Coarctation of the Aorta

Coarctation of the aorta (CoA, aortic coarctation) is a congenital heart defect that affects your baby’s aorta. This is the artery that sends blood from their heart to the rest of their body.

“Coarctation” is the medical term for narrowing. With CoA, your baby’s aorta is too narrow in one spot — typically, where the aorta turns downward after giving out branches to the upper body. This reduces blood flow to your baby’s lower body and raises blood pressure in their upper body.

How CoA affects your baby depends on the extent of the narrowing. If it’s mild, it may not cause issues right away. But your child may develop high blood pressure, leg pain with exercise or other symptoms as they get older. Severe narrowing can cause serious issues, like heart failure, shock or even death. That’s why healthcare providers classify severe CoA as a type of critical congenital heart disease (CCHD). 

Most babies and children with aortic coarctation need surgery to repair the narrowed part of their aorta. The timing depends on the severity of the condition and whether your child has any other heart defects. Your child’s care team can tell you more about what to expect.

Symptoms of aortic coarctation

CoA symptoms depend on how narrow the aorta is. Some babies and children may not look or feel any different. The first clue may be high blood pressure at a routine well-check.

Signs and symptoms in infants include:

• Excessive sleepiness
• Fast pulse and high blood pressure in their arms
• Weak pulse and low blood pressure in their legs
• Heavy sweating
• Irritability
• Fast or labored breathing
• Trouble with feeding
• Skin that looks pale or a bluish gray

Signs and symptoms in children and adults include:

• Headaches
• Nosebleeds
• Pain during exercise
• High blood pressure

In adults, if symptoms recur after a repair, that usually means that the aortic narrowing has returned. This is called aortic recoarctation. It’s rare (but possible) to get a CoA diagnosis as an adult.

Coarctation of the aorta causes

Experts don’t fully understand what causes CoA. But they believe:

• It’s genetic. Certain gene changes that happen before birth affect how the aorta develops. These changes can be passed down within families. If you have CoA, you’re much more likely to have a baby with CoA or another congenital heart condition compared with those who don’t have CoA.
• Certain genetic disorders can raise your risk of CoA. For example, babies born with Turner syndrome have a higher risk of aortic coarctation, bicuspid aortic valve and other congenital conditions.
• Abnormal ductus arteriosus closure may play a role. The ductus arteriosus is an artery that links the fetal aorta and pulmonary artery. It normally closes after birth. But as it does, some of its tissue may blend with aortic tissue, narrowing the aorta and leading to CoA.

Complications of this condition

Severe CoA in infants can cause shock or even death without prompt treatment. This is because there’s not enough blood going to the infant’s lower body, including their kidneys, liver and gut. Emergency medical attention is needed.

Without treatment, milder forms of aortic coarctation can still lead to serious problems over time, including:

• Persistent high blood pressure, with complications like premature coronary artery disease, strokes, abnormal bulging (aneurysm) in the aorta or a tear (dissection) in the aorta
• Thicker than normal heart muscle (left ventricular hypertrophy)
• A heart that’s unable to pump out enough blood (heart failure)

How doctors diagnose this condition

Infants with moderate or severe symptoms typically get a diagnosis soon after birth. Those with no symptoms or mild ones may not receive a diagnosis until later in childhood when they start to have high blood pressure. Rarely, adults receive a diagnosis.

Most babies and children receive a CoA diagnosis when a physical exam finds red flags, like:

• High blood pressure in their arms and upper body, but low blood pressure in their legs and lower body
• Differences in pulse when measured at their neck versus their groin
• A distinctive, harsh heart murmur that their provider hears when placing a stethoscope on their back

Some newborns get a diagnosis before they show visible symptoms. This happens when a pulse oximetry test shows low levels of oxygen in their blood — a sign of many possible congenital heart conditions. These babies then undergo additional tests to identify the specific problem.

Providers typically use an echocardiogram (echo) to diagnose CoA. They may also do noninvasive imaging tests, like CT or MRI scans, to learn more about the anatomy of your child’s aorta.

Associated conditions

Babies with coarctation of the aorta may also have other heart conditions, including:

• An aortic valve that only has two flaps instead of three (bicuspid aortic valve)
• A hole in the heart (atrial septal defect or ventricular septal defect)
• A fetal blood vessel that stays open longer than expected (patent ductus arteriosus)
• An underdeveloped aortic arch (aortic arch hypoplasia) or left side of the heart (hypoplastic left heart syndrome)
• Narrowing of a heart valve (aortic valve stenosis or mitral valve stenosis)

Cardiac surgeons tailor treatment to your child’s unique heart anatomy. This means treatment might be more complex for a baby who has multiple congenital heart disabilities compared to a baby who only has CoA.

How is coarctation of the aorta treated?

Treatment depends on your child’s age, the severity of aortic narrowing and any other heart conditions they have. Their provider may recommend:

• Surgery: This is the gold standard for repairing CoA in babies and young children. A surgeon removes the narrowed part of your baby’s aorta and rejoins the two ends. The surgical technique depends on whether your baby’s aortic arch is also narrowed and whether your baby has other heart conditions that need surgical correction at the same time.
• Cardiac catheterization: This is less invasive than surgery. It may be appropriate for older children with mild coarctation. It’s also a treatment for children and adults who have recoarctation. A cardiologist uses a balloon to widen the narrowed part of the aorta. They may also place a stent to keep it open.

Babies with severe symptoms right after birth may need medication before having surgery. For example:

• A medication called prostaglandin (PGE-1) can keep your baby’s ductus arteriosus open. This allows your baby to get enough blood supply to the lower part of their body while waiting for surgery.
• Your baby may need medication to help their heart pump.

When should my child see a healthcare provider?

Contact a provider if your child has any symptoms of CoA. These can point to many possible issues, not just coarctation of the aorta. But their provider will find the cause and make sure your child gets any needed treatment.

If your child received CoA treatment, their care team will tell you when to come in for follow-up visits.

What can I expect if my child has aortic coarctation?

Your child will need treatment either soon after birth or further down the road. Around the time they turn 18, they’ll transition to seeing an adult congenital heart disease specialist. This doctor will help manage their care throughout life. For example, they’ll:

• Closely monitor your child’s blood pressure
• Do routine imaging tests to check their heart function
• Offer advice on heart-healthy eating and physical activity
• Prescribe and adjust medications as needed

High blood pressure is common among adults with a history of aortic coarctation. CoA raises a person’s risk of developing coronary artery disease or having a heart attack at younger ages than others. It also raises their risk of stroke.

That’s why lifelong follow-ups with a heart doctor are so important. They’ll do everything possible to protect your child’s heart and help them live a long, healthy life.

Life expectancy

Thanks to improvements in diagnosis and repair, people with CoA who don’t have other heart issues can have a near-normal lifespan after repair. In the past, the average life expectancy for people with coarctation of the aorta was just 35 years old.