What is coarctation of the aorta?
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.
Normal vs. Coarctation of 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. It is less common, but it is also not unusual for the diagnosis to be made in adulthood.
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. Despite this, there are many people who are not diagnosed with coarctation until they are being checked for high blood pressure as an adult.
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).
Symptoms and Causes
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, or hard-to-control high blood pressure (hypertension).
Diagnosis and Tests
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:
Management and Treatment
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, stenting, stent grafting, or hybrid repair (a combination of open surgery and stent grafts). The choice of treatment is based on the individual's overall health, the size and severity of the coarctation, associated aneurysm or valve disease, 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: https://my.clevelandclinic.org/departments/heart
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 balloon is inflated 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.
When the coarctation has an associated aneurysm, the repair should be performed with a stent graft (a cloth covered stent).
Following treatment for coarctation of the aorta, the late complications include recurrence of the coarctation (narrowed again), aneurysm (ballooning of the vessel), or pseudoaneurysm (a contained leak within the aorta wall).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 any late complications occur, evaluation and treatment at an experienced Aorta Center - that provides all types of treatments from stenting to redo surgery - is critical to achieving the best outcomes.
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.
Doctors vary in quality due to differences in training and experience; hospitals differ in the number of services available. 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 Miller Family Heart, Vascular & Thoracic Institute Outcomes.
The Center for Adult Congenital Heart Disease in the Sydell and Arnold Miller Family Heart, Vascular & Thoracic 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.
Learn more about specialized teams of doctors who treat congenital heart disease.
For patients who have recurrence of coarctation or any late complications after treatment of coarctation, our Congenital heart team works with our aorta specialists to provide you with the best options for treatment.
You may also use our MyConsult second opinion consultation using the Internet.
For younger patients with congenital heart disease:
See: About Us to learn more about the Sydell and Arnold Miller Family Heart, Vascular & Thoracic Institute.
If you need more information,click here to contact us, chat online with a nurse or call the Miller Family Heart, Vascular & Thoracic Institute Resource & Information Nurse at 216.445.9288 or toll-free at 866.289.6911. We would be happy to help you.
Becoming a Patient
Congenital heart disease is diagnosed by a murmur on a physical exam and several diagnostic tests:
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