Coarctation of the aorta is a condition in which a portion of the major artery that carries oxygenated blood out of the heart becomes abnormally narrow, causing the heart to work harder.

The heart pumps oxygenated blood from the left ventricle into the aorta, distributing it throughout the body. The aorta arches upward to supply the head and arms before curving downward to deliver blood to the lower body, normally functioning without obstructions.

Coarctation of the aorta (CoA) is when the aorta narrows, typically just beyond the arteries that supply the arms and head, restricting blood flow and making the heart work harder. The exact cause is unknown but is often linked to changes in the ductus arteriosus, a fetal blood vessel that usually closes after birth, making the narrowing symptomatic.

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Symptoms

Signs of coarctation of the aorta vary with severity. Mild cases may show no symptoms. Severe narrowing in infants can cause:

  • Labored breathing
  • Difficulty feeding
  • Heavy sweating
  • Weak pulse

In older children and adults, symptoms may include:

  • Chest pain
  • High blood pressure
  • Severe headaches
  • Muscle weakness
  • Leg cramps
  • Cold feet
  • Nosebleeds

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Detecting coarctation of the aorta

Sometimes coarctation of the aorta is discovered in infants, but it can also go unnoticed until later in life.

Mild cases may go undetected until the patient is older. Outside of being discovered at birth, this condition can be detected later in life due to health complications, including:

  • Traumatic injury
  • Cholesterol buildup in the arteries, known as atherosclerosis
  • Swelling of blood vessels, known as Takayasu arteritis

Risk factors

Coarctation of the aorta can be associated with other heart defects, particularly on the left side of the heart. Problems with the aortic or mitral valves, such as a bicuspid aortic valve, are often linked with this condition. Due to these associations, anyone diagnosed with coarctation of the aorta should have a comprehensive heart evaluation.

Testing and diagnosis

Generally, the condition is diagnosed using imaging techniques, which create a visual rendering of the narrowing in the aorta. Imaging techniques can include echocardiograms (an ultrasound of the heart), MRI or CT scans, or cardiac catheterization, which measures blood pressure in different parts of the aorta.

Treatment

Most cases of aortic coarctation, especially in infants, require surgical intervention.

This standard procedure involves making an incision between the ribs to access the aorta. The narrowed segment of the aorta is then cut out, and the remaining ends are sewn together. This operation typically lasts between 2-3 hours, with the critical step of isolating the narrowed segment taking about 15-20 minutes.

Less invasive catheter-based interventions, such as balloon angioplasty, can be used, particularly in older children or adults. However, these methods are usually considered secondary to surgical repair due to their varying effectiveness and durability.

Medication

While no medication can resolve this condition, it may be used to manage symptoms.

Drugs are sometimes used to control high blood pressure or other heart-related issues. Medications are generally used along with surgical treatments to provide symptomatic relief and ensure overall better management of the condition.

World-class care, close to home

Our pediatric surgeons at WashU Medicine see patients at St.Louis Children’s Hospital, one of the top hospitals in the nation, according to U.S. News & World Report.

Recovery

Most neonates and infants don’t need additional medications after repair, but older children with pre-existing hypertension may need ongoing blood pressure management.

Lifelong follow-up with a cardiologist is crucial due to the risk of re-coarctation, with regular blood pressure checks and echocardiograms. Successful surgery generally leads to a normal, active life expectancy.

The incision wound can be painful, however medication and supplemental painkillers are typically prescribed to manage pain.

Complications

Complications from coarctation repair can include rare bleeding needing transfusion, injury to the recurrent laryngeal nerve causing temporary hoarseness, and chylothorax, a buildup of lymphatic fluid around the lungs, occurring in about 1-2 out of 100 of cases. This rare complication can possibly extend hospital stay by 3-4 weeks.