Coarctation of the aorta
The aorta carries blood from the heart to the vessels that supply the body with blood. If part of the aorta is narrowed, it is hard for blood to pass through the artery. This is called coarctation of the aorta. It is a type of birth defect.
The exact cause of coarctation of the aorta is unknown. It results from abnormalities in development of the aorta prior to birth.
Aortic coarctation is more common in people with certain genetic disorders, such as Turner syndrome.
Aortic coarctation is one of the more common heart conditions that are present at birth (congenital heart defects). This abnormality accounts for about 5% of all congenital heart defects. It is most often diagnosed in children or adults under age 40.
People who have this problem with their aorta may also have a weak area in the wall of blood vessels in their brain. This weakness causes the blood vessel to bulge or balloon out. This is known as a berry aneurysm. It can increase the risk for stroke.
Coarctation of the aorta may be seen with other congenital heart defects, such as:
Symptoms depend on how much blood can flow through the artery. Other heart defects may also play a role.
About half of newborns with this problem will have symptoms in the first few days of life. These can include breathing fast, problems eating, increased irritability, and increased sleepiness or becoming poorly responsive. In severe cases, the infant may develop heart failure and shock.
In milder cases, symptoms may not develop until the child has reached adolescence. Symptoms include:
There may also be no symptoms.
Exams and Tests
The health care provider will perform a physical exam and check the blood pressure and pulse in the arms and legs.
The provider will use a stethoscope to listen to the heart and check for murmurs. People with aortic coarctation often have a harsh-sounding murmur that can be heard underneath the left collar bone or from the back. Other types of murmurs may also be present.
Coarctation is often discovered during a newborn's first exam or a well-baby exam. Taking the pulse in an infant is an important part of the exam, because there may not be any other symptoms until the child is older.
Tests to diagnose this condition may include:
Both Doppler ultrasound and cardiac catheterization can be used to see if there are any differences in blood pressure in different areas of the aorta.
Most newborns with symptoms will have surgery either right after birth or soon afterward. They will first receive medicines to stabilize them.
Children who are diagnosed when they are older will also need surgery. In most cases, the symptoms are not as severe, so more time can be taken to plan for surgery.
During surgery, the narrowed part of the aorta will be removed or opened.
Sometimes, doctors will try to stretch open the narrowed part of the aorta by using a balloon that is widened inside the blood vessel. This type of procedure is called a balloon angioplasty. It may be done instead of surgery, but it has a higher rate of failure.
Older children usually need medicines to treat high blood pressure after surgery. Some will need lifelong treatment for this problem.
Coarctation of the aorta can be cured with surgery. Symptoms quickly get better after surgery.
However, there is an increased risk for death due to heart problems among those who have had their aorta repaired. Lifelong follow-up with a cardiologist is encouraged.
Without treatment, most people die before age 40. For this reason, doctors most often recommend that the person has surgery before age 10. Most of the time, surgery to fix the coarctation is done during infancy.
Narrowing or coarctation of the artery can return after surgery. This is more likely in people who had surgery as a newborn.
Complications that may occur before, during, or soon after surgery include:
Long-term complications include:
When to Contact a Medical Professional
Call your provider if:
There is no known way to prevent this disorder. However, being aware of your risk may lead to early diagnosis and treatment.
Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery: The Biological Basis of Modern Surgical Practice. 20th ed. Philadelphia, PA: Elsevier; 2017:chap 58.
Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Mann DL, Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 62.
Review Date: 12/8/2017
Reviewed By: Steven Kang, MD, Director, Cardiac Electrophysiology, Alta Bates Summit Medical Center, Stanford Healthcare, Oakland, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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