Overview

The aorta is the largest artery in your body. It moves oxygen-rich blood from your heart to the rest of your body. Narrowing of the aorta (aortic coarctation, pronounced ko-ahrk-TAY-shun) forces your heart to pump harder to move blood through the aorta.

Coarctation of the aorta is generally present at birth (congenital). Although the condition can affect any part of the aorta, the defect is most often located near a blood vessel called the ductus arteriosus. Symptoms can range from mild to severe. It might not be detected until adulthood, depending on how much the aorta is narrowed.

Coarctation of the aorta often occurs along with other heart defects. Though treatment is usually successful, the condition requires careful lifelong follow-up.

Symptoms

Coarctation of the aorta symptoms depend on the severity of the condition. Most people don't have symptoms. Mild coarctation may not be diagnosed until adulthood.

Babies with severe coarctation of the aorta may begin having symptoms shortly after birth. These include:

• Pale skin
• Irritability
• Heavy sweating
• Difficulty breathing
• Difficulty feeding

People with coarctation of the aorta may also have signs or symptoms of other heart defects, which often occur with the condition.

Signs or symptoms of coarctation of the aorta after infancy commonly include:

• High blood pressure
• Headaches
• Muscle weakness
• Leg cramps or cold feet
• Nosebleeds
• Chest pain

When to see a doctor

Seek medical help if you or your child has the following signs or symptoms:

• Severe chest pain
• Fainting
• Sudden shortness of breath
• Unexplained high blood pressure

While having these signs or symptoms doesn't always mean that you have a serious health condition, it's best to get checked out quickly. Early detection and treatment might help save your life.

Causes

Doctors aren't certain what causes coarctation of the aorta. The condition is generally present at birth (congenital). Congenital heart defects are the most common of all birth defects.

Rarely, coarctation of the aorta develops later in life. Conditions or events that can narrow the aorta and cause this condition include:

• Traumatic injury
• Severe hardening of the arteries (atherosclerosis)
• Inflamed arteries (Takayasu's arteritis)

Coarctation of the aorta usually occurs beyond the blood vessels that branch off to your upper body and before the blood vessels that lead to your lower body. This can often lead to high blood pressure in your arms but low blood pressure in your legs and ankles.

With coarctation of the aorta, the lower left heart chamber (left ventricle) of your heart works harder to pump blood through the narrowed aorta, and blood pressure increases in the left ventricle. This may cause the wall of the left ventricle to thicken (hypertrophy).

Risk factors

Coarctation of the aorta often occurs along with other congenital heart defects. Certain heart conditions are often associated with coarctation, including:

• Bicuspid aortic valve. The aortic valve separates the lower left chamber (left ventricle) of the heart from the aorta. A bicuspid aortic valve has two flaps (cusps) instead of the usual three. Many people with coarctation of the aorta have a bicuspid aortic valve.
• Sub-aortic stenosis. Sub-aortic stenosis occurs when there is a narrowing of the area below the aortic valve that obstructs blood flow from the left ventricle to the aorta. This narrowing may be in the form of a fibrous membrane.
• Patent ductus arteriosus. The ductus arteriosus is a blood vessel that connects a baby's left pulmonary artery to the aorta. It allows blood to bypass the lungs while the baby is growing in the womb. Shortly after birth, the ductus arteriosus usually closes. If it remains open, it's called a patent ductus arteriosus.
• Holes in the wall between the left and right sides of the heart. Some people are born with a hole in the wall (septum) between the upper heart chambers (atrial septal defect) or the lower heart chambers (ventricular septal defect).This causes oxygen-rich blood from the left side of the heart to mix with oxygen-poor blood in the right side of the heart.
• Congenital mitral valve stenosis. The mitral valve is between the upper and lower left heart chambers. It lets blood flow through the left side of your heart. In mitral valve stenosis, the valve is narrowed. As a result, the movement of blood between the upper and lower left heart chambers is reduced, increasing the pressure in the upper left chamber (atrium). The oxygen-rich blood from the lungs returns to the heart through veins that attach to the upper left chamber. Increased pressure in the left atrium may result in symptoms of lung congestion. These symptoms include shortness of breath, difficulty breathing during exercise and shortness of breath when lying flat.

Coarctation of the aorta is more common in males than in females, as well as in those who have certain genetic conditions, such as Turner syndrome.

Complications

Without treatment, coarctation of the aorta frequently leads to complications. In babies, it may lead to heart failure or death.

High blood pressure is the most common long-term complication of coarctation of the aorta. Blood pressure usually drops after the aortic coarctation has been repaired, but may still be higher than normal.

Other complications of coarctation of the aorta may include:

• A weakened or bulging artery in the brain (brain aneurysm) or bleeding in the brain (hemorrhage)
• Aortic rupture or tear (dissection)
• Enlargement in a section of the wall of the aorta (aneurysm)
• Heart failure
• Premature coronary artery disease — narrowing of the blood vessels that supply the heart
• Stroke

If the coarctation of the aorta is severe, your heart might not be able to pump enough blood to your other organs. This can cause heart damage and may lead to kidney failure or other organ failure.

Complications are also possible after treatment for coarctation of the aorta. They include:

• Aorta re-narrowing (re-coarctation, possibly years after treatment)
• High blood pressure
• Aortic aneurysm or rupture

You will need lifelong follow-up for coarctation of the aorta, and you may require additional treatments.

Prevention

Coarctation of the aorta can't be prevented, because it's usually present at birth. However, if you or your child has a condition that increases the risk of aortic coarctation, such as Turner syndrome, bicuspid aortic valve or another heart defect, or a family history of congenital heart disease, early detection can help. Discuss the risk of aortic coarctation with your doctor.

Diagnosis

The age at which coarctation of the aorta is diagnosed depends on the severity of the condition. If the aortic coarctation is severe, it's usually diagnosed during infancy. Coarctation of the aorta is occasionally diagnosed on sonogram of the fetus.

Adults and older children who are diagnosed with coarctation of the aorta may have milder cases and not have symptoms. They may often appear healthy until a doctor detects:

• High blood pressure in the arms
• A blood pressure difference between the arms and legs, with higher blood pressure in the arms and lower blood pressure in the legs
• A weak or delayed pulse in the legs
• A heart murmur — an abnormal whooshing sound caused by faster blood flow through the narrowed area

Tests

Tests to confirm a diagnosis of coarctation of the aorta may include:

• Echocardiogram. Echocardiograms use sound waves to create moving images of your heart that can be viewed on a video screen. This test can often show your doctor the location and severity of the aortic coarctation. It can also reveal other heart defects, such as a bicuspid aortic valve. Doctors often use echocardiograms to diagnose coarctation of the aorta and determine best treatment options for you.

• Electrocardiogram (ECG). An electrocardiogram records the electrical signals in your heart. During this test, sticky patches (electrodes) are attached to your chest and limbs. The patches have wires that connect to a monitor. They record the electrical signals that make your heart beat. A computer records the information and displays it as waves on a monitor or on paper.

  If the coarctation of the aorta is severe, the ECG may show thickening of the walls of the lower heart chambers (ventricular hypertrophy).

• Chest X-ray. A chest X-ray creates images of your heart and lungs. A chest X-ray might show a narrowing in the aorta at the site of the coarctation or an enlarged section of the aorta or both.
• Magnetic resonance imaging (MRI). An MRI uses a powerful magnetic field and radio waves to produce detailed images of your heart and blood vessels. This test can show the location and severity of the coarctation of the aorta, damage to other blood vessels, and any other heart defects. Your doctor may also use MRI results to guide treatment.
• Computerized tomography (CT) scan. A CT scan uses a series of X-rays to create detailed cross-sectional images of your body.
• CT angiogram. A CT angiogram uses a dye and special X-rays to show the inside of your coronary arteries. It reveals blood flow in your veins and arteries. The test can show the location and severity of the coarctation of the aorta and determine whether it affects other blood vessels in your body. A CT angiogram can also be used to detect other heart defects or help guide treatment options.

• Cardiac catheterization. During this procedure, your doctor inserts a long, thin tube (catheter) into an artery or vein in your groin, arm or neck and threads it to your heart using X-ray imaging. Sometimes, dye is injected through the catheter to help your heart structures show up more clearly on the X-ray images.

  Cardiac catheterization can help determine the severity of the aortic coarctation. Your doctor may use it to help plan surgery or other treatment, if you need it. Catheter procedures may also be used to perform certain treatments for coarctation of the aorta.

Treatment

Treatment for coarctation of the aorta depends on your age at the time of diagnosis and the severity of your condition. Other heart defects might be repaired at the same time as aortic coarctation.

A doctor trained in congenital heart conditions will evaluate you and determine the most appropriate treatment for your condition.

Medication

Medication isn't used to repair coarctation of the aorta. However, your doctor may recommend it to control blood pressure before and after stent placement or surgery. Although repairing aortic coarctation improves blood pressure, many people still need to take blood pressure medication after a successful surgery or stenting.

Babies with severe coarctation of the aorta often are given a medication that keeps the ductus arteriosus open. This allows blood to flow around the constriction until the coarctation is repaired.

Surgery or other procedures

There are several surgeries to repair aortic coarctation. Your doctor can discuss with you which type is most likely to successfully repair your or your child's condition. The options include:

• Resection with end-to-end anastomosis. This method involves removing the narrowed segment of the aorta (resection) followed by connecting the two healthy sections of the aorta together (anastomosis).
• Subclavian flap aortoplasty. A part of the blood vessel that delivers blood to your left arm (left subclavian artery) might be used to expand the narrowed area of the aorta.
• Bypass graft repair.This technique involves bypassing the narrowed area by inserting a tube called a graft between the portions of the aorta.
• Patch aortoplasty. Your doctor might treat your coarctation by cutting across the narrowed area of the aorta and then attaching a patch of synthetic material to widen the blood vessel. Patch aortoplasty is useful if the coarctation involves a long segment of the aorta.

Balloon angioplasty and stenting

This procedure may be used as a first treatment for aortic coarctation instead of surgery, or it may be done if narrowing occurs again after coarctation surgery.

During balloon angioplasty, your doctor inserts a thin, flexible tube (catheter) into an artery in your groin and threads it through your blood vessels to your heart using X-ray imaging.

Your doctor places an uninflated balloon through the opening of the narrowed aorta. When the balloon is inflated, the aorta widens and blood flows more easily. Sometimes, a mesh-covered hollow tube (stent) is placed in the aorta to keep the narrowed part of the aorta open.

Lifestyle and home remedies

Although coarctation of the aorta may be repaired, regular doctor's checkups are needed for the rest of your or your child's life to monitor for complications.

Your doctor will also check your blood pressure and treat it as needed.

Here are a few tips for managing your condition:

• Get regular exercise. Regular exercise helps lower blood pressure. Talk to your doctor about whether you need to restrict certain physical activities, such as weightlifting, which can temporarily raise your blood pressure.

  Your doctor may evaluate you and conduct exercise tests prior to deciding if you should participate in competitive sports or lift weights.

• Consider pregnancy carefully. Women with coarctation of the aorta, even after repair, may have a higher risk of aortic rupture, aortic dissection or other complications during pregnancy and delivery. If you are thinking of having a baby, talk to your doctor to determine how to safely do so. If you have had aortic coarctation repair and are considering becoming pregnant, careful management of your blood pressure is important to help keep you and your baby healthy.
• Prevent endocarditis. Endocarditis is an inflammation of the inner lining of the heart or of its structures, caused by a bacterial infection. You generally don't need to take antibiotics before certain dental procedures to prevent endocarditis. However, if you've had endocarditis in the past or had valve replacement surgery, then your doctor may recommend antibiotics.

Preparing for an appointment

If you or your child develops signs and symptoms of coarctation of the aorta, call your doctor. After an initial exam, you or your child may be referred to a doctor trained in the diagnosis and treatment of heart conditions (cardiologist).

Here's some information to help you prepare for your appointment, and what to expect from your doctor.

What you can do

• Write down any signs and symptoms you or your child has had, and for how long.
• Write down key medical information, including any other health conditions and the names of any medications you or your child is taking.
• Find a family member or friend who can come with you to the appointment, if possible. Someone who accompanies you can help remember what the doctor says.
• Write down the questions you want to be sure to ask your doctor.

Questions to ask the doctor at the initial appointment include:

• What is likely causing these symptoms?
• Are there any other possible causes for these symptoms?
• What tests are needed?
• Should a specialist be consulted?

Questions to ask if you're referred to a cardiologist include:

• Do I or does my child have coarctation of the aorta?
• How severe is the defect?
• Did tests reveal any other heart defects?
• What is the risk of complications from coarctation of the aorta?
• What treatment approach do you recommend?
• If you're recommending medications, what are the possible side effects?
• If you're recommending surgery, what type of procedure is most likely to be effective? Why?
• What will be involved in recovery and rehabilitation after surgery?
• How often should my child or I be seen for follow-up exams and tests?
• What signs and symptoms should I watch for at home?
• What is the long-term outlook for this condition?
• Do you recommend any dietary or activity restrictions?
• Do you recommend taking antibiotics before dental appointments or other medical procedures?
• Is it safe for a woman with coarctation of the aorta to become pregnant?
• What is the risk that my or my child's future children will have this defect?
• Should I meet with a genetic counselor?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time if you don't understand something.

What to expect from your doctor

A doctor who sees you or your child for possible coarctation of the aorta might ask a number of questions.

If you're the person affected:

• What are your symptoms?
• When did you first begin experiencing symptoms?
• Have your symptoms gotten worse over time?
• Do your symptoms include shortness of breath?
• Do your symptoms include headaches or dizziness?
• Do your symptoms include chest pain?
• Do your symptoms include cold feet?
• Have you had any weakness or leg cramps with exercise?
• Have you ever fainted?
• Do you have frequent nosebleeds?
• Does exercise or physical exertion make your symptoms worse?
• Have you been diagnosed with any other medical conditions?
• What medications are you currently taking, including over-the-counter and prescription drugs, as well as vitamins and supplements?
• Are you aware of any history of heart problems in your family?
• Do you or did you smoke? How much?
• Do you have any children?
• Are you planning to become pregnant in the future?

If your baby or child is affected:

• What are your child's symptoms?
• When did you first notice these symptoms?
• Is your child gaining weight at a normal rate?
• Does your child have any breathing problems, such as running out of breath easily or breathing rapidly?
• Does your child tire easily?
• Does your child sweat heavily?
• Does your child seem irritable?
• Do your child's symptoms include chest pain?
• Do your child's symptoms include cold feet?
• Has your child been diagnosed with any other medical conditions?
• Is your child currently taking any medications?
• Are you aware of any history of heart problems in your child's family?
• Is there a history of congenital heart defects in your child's family?