Coarctation of the aorta

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Coarctation of the aorta is a birth defect where part of the aorta, is narrower than usual.

Pronunciation

Coarctation of the aorta is pronounced koh-ark-TEY-shun

Birth defect

Coarctation of the aorta is a birth defect in which a part of the aorta is narrower than usual.

Needs surgery

If the narrowing is severe enough and if it is not diagnosed, the baby may have serious problems and may need surgery or other procedures soon after birth. For this reason, coarctation of the aorta is often considered a critical congenital heart defect.

Cause

The defect occurs when a baby’s aorta does not form correctly as the baby grows and develops during pregnancy. The exact cause is not known.

Location

The narrowing of the aorta usually happens in the part of the blood vessel just after the arteries branch off to take blood to the head and arms, near the patent ductus arteriosus, although sometimes the narrowing occurs before or after the ductus arteriosus.

Ductus arteriosus

• In some babies with coarctation, it is thought that some tissue from the wall of ductus arteriosus blends into the tissue of the aorta.
• When the tissue tightens and allows the ductus arteriosus to close normally after birth, this extra tissue may also tighten and narrow the aorta.

Coarctation Of Aorta

Mechanism

The narrowing, or coarctation, blocks normal blood flow to the body. This can back up flow into the left ventricle of the heart, making the muscles in this ventricle work harder to get blood out of the heart.

Effects on blood flow

Since the narrowing of the aorta is usually located after arteries branch to the upper body, coarctation in this region can lead to normal or high blood pressure and pulsing of blood in the head and arms and low blood pressure and weak pulses in the legs and lower body. If the condition is very severe, enough blood may not be able to get through to the lower body. The extra work on the heart can cause the walls of the heart to become thicker in order to pump harder. This eventually weakens the heart muscle. If the aorta is not widened, the heart may weaken enough that it leads to heart failure.

Coarctation of Aorta Close Up

Other congenital heart defects

Coarctation of the aorta often occurs with other congenital heart defects.

Incidence

• In a 2019 study, using 2010-2014 data from 39 population-based birth defects surveillance programs, the Centers for Disease Control and Prevention (CDC) estimated that about 4 of every 10,000 babies born had coarctation of the aorta.
• This means about 2,200 babies are born each year in the United States with coarctation of the aorta.
• In other words, about 1 in every 1,800 babies born in the United States each year are born with coarctation of the aorta.

Risk Factors

While the exact cause for coarctation of the aorta is unknown, some babies have heart defects because of changes in their genes or chromosomes.

coarctation of the aorta by CMR

Role of genes and environment

Heart defects, like coarctation of the aorta, are also thought to be caused by a combination of genes and other risk factors, such as things the mother comes in contact with in the environment, what the mother eats or drinks, or medicines the mother uses.

Diagnosis

Coarctation of the aorta is usually diagnosed after the baby is born. How early in life the defect is diagnosed usually depends on how mild or severe the symptoms are.

Newborn screening

Newborn screening using pulse oximetry during the first few days of life may or may not detect coarctation of the aorta.

In babies with a more serious condition, early signs usually include:

• pale skin
• irritability
• heavy sweating
• difficulty breathing

Physical exam

• Detection of the defect is often made during a physical exam.
• In infants and older individuals, the pulse will be noticeably weaker in the legs or groin than it is in the arms or neck, and a heart murmur—an abnormal whooshing sound caused by disrupted blood flow—may be heard through a doctor’s stethoscope.
• Older children and adults with coarctation of the aorta often have high blood pressure in the arms.

Tests - echocardiogram

• Once suspected, an echocardiogram is the most commonly used test to confirm the diagnosis.
• An echocardiogram will show the location and severity of the coarctation and whether any other heart defects are present.

Coarctation of the aorta

Other tests

Other tests to measure the function of the heart may be used including chest x-ray, electrocardiogram (EKG), magnetic resonance imaging (MRI), and cardiac catheterization.

Critical CHD

• Coarctation of the aorta is often considered a critical congenital heart defect (critical CHD) because if the narrowing is severe enough and it is not diagnosed, the baby may have serious problems soon after birth.
• CCHDs also can be detected with newborn pulse oximetry screening.

Treatments

No matter what age the defect is diagnosed, the narrow aorta will need to be widened surgically once symptoms are present. This can be done with surgery or a procedure called balloon angioplasty, which is done during a cardiac catheterization.

Balloon angioplasty

A balloon angioplasty is a procedure that uses a thin, flexible tube, called a catheter, which is inserted into a blood vessel and directed to the aorta. When the catheter reaches the narrow area of the aorta, a balloon at the tip is inflated to expand the blood vessel.

Coarctatio aortae - diagram - end-to-end anastomosis

Stents

• Sometimes a mesh-covered tube (stent) is inserted to keep the vessel open.
• The stent is used more often to initially widen the aorta or re-widen it if the aorta narrows again after surgery has been performed.

Surgical reconstruction of aorta

During surgery to correct a coarctation, the narrow portion is removed and the aorta is reconstructed or patched to allow blood to flow normally through the aorta.

Monitor BP

• Even after surgery, children with a coarctation of the aorta often have high blood pressure that is treated with medicine.
• It is important for children and adults with coarctation of the aorta to follow up regularly with a cardiologist to monitor their progress and check for other health conditions that might develop as they get older.

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Frequently asked question on Coarctation of the aorta

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• FAQ's on Coarctation of the aorta
• What is a characteristic sign of coarctation of the aorta?

High blood pressure especially in the upper body, low blood pressure or weak pulses in the lower part of the body, muscle weakness, headaches, etc.

• How do you assess for coarctation of the aorta?

In addition to a physical examination, tests such as echocardiogram, CT chest, X-rays, and other tests can help.

• What causes coarctation of the aorta?

The exact cause is not known. It is likely a combination of genes and other risk factors, such as things the mother comes in contact with in the environment, what the mother eats or drinks, or medicines the mother uses

• How rare is coarctation of the aorta?

1 in every 1,800 babies born in the United States each year are born with coarctation of the aorta.

• What can happen if the coarctation is not repaired?

It can lead to heart failure

• How long does surgery for coarctation of the aorta take?

While it depends, typically, it takes about 2-3 hours.

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Coarctation of the aorta on Wikipedia

• Wikipedia's article on Coarctation of the aorta