Coarctation of the Aorta

Overview

Coarctation (ko-ahrk-TAY-shun) of the aorta is a congenital (present at birth) heart defect that causes narrowing of the main blood vessel that comes out of the heart (aorta). This heart defect occurs when a baby’s aorta does not form correctly as it grows and develops during pregnancy. The damage can range from mild to severe and may not be detected until adulthood, depending on how severe the symptoms are.

Coarctation of the aorta often occurs along with other heart defects. Treatment usually is successful, but careful follow-up during infancy and throughout adulthood is always recommended.

The US Centers for Disease Control and Prevention (CDC) estimates that 4 out of every 10,000 babies are born with coarctation of the aorta. 

Causes of Coarctation of the Aorta

Causes of Coarctation of the Aorta

Congenital heart defects, such as coarctation of the aorta, are the result of problems that occurred early in the heart’s development. The defect forms while your baby’s heart is developing in the womb, but, like most congenital heart defects, there is no known cause.

In rare cases, coarctation of the aorta can develop later in life due to traumatic injury.

Severe hardening of the arteries (atherosclerosis) or a condition causing inflamed arteries (Takayasu’s arteritis) also may narrow the aorta, leading to aortic coarctation.

Coarctation of the aorta usually occurs in a spot beyond where the blood vessels branch off to the upper body. This often leads to high blood pressure in the arms, but low blood pressure in the legs and ankles.

 

Symptoms of Coarctation of the Aorta

Symptoms of Coarctation of the Aorta

Symptoms depend on the seriousness of the condition. Children with severe narrowing of the aorta most often show symptoms early in life, while mild cases may not be diagnosed until adulthood. Left untreated, aortic coarctation in babies may lead to heart failure and death.

Symptoms of severe coarctation of the aorta include:

  • Pale skin
  • Irritability
  • Heavy sweating
  • Difficulty breathing
  • Feeding difficulties

In some infants, having difficulty feeding can increase the risk of developing other conditions, including necrotizing enterocolitis, a serious intestinal disease that happens when tissue in the small or large intestine is injured or begins to die off. 

If signs or symptoms appear later in life, the most common sign is high blood pressure (hypertension). Other symptoms may include:

  • Heart murmur 
  • Shortness of breath, especially during exercise
  • Headache
  • Muscle weakness
  • Leg cramps or cold feet
  • Nosebleeds
Diagnosis and Treatment Options

Diagnosis and Treatment Options

During a routine physical exam, a doctor will check the blood pressure and pulse in the arms and legs. Symptoms that your doctor may be able to find during this exam include:  

  • Weaker pulse in the groin area or feet than in the arms or neck
  • Weaker blood pressure in your baby’s legs than in his/her arms
  • A harsh-sounding heart murmur that can be heard from the back 

Coarctation of the aorta is usually confirmed with an echocardiogram (echo). Occasionally other tests may be ordered, such as chest x-ray, computed tomography (CT) scan, electrocardiogram (EKG), pulse oximetry, cardiac catheterization, or magnetic resonance imaging (MRI).

Coarctation of the aorta angiographyResults of an angiography showing coarctation of aorta 

For more information on these tests, visit our common diagnostic tests page.

Surgery, performed by a cardiothoracic surgeon, is most often required to fix your child’s aorta, but non-surgical procedures also may be an option for some children. Most symptomatic newborns will have surgery very shortly after they are born. Children diagnosed when they are older also will need surgery, but because the symptoms are usually less severe, more time can be taken to plan for surgery.

Be sure to speak with your child’s doctor about what procedure is right for him or her and to get more information on what you should expect during and after surgery. You can print these sample questions to use as a basis for discussion with the doctor.

Surgery

Depending on the location and extent of the coarctation, your child’s cardiothoracic surgeon can approach it from the front of the chest (sternotomy) or from the left side of the chest (thoracotomy). Surgical techniques to repair aortic coarctation include:

Resection with end-to-end anastomosis – Removes the narrowed segment of the aorta (resection) and connects the two ends of the aorta (anastomosis)

Patch aortoplasty – Cuts across the constricted area of the aorta and then attaches a patch of synthetic material or a portion of the blood vessel that delivers blood to your left arm (left subclavian artery) to widen the blood vessel

Resection and interposition graft repair – Usually undertaken in older children and adults with coarctation - removes the narrowed segment of the aorta (resection). Then attaches a synthetic tube (graft) between the cut ends of the aorta

Percutaneous Coronary Intervention

Percutaneous coronary intervention (sometimes called balloon angioplasty) also is an option for treating your child’s heart defect. This procedure involves a thin flexible tube (catheter) that is inserted into your child’s artery through the groin and is threaded up through the blood vessels to the heart. An uninflated balloon is placed through the opening of the narrowed aorta. When the balloon is inflated, the aorta widens and blood flows more easily. The heart specialist may elect to leave a metal stent in the area of narrowing which will keep the aorta open after the balloon is deflated. 

Recovery

Recovery

Your child will continue to be followed by a heart specialist.

Child at follow-up appointment

The most common long-term complication of coarctation of the aorta is high blood pressure. Although blood pressure usually falls after the defect has been repaired, it may still remain higher than normal.

High blood pressure could result in the long-term use of medication, but be sure to speak with your child’s heart specialist about the need for continued medication.  

In some cases, the portion of the aorta that was repaired can re-narrow, possibly even years after treatment. This can be corrected by percutaneous intervention or, sometimes, with additional surgeries. Rarely, the segment of the aorta that has been repaired can become weak and bulge (aortic aneurysm) and may eventually rupture. 

Reviewed by: Ram Kumar Subramanyan, MD, PhD, and Lauren Kane, MD 
April 2015