Coarctation of the aorta is a type of congenital heart disease. Kink in the part of the aorta, the main artery leaving the left heart. The degree of taper may vary from person to person. In severe coarctation, symptoms usually appear within the first weeks of life. Coarctation of the aorta is sometimes detected later in childhood, and from time to time during adulthood. Surgery may be performed to repair the narrow part of the aorta. Sometimes, a balloon can be inserted into the aorta to open the nip.

On this page

• Some anatomy of the normal heart and major blood vessels
• What is coarctation of the aorta?
• What causes coarctation of the aorta?
• How common is coarctation of the aorta?
• What are the symptoms of coarctation of the aorta?
• How is coarctation of the aorta diagnosed?
• What is the treatment of coarctation of the aorta?
• Are there any complications?
• More help and information
• References

Some anatomy of the normal heart and major blood vessels

The heart is a fist-sized organ located in the chest behind the sternum (breast bone). Is a muscular pump that pushes blood through the blood vessels throughout the body. The heart is located on the diaphragm, the main muscle of respiration, which is below the lungs. The heart is considered to have two sides – the right and the left.

The heart has four chambers – an atrium and a ventricle on each side. The atria are separated by a partition wall called handset. The two ventricles are separated by a wall called the ventricular septum. The atria are both supplied by large blood vessels that carry blood to the heart.

Atria have special valves that open into the ventricles. The valve that opens from the right atrium to the right ventricle is called the tricuspid valve. The valve opens from the left atrium into the left ventricle is called the mitral valve. The ventricles have valves but also in this case open into the blood vessels. The pulmonary valve opens from the right ventricle to the pulmonary artery. The aortic valve opens from the left ventricle into the aorta. The walls of the heart chambers are made mainly of special heart muscle. The different sections of the heart have to contract (squeeze) in the correct order for the heart to pump blood efficiently with each heartbeat.

The main function of the heart is to pump blood around the body.

The right side of the heart receives deoxygenated blood (oxygen) in the body. After passing through the right atrium and the right ventricle, this blood is pumped through the pulmonary valve into the pulmonary artery to the lungs. Here blood picks up oxygen and loses another gas called carbon dioxide. Once through the lungs, blood flows back into the left atrium. It then passes into the left ventricle and is pumped through the aortic valve into the aorta, the major artery supplying the body. The oxygenated blood while the blood vessels leads to all tissues of the body. Here oxygen and other nutrients to the cells go where they are used for essential body functions.

For more information about how the heart and blood vessels work, please see separate leaflet called "Heart and Blood Vessels.

What is coarctation of the aorta?

Coarctation of the aorta is a type of congenital heart disease. (A congenital condition is a condition that you are born with.) There are a number of different types of congenital heart disease. Coarctation of the aorta is a single type.

As mentioned above, the aorta is the main artery from the left side of the heart (left ventricle) and supplies oxygen (oxygen-rich) of blood to the body. The aorta is the main artery of the body and all the other arteries of the body is derived from it. After leaving the heart, arteries other branches of the aorta and supply blood to the head and arms, but the main aorta goes through the chest, abdomen, and supplies blood to the lower body and legs.

In coarctation of the aorta, there is a narrowing in the part of the aorta, usually the upper part of the aorta, just after the arteries supplying blood to the head and have forked arms. Blood can not pass as easily through the narrow part of the aorta. This means that the blood supply to the lower half of the body may be affected (less blood is able to get through). Also, due to reduction, the heart works harder to pump and trying to squeeze through the narrowing blood. Because the heart is working harder to pump, this can cause the blood pressure to rise. Often, a person with coarctation of the aorta is high blood pressure in your upper body and arms (or arm) and low blood pressure in your lower body and legs. In addition, the muscular walls of the left ventricle may thicken (hypertrophy) due to additional work that they are having to do.

The degree of narrowing of the coarctation may be different in different people. Some babies will be born with severe narrowing that can lead to symptoms shortly after birth (see below). However, in others, the reduction is less severe symptoms and may go unnoticed until later. In some people, the gradual reduction can worsen over time and therefore lead to symptoms. Sometimes, coarctation of the aorta causes no symptoms or not detected until adulthood.

When a baby is growing inside the womb, the pulmonary artery and the aorta are joined together by a small blood vessel called the ductus arteriosus. This means that most of the blood leaving the right ventricle passes through the lungs and passes directly into the aorta. Once the baby is born, the ductus arteriosus closes, usually within the first few weeks of life. If a baby has severe coarctation of the aorta, the only way that blood can pass to the lower half of the body is through the ductus arteriosus. So, when you close the PDA in these infants, the blood will not reach the lower half of the body, causing severe symptoms and, if nothing is done about it, the baby can die.

What causes coarctation of the aorta?

Coarctation of the aorta is due to a problem with the development of the baby's aorta while growing in the womb. In most cases, do not know why this problem occurs.

Coarctation of the aorta may occur alone, or it may occur with other congenital heart problems. The other congenital heart problems that coarctation of the aorta occurs most often are either:

• A bicuspid aortic valve (aortic valve is normally composed of three leaflets or flaps, a bicuspid aortic valve has only two cusps) – O
• A ventricular septal defect (a condition in which there is a hole in the wall – septum – which separates the two ventricles).

In some babies, coarctation of the aorta may develop as part of a syndrome (set of symptoms) that a baby is born. For example, coarctation of the aorta may be part of Turner syndrome. (This is a genetic condition that only affects girls. This is a chromosome problem. The most characteristic features of the disease are being short, having certain physical characteristics, such as a typical facial appearance, and also have ovaries that do not work properly.)

How common is coarctation of the aorta?

In about 7 out of every 1,000 pregnancies, the fetus has a congenital heart disease. Coarctation of the aorta is only one type of congenital heart disease. About 5 out of every 100 babies with congenital heart disease have coarctation of the aorta. The condition is twice as common in men than in women.

What are the symptoms of coarctation of the aorta?

As mentioned above, the degree of narrowing of the aorta coarctation may vary from person to person.

If the narrowing is severe, symptoms usually become evident in the first weeks of life (such as the ductus arteriosus closes). The baby can not eat well, you can not put on weight and may tire easily. They can become very sick and may develop heart failure because the heart can not pump blood through the narrow part of the aorta. This causes a back pressure of the blood and the congestion of the lungs, resulting in symptoms such as shortness of breath.

If the narrowing is less severe, symptoms may not be evident until later in childhood (and sometimes into adulthood). This is because the body develops additional blood vessels, called collateral blood vessels, so some blood is able to bypass the narrowed part of the aorta. These collateral blood vessels may be sufficient for some time, but finally the heart is no longer able to cope and heart failure symptoms start to appear. These include difficulty breathing, coughing, feeling tired and swollen feet and legs.

Sometimes, coarctation of the aorta may be suspected before symptoms have developed. For example, during a routine check on a baby or a child, the doctor may hear a heart murmur when listening to the child's heart. It is a sound that the doctor hears through his stethoscope. It is due to turbulent blood flow through the narrow portion of the aorta. Or, a child may be found to have high blood pressure during a routine checkup. In coarctation of the aorta, the blood pressure in the arms may be high and above the legs. The doctor may also feel the difference when you check your pulse on the child's arms and legs. Due to the taper, less blood gets through the legs and lower body. This means that the pulses in the legs may feel weak.

How is coarctation of the aorta diagnosed?

If a doctor suspected coarctation of the aorta due to symptoms or a heart murmur, blood pressure or heart rate problems, usually suggest some research to confirm the diagnosis. These may include:

• Echocardiogram (ultrasound of the heart) – which usually appears narrowing of the aorta. It may also be possible to measure the pressure differences on either side of the neck (called the pressure gradient) and to give an idea of how the narrowing is severe.
• Chest X-ray – this may show that the heart is enlarged. It may show fluid in the lungs if heart failure. You can also show some "notches" (or slots) in the ribs if developed collateral blood vessels.
• Electrocardiogram (ECG) – this is a test that records the heart's electrical activity. May be abnormal in some patients with coarctation of the aorta.
• Cardiac catheterization – A catheter is a thin, flexible, hollow. Cardiac catheterization is where a thin plastic catheter into the heart chambers. The catheter can also be passed to the major blood vessels of the heart (the coronary arteries). It is sometimes performed if someone has coarctation of the aorta. The pressure difference on both sides of the narrowing may be measured to see the severity of coarctation is. Please see separate leaflet called "Cardiac Catheterization 'for details.

Sometimes, coarctation of the aorta can be detected when a woman has her routine ultrasound during pregnancy. The aorta may be abnormally small when looking at the heart of your baby. However, coarctation of the aorta can be very difficult to detect this.

What is the treatment of coarctation of the aorta?

Treatment to help stabilize the symptoms

If a newborn baby has severe coarctation of the aorta, the first thing you need treatment to help control their symptoms. This may include medicines to help treat any heart failure and sometimes you may need artificial ventilation to assist with breathing. May also be given a special drug that can help keep the ductus arteriosus open so that blood can flow to the lower body (below the narrowing of the aorta).

If a child has developed high blood pressure due to coarctation of the aorta, which may need treatment with medications.

Treatment for narrowing of the aorta

The narrowing of the aorta can be repaired with surgery. The surgeon will have to open the chest of the child, so they can access the child's heart and aorta to operate them. A clamp is placed across the aorta to stop the blood flow through it. Then, the tapered section is cut and the two sides of normal size of the aorta are joined back together. Sometimes, if a large part of the aorta must be removed due to narrowing, a patch (or graft) special synthetic material is used to fill the void and repair the aorta.

A new way to treat coarctation of the aorta is by using balloon angioplasty. This can be used in older children who have been diagnosed with the disease. A balloon catheter (a thin, flexible, hollow with a deflated balloon at the tip) is inserted into a large blood vessel, usually an artery in the groin. X-ray guidance is used and then the catheter is passed through the blood vessels within the body until it reaches the narrow part of the aorta. The balloon is inflated within the narrow section, making the overall narrowing. Sometimes a stent (a small tube, expanded metal) is placed in the narrowed segment to keep it open.

Are there any complications?

If left untreated, the prognosis (outlook) for coarctation of the aorta is usually good. However, there are some complications that can occur in some people. These may be complications related to surgery to repair the narrowing or other complications.

The complications of surgery

Any child who has had surgery for coarctation of the aorta need regular checkups with a specialist later. Many children have no serious complications from the surgery and will then lead a healthy life. However, sometimes develop complications, either upon or shortly after surgery. These may include:

• Internal bleeding during surgery.
• Infection of the wound after surgery.
• Damage to the kidneys during surgery.
• Damage to the spinal cord due to surgery. This is most likely if the operation is more complicated and the aorta must be held by a long period of time. (Because the aorta is clamped, can stop blood getting through the spinal cord.) If the spinal cord is damaged, it may mean that the child's legs can become permanent paralysis. This complication is rare, but can obviously be very serious.
• A stroke due to surgery. However, this is also very rare.

Some are also other possible complications with time after surgery. These may include:

• Return of the narrowing of the aorta. This is known as recurrent coarctation.
• An aortic aneurysm. This is a widening or dilation of the aorta. An aortic aneurysm can be a complication of coarctation of the aorta that has not been treated with surgery, but also is a rare complication after surgery. The widened portion of the aorta is weaker and the risk exists that can break (or burst), causing severe internal hemorrhage.
• Persistent high blood pressure despite the fact that the narrow part of the aorta has been extended or is no longer present. This may require treatment with medication.

Other complications

Anyone with coarctation of the aorta (if they had surgery or not) has a small risk of infectious endocarditis. This is an infection that affects part of the endocardium (the lining inside the heart chambers). The infection usually involves one or more heart valves that are part of the endocardium. Infective endocarditis is a rare but serious infection that can be fatal if not detected and treated. A separate leaflet called "Endocarditis – infectious' gives more details.

About 4 out of every 100 people with coarctation of the aorta aneurysm also have one of the small blood vessels in the brain, called berry aneurysm. As mentioned above, an aneurysm is an abnormally enlarged, or expanded, in a blood vessel. The widened portion tends to be weak and there is a risk that it may rupture. If an aneurysm in the brain bursts, this can lead to bleeding in the brain and subarachnoid hemorrhage. However, not all saccular aneurysm will burst. The symptoms of a subarachnoid hemorrhage can include severe headache (often described as feeling like someone has beaten on the back of the head), vomiting, dizziness, vision problems, or a seizure (convulsion).

A number of women who have had treatment for coarctation of the aorta will have a normal pregnancy with no problems. However, if a woman has coarctation of the aorta, or received treatment for her, and she is planning to become pregnant, you should discuss this with your cardiologist first. This is because pregnancy can put extra strain on the heart. It is best to plan a pregnancy when symptoms of women and heart condition are stable.

Also, if someone is born with congenital heart disease themselves, chances are they will have a baby born with congenital heart disease also. This risk should be discussed with a heart specialist. It may be possible to make the baby's heart scans from early pregnancy to check for heart problems.

More help and information

Children's Heart Federation

2-4 Great Eastern Street, London, EC2A 3NW
Tel (Helpline): 0808 808 5000 Web: www.chfed.org.uk
A parent-run charity dedicated to helping children with congenital or acquired heart disease and their families in the UK and Ireland.

Grown Up Congenital Heart Patients Association (GUCH)

Saracen House, 25 St Margaret Green, Ipswich IP4 2BN
Tel (Helpline): 0800 854 759 Web: www.guch.org.uk
A national organization of support and counseling to adults and adolescents born with congenital heart defects.

British Heart Foundation

Greater London House, 180 Hampstead Road, London NW1 7AW
Tel (Heart Helpline): 0300 330 3311 Web: www.bhf.org.uk