Coarctation of the Aorta

What happens in coarctation

The aorta carries oxygen-rich blood from the left side of the heart to the body. Coarctation is usually a pinch-like narrowing just past the artery to the left arm, close to where the ductus arteriosus (a fetal blood vessel) joins the aorta.

Before birth, the ductus allows blood to bypass the narrowing. When the ductus closes in the first days after birth, a severe coarctation can suddenly block blood flow to the lower body, causing shock and heart failure.

With milder narrowing:

• the heart pumps harder, so blood pressure is high in the arms and head
• blood pressure is lower in the legs, and leg pulses are weak or delayed
• the body grows collateral blood vessels around the narrowing, including enlarged arteries between the ribs

Associated conditions

• Bicuspid aortic valve: a valve with two leaflets instead of three, found in a large share of people with coarctation
• Turner syndrome: in girls, a common association
• Other heart defects: ventricular septal defect, patent ductus arteriosus, mitral valve abnormalities and hypoplastic left heart
• Brain aneurysms: more common in people with coarctation

Symptoms

In newborns and infants (critical coarctation)

• fast or labored breathing
• poor feeding and sweating with feeds
• pale, gray or mottled skin, especially the legs
• weak pulses in the legs
• irritability, then lethargy
• low urine output

These symptoms often appear when the ductus closes in the first 1 to 2 weeks. Newborn pulse oximetry screening can detect some cases but may miss coarctation.

In older children and adults

Many have no symptoms. When present:

• high blood pressure in the arms, often found at a routine check
• headaches
• nosebleeds
• cold feet
• leg cramps or tiredness with exercise
• chest pain
• a heart murmur

Diagnosis

• Blood pressure in arms and legs: a systolic pressure more than about 20 mmHg higher in the arms than the legs is a strong clue.
• Pulse check: weak or delayed femoral (groin) pulses compared with arm pulses.
• Echocardiogram: shows the narrowing and other heart defects.
• CT or MRI angiography: maps the narrowing and collateral vessels, used before repair and for follow-up.
• Chest X-ray: may show rib notching from enlarged collateral arteries in older children and adults.
• ECG: may show thickening of the heart muscle.

Treatment

Newborns with critical coarctation

• Prostaglandin infusion to reopen the ductus and restore blood flow to the lower body
• Intensive care support
• Surgical repair, usually removing the narrowed segment and joining the ends (resection with end-to-end anastomosis), in the first days to weeks

Older children and adults

• Balloon angioplasty with stent placement through a catheter is commonly used in larger children, teens and adults.
• Surgery is used for complex anatomy.
• Blood pressure medicines before and after repair if pressure stays high.

Complications

Untreated or late-treated coarctation can lead to:

• persistent high blood pressure, even after repair
• heart failure
• aortic dissection or aneurysm
• stroke and brain aneurysm rupture
• coronary artery disease
• infection of the heart lining (endocarditis)

After repair, possible problems include re-narrowing (recoarctation), aneurysm at the repair site and ongoing high blood pressure.

Living with repaired coarctation

Lifelong follow-up with a congenital heart specialist is recommended, including:

• regular blood pressure checks in arms and legs
• periodic imaging of the aorta with MRI or CT
• treatment of high blood pressure
• assessment of the aortic valve
• pre-pregnancy counseling, because pregnancy increases strain on the aorta

Most people who have repair in childhood lead active lives. Earlier repair lowers the risk of long-term high blood pressure.

Frequently asked questions

What are the signs of coarctation of the aorta in a newborn?

Signs include fast breathing, poor feeding, sweating, pale or gray skin, cold legs, weak leg pulses and lethargy, often in the first 1 to 2 weeks when the ductus arteriosus closes. This is an emergency needing immediate care.

Can coarctation of the aorta be found in adults?

Yes. Milder coarctation may not be found until adulthood, often during investigation of high blood pressure. Clues include higher blood pressure in the arms than legs, weak groin pulses, cold feet and leg cramps with exercise.

Is coarctation of the aorta genetic?

It is usually not inherited in a simple pattern, but it is more common in some genetic conditions, especially Turner syndrome, and runs in some families with other left-sided heart defects. A bicuspid aortic valve often occurs alongside it.

How is coarctation of the aorta treated?

Newborns usually need surgery to remove the narrowed segment. Older children and adults are often treated with balloon angioplasty and a stent through a catheter. Blood pressure medicines may be needed before and after repair.

What is the life expectancy after coarctation repair?

Many people live into older age after repair, especially when it is done early. However, life expectancy can be reduced by high blood pressure, aortic complications and heart disease, which is why lifelong follow-up and blood pressure control are important.