What is coarctation of the aorta?
The aorta is the main artery that carries blood from the heart to the major arteries that go to all parts of the body.
Coarctation of the aorta is a common congenital (present at birth) heart defect.
Coarctation comes from the Latin coartare, meaning "to press together." In coarctation of the aorta, the aorta is pinched in or narrowed, either in a single location or along a portion of its length. This narrowing restricts normal blood flow through the aorta.
Who is affected by coarctation of the aorta?
Coarctation of the aorta occurs in about 1 in 10,000 births and accounts for 5 to 10 percent of all congenital heart defects.
What are the long-term effects of coarctation of the aorta?
Complications can develop from untreated coarctation of the aorta, the result of long-term high blood pressure caused by the coarctation. Some of the most severe complications include stroke, early-onset coronary artery disease, and brain aneurysm or aortic rupture. If the coarctation is severe and remains untreated for a long period, kidney and liver failure can develop.
More than half of people with coarctation of that aorta also have a congenital heart valve defect (bicuspid aortic valve). Over time, the valve condition usually worsens and may require surgical valve repair or replacement. People who have valve disease are also at risk for an aortic aneurysm (weakening in the walls of the aorta that causes it to bulge out and poses a risk of rupture).
What are the symptoms of coarctation of the aorta?
Symptoms depend on the severity of the narrowing. Severe cases often are diagnosed at birth or within the first months of life. In mild cases, the individual may be symptom–free into adulthood. Symptoms in an adult may include exercise intolerance, headache, shortness of breath, chest pain, nose bleeds, cold feet or leg pain after exercise.
How is coarctation of the aorta diagnosed?
Aortic coarctation usually occurs between the site where the blood vessels branch off the aorta to the upper body and the site where the blood vessels branch to the lower body. Because of this positioning, aortic coarctation causes high blood pressure in the arms and upper body and low blood pressure in the lower body and legs. This blood pressure difference is one of the most distinguishing diagnostic factors for coarctation of the aorta. Other distinguishing clinical features include differences in the pulse at the groin and the neck and a distinctive harsh heart murmur that can be heard with a stethoscope placed over the patient’s back.
To help define the extent of the problem, a cardiologist may order several different types of imaging tests that may include:
- Electrocardiogram (ECG)
- Chest X-ray
- Chest computed tomography (CT or CAT) scan
- Magnetic resonance imaging (MRI) of the chest
- Cardiac catheterization
How is coarctation of the aorta treated?
Twenty years ago, surgery was the only treatment available for aortic coarctation. Surgery is still considered the gold standard, but today treatment options for adults with this condition also include balloon angioplasty and stenting. The choice of treatment is based on the individual’s overall health, the size and severity of the coarctation and its precise location.
An individual who is diagnosed with coarctation of the aorta should be under the care of an experienced congenital heart specialist at a major medical center. A cardiac surgeon experienced in the procedures used to treat this condition is best able to determine the optimal timing and choice of treatment.
When the coarctation is relatively small, the surgeon can remove the narrowed section of aorta and re-join the two ends. This is called an end-to-end anastamosis and is often the best surgical option to treat the condition.
Other surgical options include various types of bypass surgery in which a graft is stitched onto the aorta to divert blood around the area of the defect. In patients with valve disease, a bypass can be performed in combination with a valve repair or replacement procedure.
Coarctation of the Aorta and Endocarditis
If you have had heart surgery or angioplasty to repair a defect, your doctor will prescribe preventive antibiotics for you to take before certain medical procedures for at least 6 months after the repair procedure to reduce the risk of infective endocarditis. Your doctor can provide specific guidelines about when to take antibiotics. According to the American Heart Association, there are insufficient data to make recommendations for continuing preventive antibiotic therapy longer than 6 months.
For more information about endocarditis, visit:
Angioplasty with or without stenting
Angioplasty is another option for treating coarctation of the aorta, and at some medical centers, it is the preferred treatment. The procedure is similar to angioplasty for coronary artery disease: a long, thin tube (a catheter) with a balloon on the end of it is passed into the aorta through the blood vessels, entering at the groin. When the catheter reaches the coarctation, the congenital interventionalist inflates the balloon to expand the aorta. When the narrowed area is fully expanded, the balloon and the catheter are withdrawn.
A small metal mesh tube called a stent may be placed at the site of the coarctation to keep the aorta open after the balloon is removed. The current trend is to recommend stenting in cases of severe, long areas of coarctation and in cases when differences in blood pressure persist after angioplasty alone. Stenting appears to lower the risk for aneurysm formation and rupture of the aorta compared with balloon angioplasty alone.
Following treatment for coarctation of the aorta, there is a small risk that the problem will redevelop. After surgical treatment, the risk of recurrence is between 5 and 10 percent. After angioplasty or stenting, the risk of recurrence is between 11 and 15 percent. However, long-term data on outcomes, risks and complications associated with angioplasty and stenting are not yet available.
In the event that coarctation does recur after any treatment, angioplasty with stenting is the preferred treatment.
People who have undergone treatment for coarctation of the aorta should be under the ongoing care of a congenital heart disease specialist. Regular visits that include measurements of blood pressure and a clinical exam will be required every year. In addition, Doppler ultrasound and MRI tests should be performed at regular intervals, usually every two to five years.
People who have normal blood pressure after treatment likely will have no activity limitations. Individuals who continue to have high blood pressure may be advised to avoid certain strenuous activities or sports.
How to find a doctor if you have adult congenital heart disease
The more complex your medical problem, the greater these differences in quality become and the more they matter.
Clearly, the doctor and hospital that you choose for complex, specialized medical care will have a direct impact on how well you do. To help you make this choice, read more about our Sydell and Arnold Miller Family Heart & Vascular Institute outcomes. See: About Us to learn more about the Miller Family Heart & Vascular Institute at Cleveland Clinic.
The Center for Adult Congenital Heart Disease in the Sydell and Arnold Miller Family Heart & Vascular Institute is a specialized center involving a multi-disciplinary group of specialists, including cardiologists, cardiac surgeons and nurses from Cardiovascular Medicine, Pediatric Cardiology, Pediatric and Congenital Heart Surgery, Cardiothoracic Surgery, Diagnostic Radiology, Pulmonary, Allergy and Critical Care Medicine, and Transplantation Center, who provide a comprehensive approach to diagnosing and treating adult congenital heart disease.
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Hamdan MA, Maheshwari S, Fahey JT, Hellenbrand WE. Endovascular stents for coarctation of the aorta: initial results and intermediate-term follow-up. Journal of the American College of Cardiology. 2001 Nov 1; 38(5):1518-23.
Hassan W, Awad M, Fawzy ME, Omrani AA, Malik S, Akhras N, Shoukri M. Long-term effects of balloon angioplasty on left ventricular hypertrophy in adolescent and adult patients with native coarctation of the aorta. Up to 18 years follow-up results. Catheterization and Cardiovascular Interventions. 2007 Nov 15; 70(6):881-886.
Karl TR. Surgery is the best treatment for primary coarctation in the majority of cases. Surgery is the best treatment for primary coarctation in the majority of cases. Journal of Cardiovascular Medicine (Hagerstown). 2007 Jan; 8(1):50-6.
Koerselman J, de Vries H, Jaarsma W, Muyldermans L, Ernst JM, Plokker HW. Balloon angioplasty of coarctation of the aorta: a safe alternative for surgery in adults: immediate and mid-term results. Catheterization and Cardiovascular Interventions. 2000 May; 50(1):28-33.
Krasuski RA, Fouad-Tarazi, F. ìCoarctation of the aorta.î 923-30. Comprehensive Hypertension. Lip GYH and Hall JE, eds. Philadelphia, PA: Mosby, Inc., 2007.
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