What is Coarctation of the Aorta?

What is Coarctation of the Aorta?

Coarctation of the aorta (COA) is a congenital heart defect that occurs when the aorta artery begins to narrow. The aorta is the largest artery that carries blood from the heart to the rest of the body. Coarctation is a birth defect with an unknown cause. The abnormalities causing this disorder happen during fetal development. The narrowing, or coarctation, of the aorta depletes the body’s oxygen.

Coarctation of the aorta can cause the walls of blood vessels in the brain to weaken. This weakening could produce a bulge in a blood vessel. If the bulge in the blood vessel bursts this bleeding may cause a stroke.

Aortic coarctation is one of the most common congenital heart defects. Coarctation of the aorta frequently coappears with other types of genetic disorders. People with Turner syndrome, a chromosomal genetic disorder only affecting females, are many times also diagnosed with coarctation of the aorta. Diagnosis of aortic coarctation usually happens in children or adults under the age of 40.

Other congenital heart defects that appear with coarctation of the aorta are:

  • Bicuspid aortic valve
  • Aortic stenosis
  • Ventricular septal defect
  • Patent ductus arteriosus

Stages of Coarctation of Aorta

Stages

Classification for aortic coarctation is usually determined by how much narrowing is present in the aorta. These classifications depend on the proximity of the ductus arteriosus; this blood vessel is present in a developing fetus and connects the pulmonary artery to the descending aorta. The ductus arteriosus allows blood from the right ventricle to bypass the fetus’s fluid-filled, non-functioning lungs; this blood vessel closes after birth.

Aortic coarctation is classified as preductal coarctation, ductal coarctation, and postductal coarctation.

  • Preductal coarctation. Preductal coarctation occurs during fetal development causing a decrease in blood flowing through the left side of the heart due to the narrowing of the aorta. This condition is present in about 5% of infants diagnosed with Turner syndrome.
  • Ductal coarctation. This kind of narrowing occurs at the ductus arteriosus—the area connecting the pulmonary artery to the aortic artery. This usually appears once the ductus arteriosus closes.
  • Postductal coarctation. This narrowing is distal or located away from the point of connection at the ductus arteriosus. Even if the ductus arteriosus has not closed yet, blood flow to the lower body’s extremities can be impaired. Postductal coarctation is serious and infants born with this type of coarctation are critically ill. This condition is attributed to muscular ductal extension into the aorta during fetal development.

Signs & Symptoms of Coarctation of the Aorta

Symptoms

The extent of narrowing associated with the aorta and how soon the symptoms appear will determine the severity of the condition. A pediatric cardiologist can establish how serious the coarctation is and what treatments will bring the most positive results.

Common symptoms of coarctation of the aorta in infants include:

  • Difficulty breathing
  • Weak femoral artery pulse
  • Excessive sweating
  • Decreased appetite
  • Poor weight gain
  • Pale skin coloring
  • Detection of a heart murmur
  • Cold hands and feet

In adults, additional symptoms of coarctation commonly include:

  • Chest pain
  • Headaches
  • High blood pressure
  • Leg cramps & muscle weakness
  • Cold hands and feet
  • Frequent nose bleeds

Causes of Aortic Coarctation

Causes

The exact cause of aortic coarctation is unknown. However, doctors agree this condition is a critical congenital heart defect affecting the formation of a baby’s aorta during fetal development. Coarctation of the aorta is usually present at birth and rarely develops later in life.

Aortic coarctation and other heart defects are sometimes caused by chromosomal changes that take place during a baby’s development in the womb. It is believed to be a combination of genes and environmental factors the mother may have been exposed to during pregnancy.

Certain medications, alcohol consumption, and environmental toxins can alter the chromosomes during fetal development. Some congenital heart defects are genetic and therefore, associated with a family history of heart conditions.

Risk Factors

Risk Factors

There are no definitive known causes for aortic coarctation; however, certain genetic, environmental, or behavioral conditions could increase the risk of acquiring this disorder. Doctors believe the anomalies that affect the coarctation of the aorta are formed during fetal development.

Some risk factors commonly associated with coarctation of the aorta include:

  • Genetic disorders. It is reported that patients with Turner syndrome have a 10 to 25% chance of developing coarctation of the aorta
  • Sex. Males are more likely to be affected by this congenital heart condition
  • Infections during pregnancy. Mothers who develop viral infections while pregnant have an increased risk of a baby developing coarctation of the aorta or other heart defects
  • Congenital heart disease. Infants born with certain congenital heart disorders are at increased risk for developing aortic coarctation. These conditions include:
    • Ventricular septal defect
    • Patent ductus arteriosus
    • Mitral valve stenosis
    • Aortic valve stenosis
    • Bicuspid aortic valve

Complications

Complications

Aortic coarctation is a serious condition requiring immediate attention to avoid complications. Treatment should begin as soon as a diagnosis of coarctation of the aorta is determined. Patients with aortic coarctation frequently experience narrowing and plaques in the coronary arteries; these symptoms can lead to a heart attack or stroke.

An aortic aneurysm could develop, making the aorta enlarged and weak because of prolonged high blood pressure or stitches from previous surgeries. A heart attack or stroke could also occur if the weakened aorta ruptures.

Complications of aorta coarctation may still happen despite surgical and interventional cardiac catheterization-based therapies. These complications can include:

  • Left ventricular hypertrophy. Narrowing of the aorta eventually causes the left ventricle muscle to thicken; blood is not being pumped properly. Heart failure or abnormal heart rhythms can develop.
  • Recoarctation of the aorta. Even after surgery or other interventional therapies, the abnormalities associated with the aortic tissue of patients may continue to narrow
  • High blood pressure. Elevated blood pressure can become a chronic problem
  • Early coronary artery disease. Narrowing of the coronary arteries reduces the amount of oxygen the heart receives. This can lead to a heart attack or stroke.
  • Aortic aneurysm. Prolonged high blood pressure or previous surgeries can weaken the aorta. If the aorta ruptures, a stroke or heart attack can occur.
  • Berry aneurysm. An aneurysm can develop in the brain because of prolonged high blood pressure. If it ruptures, a stroke or death from a heart attack can occur.
  • Subacute bacterial endocarditis (SBE). Disruption of blood flow within the heart puts a person with coarctation of the aorta at risk for developing a bacterial infection of the heart that enters the bloodstream
Get Care

Trust NewYork-Presbyterian for Aortic Coarctation Care

NewYork-Presbyterian knows that little hearts need big minds. Our expert pediatric cardiologists and pediatric health care professionals at The Pediatric Interventional Cardiology program operating across NewYork-Presbyterian Morgan Stanley Children’s Hospital and NewYork-Presbyterian Komansky Children’s Hospital work tirelessly to diagnose and treat children’s heart conditions.

The interventional cardiologists at NewYork-Presbyterian have developed pediatric interventional cardiology procedures that are used throughout pediatric cardiology programs worldwide. Our specialized pediatric cardiology centers are equipped with the most advanced imaging technologies. This equipment is designed to observe the heart’s structure and functions, detecting any abnormalities causing heart problems for your child.

Learn about the treatment options for the repair of coarctation of aorta available to your child. Let NewYork-Presbyterian continue to keep you and your child amazing.