Coarctation of the Aorta (CoA)

What is coarctation of the aorta?

Coarctation of the aorta (CoA) is a congenital heart disease, which means the condition is present at birth. It involves narrowing of the aorta, the large blood vessel that caries oxygenated blood out of the left ventricle (or bottom chamber of the heart) to the body. The narrowing or “pinched” area can occur at single area or along a portion of the aorta. When the coarctation occurs, there is decreased blood flow to the lower body. The left ventricle has to pump harder to force the blood through the narrowed part of the aorta. The extra workload may cause the left ventricle to become less effective and lead to damage to the heart (heart failure).

The coarctation of the aorta condition can range from mild to severe. Many times the narrowing is noted shortly after birth, but sometimes, it is not detected until childhood or adulthood.

A coarctation of the aorta may occur as a single defect or with other congenital heart disease defects, typically involving the left side of the heart. The most common cardiac defects may include a bicuspid aortic valve, a ventricular septal defect, or complex single ventricle heart defect.

What causes of CoA in children?

Coarctation occurs in 1 out of 2,500 babies born in the United States. About 6-8% of people with congenital heart disease have coarctation of the aorta. The cause is unknown, though it is more common in babies who also have genetic conditions such as Turner syndrome.

How to diagnose coarctation of the aorta

Sometimes, the coarctation of the aorta can be diagnosed before your baby is born during a fetal ultrasound, although this may be difficult. Many coarctations are diagnosed after birth.

More often, doctors discover the coarctation of the aorta shortly after birth or even later in childhood or adulthood. The detection of the coarctation depends on the degree of narrowing within the aorta and the type of cardiac symptoms the individual develops. A moderate or severe coarctation can develop symptoms more quickly, while a mild coarctation may develop symptoms over years.

About one in four babies have a more severe coarctation of the aorta and develop cardiac symptoms within the first week of life or after the patent ductus arteriosus (PDA) closes. The PDA is a connecting vessel between the pulmonary artery (the blood vessel that carries lower oxygen carrying blood to the lungs) and the aorta. When the PDA closes, the area of narrowing can become worse, and the left ventricle has to pump against a higher body blood pressure.

Signs and symptoms

In infants with moderate to severe coarctation of the aorta, the cardiac symptoms may develop quickly. The signs and symptoms can include:

Heart murmur—an extra heart sound heard with a stethoscope
Pale or gray skin color
Irritability
Heavy sweating
Heavy and/or rapid breathing
Poor feeding
Poor weight gain
Coolness of the feet, ankle or legs
Strong pulses in the arms and weak or absent pulses in the femoral artery pulse (taken in the groin area) or in the legs
High blood pressures in the arms and lower/absent in the legs.

In people with a mild coarctation of the aorta, the symptoms may occur more slowly and not be detected until the child is older or possibly in adulthood. The signs and symptoms may include:

High blood pressure in the arms
Cold feet or legs
Shortness of breath with exercise
Chest pain
Dizziness
Fainting
Nosebleeds
Headaches
Leg cramps
Muscle weakness
Heart murmur

Diagnosis and testing for coarctation of the aorta

These tests can help your care team diagnose the coarctation of the aorta and create a treatment plan for your baby:

Basic Testing:

Pulse oximetry: a way to monitor the oxygen content of the blood via a light probe placed on your baby’s hand or foot. This test is not painful.
Electrocardiogram (ECG): a visual representation of the heart's electrical activity captured via monitors placed on the skin. This test is not painful.
Echocardiogram (echo): an ultrasound of the heart that evaluates the structure and the function of the heart by using sound waves. Still and moving pictures of the heart structures, heart valves, and heart function are recorded for review by a cardiologist. This test is not painful.
Chest X-ray: a test that uses a small amount of radiation to create an image (or picture) within the chest to include the heart, lungs, blood vessels and bones. This test is not painful.

Advanced Testing—These studies may require sedation for completion:

CT (Computed tomography) scan: a test that uses x-rays and computers to produce images of the body/heart from different angles. The test is completed in radiology. The patient lies on a table which moves through a large, donut-shaped scanning device. This test is not painful.
Cardiac MRI: a test that produces images (or pictures) of the body with the use of x-rays. The MRI uses a large magnet, radio waves, and a computer program to produce three-dimensional image of your chest that can show heart abnormalities. This test is not painful.
Cardiac catheterization: a procedure where a catheter (small tube) is inserted into your baby’s heart through a large vein or artery in the leg to take pictures and pressure measurements. There may be some soreness at the insertion site following this procedure.

Treatment for coarctation of the aorta

Treatment for coarctation of the aorta is determined by the severity of the narrowing of the aorta and the symptoms.

Infants with severe or tight narrowing of the aorta will be admitted to the hospital, either in the Neonatal Intensive Care Unit or the Pediatric Intensive Care Unit, for close monitoring for signs of poor heart function or decreased blood flow to the lower body. A continuous medication called Prostaglandin may be needed to keep the patent ductus arteriosus open and provide more blood flow to the lower body past the area of the coarctation. Repair of the severe coarctation of the aorta will be necessary before the infant is able to go home.

The coarctation of the aorta is treated with a repair of the narrowed aorta, either during a surgery or sometimes during a cardiac catheterization procedure. The specific type of repair of the coarctation will be determined by the severity of the narrowing, the cardiac symptoms, and the age of the individual.

There are several ways the coarctation can be repaired during the cardiac surgery. Most of the coarctation repair surgeries are done through an incision on the left side of the chest called a thoracotomy.

Surgical options for coarctation of the aorta include:

End-to-end anastomosis: The narrowed area of the coarctation is removed or cut out and the two ends of the aorta vessel are sewn together.
Patch aortoplasty: The narrowed area of the coarctation is opened and a patch is sewn in place to enlarge the size of the aorta.
Subclavian patch: The narrowed area of the coarctation is repaired using a blood vessel called a subclavian artery to widen the narrowed area.

A cardiac catheterization procedure is another option for repair of the coarctation in some individuals. The procedures may include a balloon dilatation (angioplasty) and/or device stent placement at the site of the narrowing.

During the cardiac catheterization, a cardiologist inserts a small catheter or flexible tube into a femoral blood vessel (near the groin area or upper leg area). The catheter is moved towards the heart and the area of the coarctation of the aorta. A catheter with a small balloon is inflated at the site of the narrowing and the area is stretched (or dilated) to increase the size of the aorta. This procedure is called a balloon angioplasty. A stent (or special device) may also be placed in the narrowed area after the balloon dilatation to keep the area opened.

Immediately after the heart surgery, your child will go to our cardiac intensive care unit for close monitoring. Once your child’s medical condition and vital signs are stable, they will transferred from the intensive care unit and continue to receive care on our cardiology floor.

Children’s Mercy has many skilled health care providers to care for your baby. The Neonatal and the Pediatric Intensive Care Units are staffed with doctors and nurses with years of experience who are able to care for the children with all levels of medical needs. The cardiology providers and cardiac surgeons are the heart experts. The cardiac providers will meet with your family and care for you child throughout the hospitalization. The cardiac providers will provide information to you family, assist with the care of your child, and continue to manage your child within the cardiac clinic.

Healthy Heart

Sometimes, it’s easier to understand how your child’s heart is different if you have a clearer picture of how a healthy heart works. Experts at the Heart Center have provided the basics to help you learn about the heart’s structure and function.

Learn how a healthy heart works

Choosing the best home for your child’s care

The Heart Center at Children’s Mercy provides comprehensive care for your child as they grow. In addition to top-ranked medical care, we will support your entire family through our Thrive program, which gives you resources and care throughout your journey.

When you’re choosing a care team for your child, it can be helpful to see how often our doctors perform certain procedures and how well children do after the surgery is over. Our surgical team performs hundreds of pediatric heart surgeries each year, often with outcomes that are better than the national average. The charts on our Cardiovascular Surgery Outcomes page help you see the number of cases we complete each year, organized by procedure, along with a numerical score that represents our outcomes—how the children did after surgery. The Society of Thoracic Surgeons (STS) score is calculated from a database of surgical outcomes from congenital heart surgery centers across North America. By comparing our numbers to the STS score, you can see how we perform compared to other surgical centers who complete the same types of surgeries.

Learn more about our Heart Center

Care for your growing child

Following your baby’s surgery, they will need close follow-up care with a cardiology provider. These visits will be spaced out as your baby grows. Even though most children lead healthy, active lives, your child will need cardiology care for their whole life.

Your child will be seen by a cardiology provider to monitor for cardiac findings to include hypertension (high blood pressure) and the possible recurrence or the development of narrowing at the site of the surgery or cardiac catheterization treatment.

Your child’s cardiology provider will continue to monitor your child’s heart during clinic appointments. Cardiac tests such as echocardiogram (heart ultrasound), electrocardiogram (heart electrical activity), Cardiac MRI, and exercise stress tests may be recommended.

Since most children will live full lives into adulthood, as they reach an appropriate age, we will help your child transition to an adult congenital heart specialist.