary artery, is then removed from its usual location and sewn into the aortic position. This is called an autograft. The surgeon then reattaches the coronary arteries to the autograft.

    A donor cadaver pulmonary valve surrounded by a cylinder of pulmonary artery is then attached replacing the original pulmonary valve. This is called a homograft.

    The Ross procedure is a highly technical surgery. At the Valve Center at Children's Hospital of Philadelphia, our team uses cutting edge imaging to view each patient's heart before surgery. This allows us to develop a highly personalized surgical plan based on your child's unique anatomy. As with any heart surgery, the Ross procedure has advantages and disadvantages.

    Because the Ross procedure uses the patient's own tissue, there's little risk of clotting. This means your child won't need to take long-term blood thinners. The pulmonary valve in the aortic position, remember this is called the autograft, continues to grow with your child, so it won't need to be upsized as your child grows. Sometimes, however, the autograft has to be replaced because of wear. The donor pulmonary valve, remember this is called the homograft, does not grow.

    In kids, it will eventually need to be upsized, which will require a future operation. In teenagers and adults, the homograft won't need to be upsized, but the valve inside the homograft might become leaky. If that happens, the valve can probably be replaced through a catheterization instead of surgery. Our team is a world leader in the Ross procedure.

    We work together and with you, to make the best decisions for your child before, during, and after surgery.

Transcript Transcript

Outlook for bicuspid aortic valve

Thanks to huge advances in medicine and technology, most children with a bicuspid aortic valve grow up to live healthy, active lives as adults.

Follow-up care for aortic valve

Through age 18

Children with a bicuspid aortic valve require regular checkups with a pediatric cardiologist. Some children have to stay on medication and limit their physical activity.

Into adulthood

Bicuspid aortic valve can cause health problems in adults. It is very important that adults with this condition are monitored by a cardiologist regularly for the rest of their lives. 

Our pediatric cardiologists follow patients until they are young adults. The Philadelphia Adult Congenital Heart Center, a joint program of CHOP and Penn Medicine, meets the unique needs of adults who were born with heart defects. At CHOP’s Cardiac Center, we support our adolescent and young adult patients through a smooth and seamless transition to adult cardiology care at the Philadelphia Adult Congenital Heart Center or, if desired, to a cardiologist with congenital heart disease expertise in another location.

Patient Outcomes at the Cardiac Center

Children’s Hospital of Philadelphia's pediatric heart surgery survival rates are among the best in the nation.

Resources to help

Cardiac Center Resources

We know that caring for a child with a heart condition can be stressful. To help you find answers to your questions – either before or after visiting the Cardiac Center – we’ve created this list of educational health resources.

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