Coarctation of the Aorta

Coarctation of the aorta (CoA) is a congenital heart defect where there is a narrowing, or constriction, in a portion of the aorta (the major blood vessel the major blood vessel that takes blood from the heart to the rest of the body). This narrowing forces the heart to pump harder to move blood through the aorta and may restrict blood to the lower body.

About Coarctation of the Aorta

Coarctation of the aorta (CoA) is diagnosed when there is a narrowing, or constriction, in a portion of the aorta (the major blood vessel that takes blood from the heart to the rest of the body). This narrowing causes the heart to pump harder to move blood through the aorta and to the rest of the body, which may restrict blood to the lower body.

CoA often develops without discernible cause, though genetics, a chromosome abnormality, or environmental exposure may also cause coarctation of the aorta. Families who have a history of left-sided heart problems and children with specific genetic abnormalities tend to be at a higher risk. Most people who have CoA are born with it or develop it in the first few days of life as natural changes in blood circulation occur after birth. In rare cases, CoA may develop in adulthood from an injury or a condition that affects the arteries.

With a narrower aorta, the left ventricle in the heart compensates by working harder to push blood through the vessel and blood flow may be reduced. As a result of being overworked, the muscle in the left ventricle can weaken, thicken, or fail to function properly. CoA leads to higher blood pressure before the narrow portion. The greater pressure in blood vessels in the head may cause headaches and other complications in older children. Inadequate blood flow can cause cramps in the legs or abdomen and the kidneys may not produce adequate urine if they don’t receive enough blood at the right pressure. Without treatment, high blood pressure from coarctation of the aorta may weaken arteries in the head or arms, causing tears that could trigger a stroke or bleeding.

Symptoms of Coarctation of the Aorta

Children are more likely to exhibit symptoms earlier if the narrowing is more severe. While some children may show symptoms as babies, others may not have any symptoms until they are in elementary school or even during adolescence. CoA symptoms may resemble other conditions. Speak with your child’s doctor or make an appointment with a pediatric cardiologist if any abnormalities are detected.

Common symptoms of CoA may include:

• Circulatory collapse
• Cold extremities
• Heavy or rapid breathing
• Heavy sweating
• Infection in the intestines
• Irritability
• Pain in lower legs while walking
• Pale skin
• Poor growth or weight gain
• Tiredness with exercise
• Trouble feeding in infants

Diagnosing Coarctation of the Aorta

During physical examinations, your child’s doctor listens to your child’s heart and lungs and may detect a heart murmur, which are extra sounds heard throughout the cardiac cycle due to increased blood flow. If your pediatrician suspects increased blood flow, a recommendation to see a pediatric cardiologist may be made. In some cases, CoA can be detected with fetal echocardiography (ultrasound) before your baby is born. A fetal echocardiography can be performed as early as 16 weeks of gestation.

Tests performed when diagnosing CoA may include:

• Cardiac Catheterization: During cardiac catheterization, a small catheter (thin tube) is inserted into a larger blood vessel, typically in the groin, and guided to the heart where blood pressure and oxygen measurements can be taken in the aorta and pulmonary artery as well as the four chambers of the heart. A dye can also be injected through the tube to make the heart’s structure more visible on an X-ray.
• Cardiac MRI or CT Scan: A cardiac MRI or CT scan is used to take more detailed images of the heart to help define the anatomy and detect anomalies.
• Chest X-Ray: A chest X-ray produces an image of the tissue and bones in the heart and lungs and helps your provider assess the shape, size, and structure of the heart and lungs as well as the aeration of or any congestion in the lungs.
• Echocardiogram: An echocardiogram uses ultrasound technology to create a moving image of the heart and its valves, allowing your provider to assess the structure and function of the heart. An echocardiogram also helps provide information about blood flow and how well the heart is pumping blood.
• Electrocardiogram (ECG or EKG): An electrocardiogram uses electrodes that are placed on the body to record the electrical activity taking place in the heart. An ECG/EKG test helps detect abnormal rhythms, such as cardiac arrhythmias, stress on the heart, and damage to the heart muscles.

Treating Coarctation of the Aorta

Treatment plans to repair the narrowed vessel can vary based on your child’s age, health, and medical history as well as the level of CoA and the prognosis. Your pediatric cardiologist may prescribe medication to control blood pressure prior to surgery and babies may be given medication to keep the blood vessel between the pulmonary artery and the lungs open.

Treatment options for CoA may include:

• Balloon Dilation: Using the same method as cardiac catheterization, a small tube can be inserted through a blood vessel in the groin and guided to the heart. A balloon can be pushed through the tube and inflated in the aorta to stretch the narrow portion. A stent may be added after the balloon is removed to ensure the aorta stays open.
• Surgery: A pediatric heart surgeon may perform a surgical repair utilizing different methods, such as removing the narrow part, widening it with a patch or part of another artery, or bypassing the section with a plastic tube.

After resting and recuperating, your child should be able to resume sports and physical activities without any limitations. Ongoing care from a pediatric cardiologist is recommended to ensure that your child remains in good health. Even after successful treatment, CoA requires meticulous follow-up care through adulthood.

Care Team Approach

The Texas Center for Pediatric and Congenital Heart Disease, a clinical partnership between Dell Children’s Medical Center and UT Health Austin, takes a multidisciplinary approach to your child’s care. This means your child and your family will benefit from the expertise of multiple specialists across a variety of disciplines. Your care team will include pediatric cardiologists, cardiothoracic surgeons, interventional cardiologists, critical care specialists, hospitalists, anesthesiologists, perfusionists, nurses, advanced practice providers, social workers, psychologists, child life specialists, dietitians, physical and occupational therapists, pharmacists, and more, who work together to provide unparalleled care for patients every step of the way. We collaborate with our colleagues at the Dell Medical School and The University of Texas at Austin to utilize the latest research, diagnostic, and treatment techniques, allowing us to identify new therapies to improve treatment outcomes. We are committed to communicating and coordinating your child’s care with referring physicians and other partners in the community to ensure that we are providing comprehensive, whole-person care.

Learn More About Your Care Team