Topics
What is aortic stenosis?
Aortic stenosis is a congenital heart defect in which the aortic valve did not form properly, reducing or obstructing blood flow out of the heart.
The aortic valve is the heart valve located between the left ventricle (lower left-sided pumping chamber of the heart) and the aorta, the main artery supplying oxygen-rich blood to the body.
The aortic valve opens with each heartbeat to allow blood to flow out of the heart and into the aorta, then closes to prevent blood from leaking back from the aorta into the left ventricle. The normal valve consists of three leaflets (flaps) of nearly equal size. A normal aortic valve has 3 parts (leaflets) that act as a one-way door. In aortic stenosis, the valve doesn’t work as it should. That makes it harder for the leaflets to open and let blood flow from the left ventricle to the aorta.
Aortic stenosis can range from mild to severe. It may also occur with other heart problems or conditions. It depends on how much of the blood is blocked. It can worsen over time, in some cases progressing to a severe form of heart disease called hypoplastic left heart syndrome (HLHS). HLHS is a life-threatening condition where the left side of the baby’s heart is too small or dysfunctional to use at birth. It can be associated with other defects including coarctation of the aorta.
Moderate to severe aortic stenosis may affect the heart and blood vessels in these ways:
- Over time the left ventricle becomes larger and can’t pump blood to the body very well.
- The aorta may also become larger.
- The coronary arteries that send blood to the heart muscle may not get enough blood.
Aortic stenosis means that your child has a heart valve that is too narrow or is blocked. The aortic valve is 1 of 4 heart valves that keep blood flowing through the heart. The valves make sure blood flows in only one direction. The aortic valve keeps blood flowing from the left ventricle to the aorta. Your child may be born with aortic stenosis (congenital). Or it may develop later (acquired). It occurs more often in boys than in girls.
In babies with aortic stenosis the aortic valve may be smaller and thicker than normal. The leaflets may be fused together, so instead of three leaflets, there may be only two leaflets (bicuspid aortic valve). The most severe cases of aortic stenosis may even have a single leaflet (unicuspid valve). When the leaflets are fused, they aren’t able to open normally. This results in varying degrees of blockage (stenosis) of blood flow across the aortic valve. In addition, the abnormal leaflets may not close normally, resulting in varying degrees of leakage (regurgitation).
What causes aortic stenosis?
A child can be born with aortic stenosis. This means the aortic valve didn’t form as it should before birth. Sometimes this problem is caused by gene problems. But most of the time, the cause for this isn’t known. In older children, aortic stenosis may occur after an untreated strep infection.
How does aortic stenosis affect my baby?
The effects of aortic stenosis depend on the severity. The more severe the condition, the greater the consequences. In cases where the valve opening is significantly narrowed or obstructed, the fetal heart has to work harder to pump enough blood out to the body. This places extra stress on the left ventricle. In utero, in severe cases – known as critical aortic stenosis – the left ventricle can weaken, stop growing and if left untreated, may progress to HLHS.
Babies with significant aortic stenosis may suffer from heart failure if the blockage is severe and/or if the left ventricle is unable to squeeze (pump) normally.
Babies born with less severe forms of aortic stenosis may have a heart murmur but no other symptoms. These children may not be diagnosed until later in life.
What are the symptoms of aortic stenosis?
When the stenosis is severe or critical, infants can be quite ill with signs and symptoms of heart failure, including breathing difficulty, poor feeding, fatigue, irritability, and cool, pale extremities. These signs and symptoms are evident early in the newborn period.
The symptoms of aortic stenosis vary, depending on how old your child is. They also vary by how severe the blockage is. For example, a child with mild aortic stenosis may have few symptoms. Or he or she may not have any symptoms. Symptoms may not show up until adulthood. Or an infant may have trouble feeding and may not gain weight. In severe (critical) aortic stenosis, infants are very ill.
More severe aortic stenosis may cause:
- Fatigue or tiredness
- Dizziness or lightheadedness, especially with physical activity
- Fainting (syncope)
- Shortness of breath
- Irregular heartbeats or feeling the heart beat (palpitations)
- Chest pain or pressure
The symptoms of aortic stenosis can be like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.
How is aortic stenosis diagnosed?
Your child's healthcare provider may have heard a heart murmur when listening to your child’s chest with a stethoscope. A heart murmur is an abnormal sound as blood moves through the heart. A heart murmur may mean that your child has a heart defect. Your child’s symptoms are also part of figuring out the diagnosis.
Aortic stenosis may be diagnosed during a routine prenatal ultrasound, typically at 18 to 20 weeks. Additional testing, including fetal echocardiography, is typically needed to confirm the diagnosis and learn more about the defect.
Your child may need to see a pediatric cardiologist to confirm the diagnosis. This is a doctor with special training to treat heart defects and other heart problems in children. Your child may also have tests, such as:
- Chest X-ray. This gives an overall picture of your child's heart and lungs.
- Electrocardiogram (ECG). This test that measures the electrical activity of the heart.
- Echocardiogram (Echo). This test uses sound waves to give a moving picture of the heart and valves. This is one of the best tests for aortic stenosis.
Cardiac catheterization. This test shows details of the structures of the heart. Your child will have this test while asleep. The doctor will put a thin, flexible tube (catheter) into one of your child’s blood vessels. The doctor will slowly guide the catheter to the heart. Contrast dye may be injected to let the cardiologist to see more detail.
How is aortic stenosis treated?
Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. If your child doesn’t have symptoms or if symptoms are mild, his or her healthcare provider may just watch symptoms closely. This means your child may often have office visits and tests.
The pediatric cardiologist and a cardiothoracic surgeon will figure out whether your child needs an aortic valve procedure. The procedures include:
- Balloon aortic valvuloplasty. This is done with cardiac catheterization using a catheter with a deflated balloon in the tip. The catheter is put into a blood vessel. It is moved to the narrowed valve, and the balloon is inflated to open the valve. Many providers prefer this procedure.
- Surgical aortic valvotomy. This is surgery to remove scar tissue from the aortic valve leaflets. This lets the leaflets to open as they should.
- Aortic valve replacement. This is surgery to replace the aortic valve with a new valve. Replacement valves are either artificial or from donor organs or animals.
- Pulmonary autograft (Ross procedure). This is surgery to replace the aortic valve and part of the aorta. Your child's own pulmonary valve and part of the pulmonary artery are used to replace the damaged aortic valve. A pulmonary valve and part of the pulmonary artery from a donor organ are used to replace the transplanted valve and artery. Many surgeons prefer this method because it continues to work well as a child grows.
Before the procedures you might expect the following:
- An infant who has critical aortic stenosis will be in an intensive care unit (ICU). He or she may need emergency repair of the valve. Infants who aren’t as sick have the procedure planned.
- A child with severe aortic stenosis may not be able to take part in sports. This is especially true for sports that have intense or long periods of activity.
Aortic Stenosis Treatment Before Birth
Pregnancies involving fetal aortic stenosis require close monitoring for early detection of signs the condition is worsening, including fetal non-immune hydrops (fluid accumulation in multiple areas of the baby’s body) and fetal heart failure. A fetus with aortic stenosis may have a normal sized left heart early in pregnancy that progresses to critical aortic stenosis and left heart abnormalities by mid-pregnancy.
In most cases, a fetus with aortic stenosis won’t require treatment before birth.
If the condition is expected to evolve in utero into HLHS, in select cases a fetal procedure may be considered called a balloon dilation of the aortic valve (also known as a fetal aortic valvuloplasty).
During this ultrasound-guided procedure, the surgeon inserts a special needle through the mother’s abdomen and uterus, and into the baby’s heart. A tiny balloon is then inserted across the aortic valve and gently inflated to widen a severely narrowed or blocked opening. The goal is to restore blood flow, improving the function and growth of the left ventricle and the baby’s prognosis at birth.
Following the procedure, mother and baby will be closely monitored for the remainder of the pregnancy with frequent ultrasounds and fetal echocardiograms. The baby will be delivered as close to term as possible, giving the fetal lungs, brain and other vital organs a chance to mature so the newborn is more stable before undergoing a procedure or heart surgery after birth.
Texas Children’s Fetal Center is one of the few centers in the world offering this procedure, providing new hope and more options for families facing fetal aortic stenosis. In collaboration with Texas Children’s Heart Center, our Fetal Center has successfully performed fetal balloon dilation of aortic valve since 2012.
Aortic Stenosis Treatment During Delivery
For the best possible outcomes, delivery should take place at a center with the highest quality congenital heart program, ensuring the expertise and resources required to treat aortic stenosis at birth, including an advanced neonatal (NICU) and cardiac intensive care unit (CICU).
Delivery and postnatal care should be carefully planned and coordinated. Our Fetal Center team works closely with Texas Children’s Heart Center every step of the way, seamlessly transitioning your baby’s care at delivery to this national leader in pediatric cardiology and heart surgery. Here, the heart specialists treating your newborn have been an integral part of their care team since before birth.
Aortic Stenosis Treatment After Birth
Treatment is individualized for each baby based on the severity of the disease and any associated defects. Mild cases usually require only observation. These children should have regular checkups with a cardiologist throughout their lives.
More severe cases may require intervention to reduce the degree of blockage and improve delivery of blood flow out to the body. A procedure called a cardiac catheterization may be done, where a thin, flexible tube (catheter) with a deflated balloon on the tip is put into a blood vessel, guided into the heart and placed across the valve. The balloon is then inflated to open the valve. Surgical repair or replacement are alternate possibilities.
If aortic stenosis has progressed in utero to hypoplastic left heart syndrome at birth, treatment may involve a series of heart surgeries within the first few years of life to reroute and improve blood flow to the lungs and body while reducing the workload on the left ventricle, enabling the heart to function with one ventricle (also known as single ventricle palliation or pathway).
What are the complications of aortic stenosis?
Complications of moderate to severe aortic stenosis include:
- Bulging or weakening (aneurysm) of the aorta
- Tear (dissection) of the aorta
- Infection of the lining of the heart, valves, or blood vessels (infective endocarditis)
- Heart is not able to pump as it should (heart failure)
- Death
Talk with your child’s healthcare provider about his or her risk for these problems.
Can aortic stenosis be prevented?
Congenital aortic stenosis can’t be prevented. But all newborns are screened for congenital heart disease with pulse oximetry. This is a simple, painless test to check the amount of oxygen in the blood. It’s done by placing a small probe on the infant’s arm and leg. If the oxygen level is low, it may mean there is a heart defect. More testing and treatment will be done if a problem is found.
What is living with aortic stenosis like after a procedure?
Most children who have had an aortic valve repair or replacement live active, healthy lives. Your child’s activity levels, appetite, and growth usually return to normal. Your child should get regular follow-up care with a cardiologist throughout his or her life.
Your child may also need:
- Regular blood pressure checks and management of high blood pressure
- Regular dental care. This is to prevent infections that may lead to a heart infection (endocarditis).
- Limited physical activity. Talk with your child’s healthcare provider about safe activities for your child.
- Antibiotics before procedures. This includes dental work. This depends on whether your child has had a valve repair or replacement.
- Blood thinners (anticoagulants). These prevent blood clots from forming on a mechanical valve. Blood tests to check the blood thinners are also done.
- Possible repeat valve repair or replacement
Talk with your child's provider about what to expect with your child.
When should I call my child’s healthcare provider?
Contact your child’s healthcare provider if you notice:
- Symptoms such as chest pain or trouble breathing that get worse
- Dizziness or tiredness with physical activity
If you child has had a procedure, make sure to follow all instructions from the surgeon. And make sure to keep all follow-up appointments with your child’s cardiologist and surgeon.
Key points About Aortic Stenosis
- Aortic stenosis means that a valve in your child’s heart is too narrow or is blocked. This condition can be mild, moderate, or severe.
- Common symptoms are tiredness, dizziness, shortness of breath, chest pain or discomfort, and a fast heart beat.
- A pediatric cardiologist usually diagnoses and manages aortic stenosis.
- Several procedures are available to repair or replace the aortic valve.
A child with aortic stenosis should be checked often. Follow-up care is needed throughout his or her life.
Why Texas Children’s Fetal Center?
- A single location for expert maternal, fetal and pediatric care. Texas Children’s Hospital offers mothers and babies the specialized care required for the diagnosis and treatment of congenital heart conditions all in one location, for highly coordinated care and treatment planning.
- A skilled, experienced team with proven outcomes. We have a dedicated team of maternal-fetal medicine specialists, fetal imaging experts, fetal and pediatric cardiologists, congenital heart surgeons, neonatologists and others who work in concert to care for you and your baby every step of the way, using proven protocols we’ve developed over the years. With their combined expertise and unified approach, this team offers the best possible care for pregnancies involving fetal aortic stenosis.
- We care for your child’s cardiac needs at every stage of life, from fetus to adulthood. Our comprehensive approach starts with your first prenatal visit and continues through delivery, postnatal care, childhood and adulthood, thanks to one of the nation’s leading teams of fetal, pediatric and adult congenital heart specialists.
Texas Children’s – #1 in the Nation for Pediatric Cardiology and Heart Surgery
Our Fetal Cardiology Program is a collaboration between Texas Children’s Fetal Center and Texas Children’s Heart Center, ranked #1 in the nation for pediatric cardiology and heart surgery by U.S. News & World Report for the third consecutive year, with outcomes among the best in the country.