Coarctation of the aorta is a congenital heart defect in which your baby’s aorta (the largest artery in their body) is pinched in or narrowed in one spot. This restricts normal blood flow to their lower body. Surgery or catheterization can repair the problem and relieve symptoms. Long-term follow-ups are essential even after repair.
Coarctation of the aorta is a congenital (present at birth) heart defect. It’s also called aortic coarctation (pronounced “ay-or-tuhk” “koh-ark-TEY-shun”). This defect affects your baby’s aorta, which is the largest artery in their body. It carries oxygen-rich blood from your baby’s heart to the rest of their body.
If your baby has aortic coarctation, one part of their aorta is narrower than it should be. Picture a long balloon that’s used to make balloon animals for kids. You twist the balloon at one point to begin forming a shape. This causes the balloon to be pinched inward at that point. The pinch in the middle of the balloon is similar to what an aortic coarctation looks like.
That pinched point might be very narrow and cause severe symptoms soon after birth. Or it might be narrower than normal but wide enough to let blood pass through. In that case, symptoms might not appear until later in childhood or adolescence. Symptoms such as hypertension (high blood pressure) may lead to detection of aortic coarctation.
Babies who have coarctation of the aorta may also have other heart problems, including:
Aortic coarctation needs treatment to prevent serious complications. Once a diagnosis of aortic coarctation is made, either surgical repair or balloon angioplasty should be performed.
If your baby has aortic coarctation, that means one part of the aorta is narrower than normal. Usually, this narrowing occurs in the descending aorta.
About 4 in 10,000 babies in the U.S. have coarctation of the aorta. Each year, about 2,200 babies are born with the condition.
Of all babies born with some form of congenital heart disease, about 7 in 100 have aortic coarctation.
Coarctation of the aorta prevents blood from flowing normally throughout your baby’s body. The narrowing causes the main pumping chamber of your baby’s heart (left ventricle) to pump harder than normal. This puts a lot of stress and strain on their left ventricle. In cases where the narrowing is severe, not enough blood will get to their body and their brain, which can cause shock and even death. In milder cases, this extra strain can cause your baby’s heart muscle to get thicker (hypertrophy).
Coarctation also causes higher blood pressure in your baby’s upper body, and lower blood pressure in their lower body. Your baby may have reduced blood flow to their kidneys, liver and other organs in their belly.
The symptoms of aortic coarctation depend on how narrow the aorta has become. Some infants have no symptoms because the coarctation (narrowing) is very mild. But symptoms can appear within the first two weeks of birth if the coarctation is moderate or severe and the ductus arteriosus (a special fetal blood vessel) closes.
Symptoms in infants include:
Severe coarctation in infants can lead to shock and even death if not recognized and treated promptly.
Most children don’t have any symptoms. They’re usually diagnosed after their provider notices they have high blood pressure.
Some children may have symptoms if their blood pressure is too high in their upper body or too low in their lower body. These include:
Symptoms in adults are usually due to recoarctation of the aorta. This is aortic narrowing that returns sometime after a previous repair. Symptoms may include:
But these symptoms are linked with many other health issues in adults. So, having these symptoms doesn’t mean you have aortic coarctation. Call a healthcare provider to discuss any symptoms you have. They’ll give you a physical exam and run tests to diagnose what’s wrong.
The exact cause of coarctation of the aorta isn’t fully known. However, congenital heart defects are often caused by genetic changes that happen before birth. Babies with Turner syndrome have a higher risk of aortic coarctation and other defects that affect the left side of their heart.
Sometimes, environmental factors play a role in causing heart defects, too. These are factors related to the birthing parent. They include:
The ductus arteriosus may cause coarctation of the aorta. The ductus arteriosus is a small artery that connects the fetal aorta and pulmonary artery. It helps your fetus get enough oxygen-rich blood during gestation when their lungs aren’t working yet.
Once your baby is born, their lungs start working. That means they don’t need the ductus arteriosus anymore. So, it usually closes up within a few days of birth. But when it closes, something may go wrong. Some tissue from the ductus arteriosus may blend in with tissue from their aorta. When this tissue tightens to close up the ductus arteriosus, it may narrow their aorta as well and lead to coarctation.
Coarctation of the aorta usually occurs in your baby’s descending aorta near their ductus arteriosus. But it can also happen in other parts of their aorta. These include other areas of the aortic arch or further down in your baby’s chest or belly.
Your baby’s aorta is a long, curved blood vessel that arches upward from the top of their heart and then curves down through their chest and belly. It’s shaped like a walking cane with a round handle. The “handle” curves upward (ascending aorta) and then downward (descending aorta). The top of that curve is called the aortic arch.
The location of aortic coarctation matters because it affects your baby’s symptoms. You can think of your baby’s aorta like a main road that has lots of intersections. At each intersection, another artery or group of arteries connects with the aorta and carries blood in a different direction.
Aortic coarctation often occurs between two major intersections. The first is where arteries branch off to the upper body. The second is where arteries branch off to the lower body. So, blood leaves your baby’s heart and travels along just fine. It reaches the first intersection and easily flows into the arteries that lead to your baby’s upper body. Meanwhile, some blood keeps moving along in their aorta. And soon it runs into trouble.
It’s like when construction forces a five-lane highway down to one lane. Only so many cars can pass through. It's the same with your baby’s aorta. That pinched, narrowed part can only let a little blood pass through. So, it’s harder for blood to reach the second intersection, where arteries carry blood to your baby’s lower body. The coarctation slows down blood flow in those arteries.
This is why the blood pressure in your baby’s arms and upper body will be high. But the blood pressure in their lower body and legs will be low. This blood pressure difference is a red flag that leads to an aortic coarctation diagnosis.
Coarctation of the aorta is usually diagnosed during infancy or early childhood. The timing depends on the severity of the symptoms. Infants with moderate or severe symptoms are typically diagnosed soon after birth. Infants with mild or no symptoms may not be diagnosed until later in childhood when they begin to have high blood pressure. Aortic coarctation is rarely diagnosed in adulthood.
Some newborns are diagnosed before they show visible symptoms. This happens when a pulse oximetry test shows low levels of oxygen in their blood. Low oxygen can be a sign of a serious heart defect like coarctation of the aorta. So, babies with low oxygen would then receive more tests to identify the specific problem.
Most babies and children are diagnosed with coarctation of the aorta when a physical exam reveals certain red flags. Signs that your baby or child may have coarctation of the aorta include:
When a physical exam raises red flags, your baby or child needs more tests to confirm the aortic coarctation diagnosis. These tests include:
Surgery is the gold standard for repairing coarctation of the aorta. But there are other treatment options, too. The best method for repairing aortic coarctation depends on many factors, including:
Babies and children diagnosed with coarctation of the aorta need the care of a congenital heart specialist. You’ll find such specialists at high-volume hospitals that treat congenital heart disease every day. If your baby was diagnosed with aortic coarctation, they should be evaluated by a cardiac surgeon at a major hospital. The surgeon will identify the best form of treatment and the best timing.
Babies with severe symptoms right after birth may need medication before having surgery. A medication called prostaglandin (PGE-1) is used to keep your baby’s ductus arteriosus open. This allows your baby to get enough oxygen and become stable enough for surgery. Some babies may need medication to help their heart pump.
Surgeries to repair aortic coarctation can be divided into two major categories: those that can be done through an incision in your baby’s side (thoracotomy), and those that must be done through an incision in the front (sternotomy). The types of repairs done from a thoracotomy approach include:
Cardiac catheterization is a good option for older children who have mild coarctation. It’s also used for children and adults who have recoarctation (when the aorta narrows again sometime after repair). It’s less invasive than surgery. Options include:
Complications of aortic coarctation repair include:
Your child’s surgeon will explain the risks and complications in greater detail. It’s important to understand the possible complications. But it’s also important to know that aortic coarctation is essential and life-saving for your child. Without repair, aortic coarctation is fatal.
Coarctation of the aorta can’t be cured. Surgeries and interventions repair the problem, but there’s always a risk of recurrent coarctation. Even after treatment, people with aortic coarctation face a higher lifelong risk of cardiovascular problems.
Your child will need lifelong follow-ups with a congenital heart disease specialist. They’ll offer advice and keep an eye out for any possible issues.
There’s no specific way to prevent aortic coarctation. But prenatal care may impact your baby’s chances of having congenital heart disease. If you’re pregnant or planning a pregnancy, it’s important to:
Talk with your provider about any concerns you have. Genetic testing may be helpful if you or other biological family members have congenital heart disease.
Thanks to improvements in diagnosis and repair, people with coarctation of the aorta can live to at least age 60. In the past, the average life expectancy for people with coarctation of the aorta was just 35 years old.
Your child may or may not have restrictions on activities. If your child had aortic coarctation repair and there aren’t any persistent blood pressure problems, they may have no limitations. Check with your child’s provider to learn more about their specific situation and needs.
For six months following aortic coarctation repair, your child will need to take antibiotics before visiting the dentist or having some surgeries. This is to prevent an infection of the heart called endocarditis.
Coarctation of the aorta can put adults at a higher risk of cardiovascular problems. Untreated coarctation of the aorta is most dangerous. About 9 out of 10 adults with untreated aortic coarctation don’t survive to age 50.
But nowadays, it’s rare for someone to be diagnosed as an adult. Most adults with aortic coarctation had repair surgery or catheterization as a child. And they go on to live healthy and happy lives.
Still, adults with a history of aortic coarctation face a higher risk of:
If you had a repair procedure for aortic coarctation, you probably see your cardiologist regularly for check-ups. These are essential for making sure your heart is functioning well. You probably also think about your heart health more often than your friends who didn’t have surgery or catheterization when they were younger.
Life gets busy, though. And you may not always have the time or energy to cook heart-healthy meals or get in enough exercise. Plus, if you feel healthy overall, it’s easy to forget about what’s going on inside of your body that you can’t see. You shouldn’t let worry consume you, but it’s important to do whatever you can to reduce your risk for future problems.
Here are some steps you can take to keep your heart health a priority:
Thanks to medical advances, more babies with congenital heart defects survive into adulthood. That means an increasing number of adults are living with congenital heart disease. It also means there’s a growing amount of resources and support available to you. Talk with your healthcare team about how to connect with resources and meet other adults who are in your shoes.
A note from Cleveland Clinic
Each year, 1 in 2,500 babies in the U.S. are born with aortic coarctation. If your baby is that “1,” you might be wondering why this happened. Researchers can’t always know the exact cause of a congenital heart defect. What is known is that advances in medical care allow babies born with heart disease to live longer than ever before.
Early diagnosis and treatment are critical for managing aortic coarctation. Talk with your child’s provider about next steps for care and treatment.
Last reviewed by a Cleveland Clinic medical professional on 08/28/2022.
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