Coarctation of the aorta
Coarctation (ko-ahrk-TAY-shun) of the aorta — or aortic coarctation — is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this occurs, your heart must pump harder to force blood through the narrowed part of your aorta.
Coarctation of the aorta is generally present at birth (congenital). The condition can range from mild to severe, and 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. While treatment is usually successful, the condition requires careful lifelong follow-up.
Coarctation of the aorta symptoms depend on the severity of the condition. Most people don't have symptoms. Children with serious aortic narrowing may show signs and symptoms earlier in life, but mild cases with no symptoms might not be diagnosed until adulthood. People may also have signs or symptoms of other heart defects that they have along with coarctation of the aorta.
Babies with severe coarctation of the aorta may begin having signs and symptoms shortly after birth. These include:
- Pale skin
- Heavy sweating
- Difficulty breathing
- Difficulty feeding
Left untreated, aortic coarctation in babies might lead to heart failure or death.
Older children and adults with coarctation of the aorta often don't have symptoms because their narrowing may be less severe. If you have signs or symptoms that appear after infancy, you most commonly will have high blood pressure (hypertension) measured in your arms. However, your blood pressure is likely to be lower in your legs. Signs and symptoms might include:
- High blood pressure
- Muscle weakness
- Leg cramps or cold feet
- Chest pain
When to see a doctor
Seek medical help if you or your child has the following signs or symptoms:
- Severe chest pain
- Sudden shortness of breath
- Unexplained high blood pressure
While experiencing these signs or symptoms doesn't necessarily mean that you have a serious problem, it's best to get checked out quickly. Early detection and treatment might help save your life.
Doctors aren't certain what causes coarctation of the aorta (aortic coarctation). For unknown reasons, mild to severe narrowing develops in part of the aorta. Although aortic coarctation can occur anywhere along the aorta, the coarctation is most often located near a blood vessel called the ductus arteriosus. The condition generally begins before birth (congenital). Congenital heart defects are the most common of all birth defects.
Rarely, coarctation of the aorta develops later in life. Traumatic injury might lead to coarctation of the aorta. Rarely, severe hardening of the arteries (atherosclerosis) or a condition causing inflamed arteries (Takayasu's arteritis) can narrow the aorta, leading to aortic coarctation.
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).
Coarctation of the aorta often occurs along with other congenital heart defects, although doctors don't know what causes multiple heart defects to form together. The condition is more common in males than in females. You or your child may be more likely to have aortic coarctation if certain heart conditions exist, 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.
Patent ductus arteriosus. Before birth, the ductus arteriosus is a blood vessel connecting the left pulmonary artery to the aorta — allowing blood to bypass the lungs.
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. You may have a hole in the wall (septum) between the upper chambers of the heart (atrial septal defect) or the lower chambers of the heart (ventricular septal defect) when you're born.
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.
Aortic valve stenosis. This is a narrowing of the valve that separates the left ventricle of the heart from the aorta (aortic valve). This means your heart has to pump harder to get adequate blood flow to your body.
Over time, this can cause your heart muscle to thicken and lead to symptoms such as chest pain, fainting spells and breathlessness, or heart failure.
- Aortic valve regurgitation. This occurs when the aortic valve doesn't close tightly, causing blood to leak backward into the left ventricle.
Mitral valve stenosis. This is a narrowing of the valve (mitral valve) between the upper left heart chamber (left atrium) and the left ventricle that lets blood flow through the left side of your heart.
In this condition, blood may back up into your lungs, causing shortness of breath or lung congestion. Like aortic valve stenosis, this condition can also lead to heart failure.
- Mitral valve regurgitation. This occurs when the mitral valve doesn't close tightly, causing blood to leak backward into the left atrium.
Coarctation of the aorta is also more common in people who have certain genetic conditions, such as Turner syndrome. Women and girls with Turner syndrome have 45 chromosomes, with one missing or incomplete X chromosome, instead of 46. About 10 percent of women and girls with Turner syndrome have aortic coarctation.
Talk to you doctor if you or your child has of any of these risk factors or a family history of congenital heart disease.
Untreated coarctation of the aorta frequently leads to complications. Some complications may be a result of long-standing high blood pressure caused by the aortic coarctation. Complications are also possible after treatment for coarctation of the aorta.
Complications of coarctation of the aorta may include:
- Narrowing of the aortic valve (aortic stenosis)
- High blood pressure
- Enlargement in a section of the wall of the aorta (aneurysm)
- Aortic rupture or tear (dissection)
- Premature coronary artery disease — narrowing of the blood vessels that supply the heart
- Heart failure
- A weakened or bulging artery in the brain (brain aneurysm) or bleeding in the brain (hemorrhage)
In addition, 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 damage to your heart and also can result in kidney failure or other organ failure.
If your coarctation of the aorta was treated when you were young, you have a risk of the aorta re-narrowing (re-coarctation) over time. You also have a higher risk of developing high blood pressure. You will need lifelong follow-up for coarctation of the aorta, and you may require additional treatments.
Coarctation of the aorta can't be prevented, because it's usually present at birth (congenital). 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.
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. Testing for coarctation of the aorta before birth often isn't possible.
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 to confirm a diagnosis of coarctation of the aorta may include:
Echocardiogram. Echocardiograms use high-pitched sound waves to produce an image of your heart. Sound waves bounce off your heart and produce moving images that can be viewed on a video screen.
An echocardiogram can often detect the location and severity of the aortic coarctation and show other heart defects, such as a bicuspid aortic valve. Doctors often use echocardiograms to diagnose coarctation of the aorta and determine the most appropriate treatment options for you.
Electrocardiogram (ECG). An ECG records the electrical activity in your heart each time it contracts. During this procedure, you will have patches with wires (electrodes) placed on your chest, wrists and ankles. The electrodes measure electrical activity, which is recorded on paper or a computer monitor.
If the coarctation of the aorta is severe, the ECG may show that the walls of the lower heart chambers are thickened (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.
An MRI can reveal the location and severity of the coarctation of the aorta, determine whether it affects other blood vessels in your body, and detect whether you have other heart defects. Doctors may also use this test to determine your treatment options.
Computerized tomography (CT) scan. A CT scan uses a series of X-rays to create detailed cross-sectional images of your body.
In a CT angiogram, your doctor injects a dye into a blood vessel to highlight blood flow in your arteries and veins. A CT angiogram allows your doctor to see the location and severity of the coarctation of the aorta, determine whether it affects other blood vessels in your body, and detect other heart defects. Your doctor may also use this test to determine your 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.
Your doctor may inject a dye through the catheter to make your heart structures visible on X-ray pictures. The dye can also measure pressures and oxygen levels in the chambers of the heart and in the blood vessels. Cardiac catheterization can help determine the severity of the aortic coarctation.
This test isn't often used to diagnose coarctation of the aorta, but your doctor may use it to help plan surgery or other treatment, if you need it. Catheter procedures may be used to perform certain treatments for coarctation of the aorta.
Treatment options for coarctation of the aorta depend 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.
Treatment approaches usually consist of surgery or a procedure called balloon angioplasty or stent placement. A doctor trained in congenital heart conditions will evaluate you and determine the most appropriate treatment for your condition.
There are several surgical techniques 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 ends of the aorta together (anastomosis).
- Subclavian flap aortoplasty. A portion 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 plastic 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
Balloon angioplasty, often with stenting, is an option for initially treating aortic coarctation or for treating re-narrowing (re-coarctation) that has occurred after surgery.
During this procedure, 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. In some cases, your doctor may insert a mesh-covered hollow tube (stent) in the aorta to keep the narrowed part of the aorta open.
Medication isn't used to repair coarctation of the aorta, but it might be used to control blood pressure before and after stent or surgery. Although repairing aortic coarctation improves blood pressure, many people still need to take blood pressure medication, even 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 provides a bypass for blood flow around the constriction until the coarctation is repaired.
The most common long-term complication of coarctation of the aorta is high blood pressure. Although your blood pressure usually falls after the aortic coarctation has been repaired, it might still remain higher than normal.
Occasionally, the segment of the aorta that has been repaired will become weak and enlarge (aortic aneurysm) and might eventually rupture. In some cases, the coarctation can recur, possibly even years after treatment. It's possible to have additional surgeries or procedures to correct the re-narrowing or treat other complications.
Lifestyle and home remedies
Although coarctation of the aorta may be repaired, you'll need careful follow-up throughout life in order for doctors to monitor you for complications and recurrences.
Your doctor may suggest that you have regular follow-up appointments with a doctor who specializes in congenital heart disease. In follow-up appointments, your doctor may evaluate you and order imaging tests to monitor your condition. 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 weightlifting.
Consider pregnancy carefully. Before becoming pregnant, talk to your doctor to determine if you can undergo pregnancy safely. 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. Doctors will also evaluate whether your blood pressure is controlled.
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 won't need to take antibiotics before certain dental procedures to prevent endocarditis. However, if you've had endocarditis in the past, or if you had a stent placed or had valve replacement surgery, then your doctor may recommend antibiotics.
Preparing for an appointment
If you or your child develops signs and symptoms common to coarctation of the aorta, call your doctor. After an initial exam, it's likely that the doctor will refer you or your child 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 headache 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?