Coarctation of the Aorta

The aorta is the largest artery in the body. It rises from the heart's left ventricle (the major chamber that pumps blood out of the heart) and is filled with oxygen-rich blood that travels throughout the body.

Coarctation of the aorta (CoA) is a narrowing of the inside of the aorta. An aortic aneurysm can develop as a complication of this condition. This abnormality is generally present from birth (congenital) and makes up between 7 and 8 percent of all congenital heart defects. The condition usually occurs along with other heart defects such as:


Symptoms of CoA usually depend on the severity of the condition. Patients with a more serious form of the condition may show signs and symptoms shortly after birth, while more mild forms of the condition may not display obvious signs or symptoms until adulthood.

When the condition is severe and diagnosed in children, symptoms may include:

  • Sudden heart failure
  • Difficulty breathing
  • Pale skin
  • Heavy sweating
  • Irritability
  • Sickly sweet-smelling breath
  • Vomiting

The symptoms of bad breath and vomiting occur due to a major rise in the acid level of the blood and tissues as the flow of blood to far parts of the body is impaired.

Symptoms in older children and adults may include:

  • High blood pressure in general or high blood pressure in the arms compared to the legs
  • A soft sound heard in the lungs, usually louder in the back. This often occurs where the defect is.
  • Shortness of breath
  • Headaches
  • Muscle weakness
  • Leg cramps
  • Pulses in the legs can be felt but are softer and delayed compared with those in the arms
  • Nosebleeds
  • Fainting
  • Chest pain

Another symptom found in older children and adults with the condition is increased blood flow through the arteries of the chest, which is visible on X-rays. This usually doesn't appear before the patient is 10 to 12 years old.

Causes and Risk Factors

The cause of CoA is unknown. Because CoA often develops along with other congenital heart defects, having one of the following conditions may increase the chances of a patient having CoA:

The condition generally develops before birth; however, in some rare cases patients may develop CoA later in life. Developing the condition later in life may arise from:

  • A traumatic injury
  • Severe hardening of the arteries (atherosclerosis)
  • Inflammation of the arteries (Takayasu's arteritis)


Diagnosis of a congenital heart defect is based on finding that the heart is not working properly as indicated by the symptoms above. After taking a medical history, a physician will perform a physical examination and order a chest X-ray and an electrocardiogram (EKG), which measures electrical action of the heart. These steps are usually enough to find a defect.

Additional tests may be done depending on the initial test results and the needs of the patient. These tests may include:

  • An echocardiogram, which uses ultrasound to check how well the heart is operating
  • Cardiac catheterization, which inserts a tube into the heart to inject dye to better see the heart structure with a special live X-ray machine
  • Angiocardiography, which injects a special substance to allow the physician a view of the heart and blood vessels

Other laboratory studies may be needed to confirm that there is a congenital heart disorder and how severe it is.

If a baby is born with bluish or purplish skin from lack of oxygen, a thorough search for causes other than congenital heart defects will be made as well. This condition is often a result of respiratory problems, including blockages in the air passages of the lung, the presence of large masses where the lung should be, or a disease that prevents oxygen getting to the blood. Additional causes may include an abnormally low body temperature, low blood sugar, disorders of the central nervous system, a lack of calcium in the blood, or an infection.


Treatment of CoA will vary from one patient to another and will depend on the how severe the condition is. Treatment options generally include medication and surgery.

Babies diagnosed with the condition will usually have surgery immediately or soon after their birth. Medication will also be used to help stabilize them so that the surgery can be done more safely. Older children and adults diagnosed with CoA generally have milder cases and surgery can be planned out over a longer period of time.

Surgery in babies will focus on removing or opening the narrow part of the aorta. If the defective portion of the aorta is small, then the surgeon will reconnect the two ends of the blood vessel, a procedure known as anastomsis. If a larger portion needs to be removed, a graft may be needed which may use either manmade or donor material.

In some cases, the medical team may try to widen that area of the aorta by inserting a thin tube into the artery that has a balloon on the end. The balloon is then inflated one or more times to widen the artery. This is known as balloon angioplasty and may be done in place of surgery to remove the defective portion of the aorta.

Medications do not correct the defect. However, they may be used to stabilize the patient before surgery or to help with remaining symptoms after surgery. These medications are often used to help control blood pressure. In babies, a medication known as prostaglandin E. may be administered to help keep a specific blood vessel (ductus arteriosus) open, rather than allowing it to close as it naturally would after birth. The ductus arteriosus is a blood vessel that allows blood coming from the heart to bypass an unborn baby's nonfunctioning lungs and go directly to the portion of the aorta that runs down to the abdomen.

Aforementioned treatment options for babies do not ally for adults. Adult patients usually are treated with a stent or a bypass graft to help improve blood flow past the CoA. In adult patients with the CoA aneurysm — a complication of CoA surgery on babies — the aneurysm is treated with a stent graft or surgical resection.

The knowledgeable and highly trained staff at the Cedars-Sinai Heart Institute will work with each patient to determine the best treatment option.