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Aortic Stenosis

Aortic Stenosis

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What is aortic stenosis?

When the heart squeezes, the left ventricle (the lower left chamber) contracts, pushing blood into the aorta, the main artery that takes blood to the body. The aortic valve is located between the left ventricle and the aorta and prevents blood from leaking back into the heart between beats.

A normal aortic valve is made up of three thin leaflets. In the congenital heart disease aortic stenosis these leaflets are fused or too thick. As a result, the aortic valve is too narrow and causes obstruction to blood flowing out of the heart. To compensate, the heart has to work harder to pump enough blood to the body. Aortic stenosis can be trivial, mild, moderate, severe or critical.

Sometimes the stenosis (narrowing) is below the valve in the left ventricle, caused by a fibrous membrane or a muscular ridge. This is called subaortic stenosis. Also, the stenosis can occur above the valve, in the aorta itself; this is called supravalvular aortic stenosis.

Signs and symptoms of aortic stenosis

In young infants, severe or critical aortic stenosis can cause decreased blood flow, which causes symptoms such as a lack of energy (lethargy), poor feeding and respiratory distress.

Milder forms of aortic stenosis usually won’t cause symptoms in infants or small children. As the child gets older, aortic stenosis symptoms may appear, including fatigue, a heart murmur (an extra heart sound when a doctor listens with a stethoscope), or, rarely, chest pain, fainting or arrhythmias (abnormal heart rhythm).

Testing and diagnosis for aortic stenosis

In rare cases, newborns have critical aortic stenosis, which requires immediate medical attention. Sometimes these severe cases are diagnosed before birth, through the Fetal Heart Program at Children’s Hospital of Philadelphia (CHOP).

In most cases, we diagnose aortic stenosis after a primary care doctor detects a heart murmur and refers a child to us. Diagnosis may require some or all of these tests:

  • Pulse oximetry, which monitors the oxygen content of the blood with a small finger attachment
  • Chest X-ray, which can help identify problems with the heart and lungs
  • Echocardiogram (also called "echo" or cardiac ultrasound), which uses ultrasound waves to create an image of the heart; it can show the size, shape and movement of the heart's valves and chambers, as well as the flow of blood through the heart
  • Electrocardiogram (ECG), a record of the electrical activity or rhythm of the heart
  • Cardiac MRI, a three-dimensional image showing the heart's abnormalities
  • Cardiac catheterization, in which a thin tube (called a catheter) is inserted into the heart through a vein or artery in the leg or belly button

Aortic stenosis can run in families, so be sure to tell your cardiologist if there is a history of a heart murmur in other close family members.

Aortic stenosis treatment

  • 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

Treatment for aortic stenosis depends on a child's unique heart anatomy. Trivial and mild aortic stenosis typically do not require treatment. Moderate, severe and critical aortic stenosis, however, do require treatment, which may include one or more of the following options:

Cardiac catheterization

In most cases, aortic stenosis can be treated with a balloon valvuloplasty, which requires cardiac catheterization. An interventional cardiologist will insert a thin tube (catheter) into an artery in the leg, then guide the tube through the body, across the aortic valve and into your child’s heart.

The catheter will have a balloon on the end of it. The balloon will be briefly inflated to open up the narrow valve, then deflated and withdrawn. Sometimes, two catheters and balloons are used. In newborns, the blood vessels in the umbilical cord are sometimes used as the site where the catheters are inserted and advanced toward the heart.

Older children will typically spend one night in the hospital after this procedure, in CHOP’s dedicated post-catheterization recovery unit, before returning home. They will also need to rest for the next few days, but then can resume normal activity. Newborns with critical conditions or children who are already inpatients at CHOP may stay in the Hospital slightly longer, either in the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU), where they will receive around-the-clock care from a team of dedicated cardiac critical care medicine specialists, or in the Cardiac Care Unit (CCU).

Valvuloplasty

Repairing or replacing a faulty or blocked heart valve may be necessary in severe cases. Depending on your child’s age, needs and valve anatomy, physicians may attempt to repair the valve or improve its function with a procedure called a 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 valves

Another option for aortic stenosis treatment includes the use of mechanical (artificial) valves as replacement valves. If this is the case, your child may need to stay on blood-thinning medicines for the rest of their life.

  • 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

Surgery to replace a heart valve may be necessary in severe cases. Surgeons may decide a Ross Procedure – also known as a switch procedure -- is the best option to treat aortic stenosis. In this operation, the aortic valve is replaced with the patient's pulmonary valve. The pulmonary valve is then replaced with a valve from a donated organ. This procedure allows the patient's pulmonary valve (now in the aortic position) to grow with the child.

  • 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

Subaortic and supravalvular stenosis treatment

Subaortic and supravalvular stenosis do not improve with balloon dilation and will require heart surgery if the amount of obstruction is moderate or severe, or, in the case of subaortic stenosis, the aortic valve begins to leak significantly. 

Outlook for aortic stenosis

Because of enormous strides in medicine and technology in recent years, most children with heart conditions today go on to lead healthy, productive lives as adults. All patients with aortic valve disease will need some form of lifelong follow-up care with a cardiologist.

Follow-up care

Through 18 years

Children with aortic valve stenosis require regular checkups with a pediatric cardiologist. Some children must remain on medicine and limit physical activity.

As a child with aortic stenosis grows, blood may begin to leak through the abnormal valve. This is called aortic regurgitation or aortic insufficiency. In other children, the stenosis can reoccur. When this happens, balloon valvuloplasty can be repeated, as long as there isn't significant aortic regurgitation. In severe cases, additional surgery may be necessary.

At Children’s Hospital of Philadelphia (CHOP), our pediatric cardiologists follow patients until they are young adults and coordinate care with primary care physicians.

Into adulthood

Aortic stenosis can continue to cause health problems in adults. It is very important that adults with aortic stenosis are monitored by a cardiologist regularly for the rest for their lives. CHOP’s Cardiac Center can help with the transition to an adult cardiologist.

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.

Why Choose Us

Our specialists are leading the way in the diagnosis, treatment and research of congenital and acquired heart conditions.

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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