Congenital Heart Disease: What Is Coarctation Of The Aorta (COA)? Symptoms, Diagnosis, And Treatment

Coarctation of the aorta is a narrowing of the major artery (aorta) that carries blood from the heart to the rest of the body. Coarctation of the aorta is a congenital heart defect, which means that it is present at birth. Coarctation of the aorta negatively impacts blood flow, leading to high blood pressure and heart damage. Since the heart has to work harder to pump blood, that leads to a thickening of the aorta and damages the heart muscle.

What causes coarctation of the aorta?

The cause of this disease is not yet known known, but children with coarctation of the aorta also often have other heart defects. In rare cases, coarctation of the aorta develops later on in life due to traumatic injury or atherosclerosis, a disease characterized by severe hardening of the arteries.

What are the symptoms of coarctation of the aorta?

Symptoms of coarctation of the aorta largely depend on how severe the condition is. Thankfully, most people don’t experience symptoms. Only children with severe narrowing of the aorta tend to show signs and symptoms early on in life, while most mild patients don’t get diagnosed until they become adults. If babies have severe coarctation of the aorta, these are the signs and symptoms that may develop right after they are born:

• Having pale skin
• Being irritable
• Experiencing heavy sweating
• Having difficulty breathing
• Having difficulty feeding

If untreated, coarctation of the aorta can cause heart failure or death in babies. Generally, older children or adults with coarctation of the aorta won't develop symptoms as the narrowing is likely mild. If you do develop signs of coarctation of the aorta after infancy, these are symptoms you will likely present with:

• High blood pressure
• Headaches
• Muscle weakness
• Leg cramps or cold feet
• Frequent nose bleeds
• Chest pain

What are the risk factors associated with coarctation of the aorta?

The risk factors for coarctation of the aorta include:

• Gender is a risk factor for this defect as it is more common in males than females.
• Certain genetic conditions, such as Turner syndrome, are associated with a higher incidence of coarctation of the aorta. In fact, 10 percent of females with Turner syndrome have coarctation of the aorta.

Additionally, coarctation of the aorta often develops alongside other congenital heart defects, though it is not quite known why multiple heart defects occur at the same time. Someone with coarctation of the aorta may also have: 

• Bicuspid aortic valve
• Patent ductus arteriosus​
• A hole in the wall that separates the left and right side of the heart 
• Aortic valve stenosis
• Aortic valve regurgitation
• Mitral valve stenosis
• Mitral valve regurgitation

What complications are associated with coarctation of the aorta?

Multiple complications are associated with untreated coarctation of the aorta:

• Aortic valve that is very narrow, also known as aortic stenosis
• High blood pressure
• Stroke
• Aneurysm, an enlargement in the wall of the aorta
• A rupture or tear in the aorta
• Premature coronary artery disease, a narrowing of blood vessels on the muscles of the heart
• Heart failure
• Brain aneurysm, a weak and bulging artery in the brain
• Brain hemorrhage
• In extreme cases, kidney or other organ failure

How can I prevent coarctation of the aorta?

Unfortunately, there is nothing you can do to prevent this defect as it is present from birth. However, early detection of the disease can help. Therefore, if your child has an increased risk of congenital heart disease (for example, if they have Turner syndrome), you should discuss the risk of this defect with your doctor.

How is coarctation of the aorta diagnosed?

Similar to many other congenital heart diseases, if the condition is not severe then it often isn't diagnosed until adulthood. However, if coarctation of the aorta is severe, then it will be diagnosed during infancy. The following diagnostic tests can be administered to diagnose children with this disease:

• Echocardiogram, a technique that uses sound waves to create a moving image of the heart.
• Electrocardiogram, a technique that helps measure the heart's electrical activity when it contracts.
• Chest X-ray, which provides images of the structures located in the chest, including heart and lungs.
• Magnetic resonance imaging (MRI), a technique that uses a magnetic field to create a detailed image of the heart and blood vessels.
• CT scan, which uses X-rays to create a detailed image of the body, including the heart.
• Cardiac catheterization, a procedure in which a catheter is inserted into the artery and moved towards the heart for X-ray imaging.

How is coarctation of the aorta treated?

There are several treatments for coarctation of the aorta, depending on the patient's age and severity of their condition.

Surgery

The various surgical options include:

• Resection with end-to-end anastomosis, a technique in which the narrowed segment of the aorta is removed, and the two ends of the aorta are joined together.
• Subclavian flap aortoplasty, in which a part of the blood vessel that takes blood to your left arm is used to expand the narrowed area.
• Bypass graft repair, in which the narrowed area is “bypassed” by putting a plastic tube between these portions of the aorta.
• Patch aortoplasty, in which the narrowed area of the aorta is cut across and then a patch of synthetic material is attached to help widen the blood vessel.

Balloon angioplasty and stenting

Balloon angioplasty and stenting involves inserting a tube attached to an uninflated balloon into a blood vessel and then guiding it to the heart. Once there, the balloon is inflated to widen the aorta and enable the blood to flow more easily.