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Introduction
Coarctation of the aorta — or aortic coarctation — is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this vascular defect occurs, your heart must pump harder to force blood through the constricted segment of your aorta.
Coarctation of the aorta is a condition that's generally present at birth (congenital). It's relatively common, accounting for 5 percent to 10 percent of all congenital heart conditions, and often occurs along with other heart defects. Coarctation of the aorta may range from mild to severe. It usually requires treatment, and there are a number of options to repair the constriction. While treatment for coarctation of the aorta is usually successful, it's a condition that requires careful follow-up through infancy and into adulthood.
Signs and symptoms
The signs and symptoms of coarctation of the aorta depend on the severity of the condition. People with serious aortic narrowing tend to show signs and symptoms early in life, while mild cases may not become apparent until adulthood.
Babies with severe coarctation of the aorta usually begin having signs and symptoms shortly after birth. They may suddenly become pale, irritable and sweaty and have difficulty breathing. Left untreated, aortic coarctation in babies may lead to heart failure and death.
Older children and adults often don't have symptoms, because they tend to have more-mild cases of coarctation of the aorta. If signs or symptoms appear, the most common sign is high blood pressure (hypertension) measured in the arm. Other signs and symptoms may include:
- Shortness of breath, especially during exercise
- Headache
- Muscle weakness
- Leg cramps or cold feet
- Nosebleeds
Causes
Coarctation of the aorta generally occurs during fetal growth. Although aortic coarctation can occur anywhere along the length of the aorta, most people with the condition have what's called "juxtaductal" coarctation. This means that the coarctation is located near a blood vessel, called the ductus arteriosus, which connects the left pulmonary artery to the aorta. Shortly after birth, the ductus arteriosus closes. While it's not certain what causes coarctation of the aorta, it's possible that some of the tissue from the ductus arteriosus migrates into the aortic wall during fetal development. As the ductus arteriosis closes, it may cause the aorta to narrow.
Rarely, a coarctation of the aorta may develop later in life. Severe hardening of the arteries (atherosclerosis) or a condition causing inflamed arteries (Takayasu's arteritis) may lead to aortic coarctation.
Coarctation of the aorta is most commonly located just beyond the blood vessels branching off the aorta to the upper body and before the blood vessels leading to the lower body, elevating blood flow before the constriction and reducing blood flow after the constriction. This often results in high blood pressure in the upper body and arms and low blood pressure in the lower body and legs.
Risk factors
Coarctation of the aorta is more common in people who have certain genetic conditions, such as Turner's syndrome. People with this syndrome have 45 chromosomes, including one missing or incomplete X chromosome, instead of 46. Up to one in 10 people with Turner's syndrome have aortic coarctation.
Coarctation of the aorta often occurs along with other heart defects, although it's not certain what causes multiple heart defects to form together in a developing fetus. People with the following heart conditions are more likely to have aortic coarctation:
- Bicuspid aortic valve. The aortic valve separates the left ventricle of the heart from the aorta. A bicuspid aortic valve has two leaflets instead of three.
- Ventricular septal defect. In this condition, there's a hole in the wall that separates the two ventricles of the heart. Oxygen-poor blood from the right ventricle mixes with oxygen-rich blood from the left ventricle, which pumps blood out to the body.
- Patent ductus arteriosus. In a developing fetus, the ductus arteriosus is a blood vessel connecting the left pulmonary artery to the aorta, allowing blood to bypass the lungs. Shortly after birth, the ductus arteriosus usually closes. If it remains open, it's called a patent ductus arteriosus.
- Aortic or mitral valve stenosis. This is a narrowing of the valve that separates the left ventricle of the heart from the aorta (aortic stenosis) or the left atrium from the left ventricle (mitral stenosis).
When to seek medical advice
If you have signs or symptoms, such as severe chest pain, fainting or sudden onset of shortness of breath, contact your doctor or call 911. Unexplained high blood pressure or high blood pressure that is difficult to control may also be a sign of coarctation of the aorta.
While experiencing these signs or symptoms doesn't necessarily mean that you have a serious problem, it's best to get checked out quickly. Early detection and treatment may help save your life.
Screening and diagnosis
The age at which people with coarctation of the aorta are diagnosed depends on the severity of the condition. If the aortic coarctation is severe, it's usually diagnosed during infancy. Older people tend to have milder cases and usually appear healthy until a doctor detects the following:
- High blood pressure in your arms
- Blood pressure difference between your arms and legs
- Weak or delayed pulse in your legs
- Heart murmur (abnormal whooshing sound caused by turbulent blood flow)
Diagnostic tests
Tests to help confirm a diagnosis of coarctation of the aorta include:
- Chest X-ray. X-rays produce pictures by passing an X-ray beam through your body, which is absorbed in different amounts by body structures. A chest X-ray may show an enlarged heart or an indention in the aorta at the site of the coarctation.
- Echocardiogram. Echocardiograms use high-pitched sound waves to produce an image of the heart. Sound waves bounce off your heart and produce moving images that can be viewed on a video screen. An echocardiogram may help detect the location and severity of the aortic coarctation and can assess for other heart defects, such as a bicuspid aortic valve.
- Electrocardiogram. An electrocardiogram records the electrical activity in your heart each time it contracts. During this procedure, patches with wires (electrodes) are placed on your chest, wrists and ankles. The electrodes measure electrical activity, which is recorded on paper. If the coarctation of the aorta is severe, the electrocardiogram will demonstrate evidence of a thickened heart muscle (ventricular hypertrophy).
- Magnetic resonance imaging (MRI). An MRI scan is a test that uses a magnetic field and pulses of radio wave energy to make pictures of the body. Contrast material may be injected into a vein to enhance the pictures. An MRI of the chest will reveal the location of the coarctation of the aorta and determine whether it affects other blood vessels.
- Cardiac catheterization. During this procedure, your doctor inserts a thin flexible tube (catheter) into an artery or vein in your groin and threads it up to your heart. A dye is injected through the catheter to make your heart structures visible on X-ray pictures. Cardiac catheterization helps determine the severity of the aortic coarctation.
Complications
Untreated coarctation of the aorta frequently leads to complications. Several of the complications are a result of long-standing high blood pressure caused by the aortic coarctation. These include:
- Stroke
- Rupture of the aorta
- Premature coronary artery disease — narrowing of the blood vessels that supply the heart
- Cerebral aneurysm — weakened or bulging artery in the brain
In addition, if the coarctation of the aorta is severe, the heart may not be able to pump adequate blood to the organs of the body, resulting in the failure of organs such as the kidneys or liver.
Treatment
Treatment options for coarctation of the aorta depend on your age when the condition is diagnosed and the degree to which the aorta is narrowed. Other heart defects may be repaired at the same time as aortic coarctation. Treatment approaches usually consist of surgery or a procedure called balloon angioplasty.
Surgery
There are several surgical techniques to repair aortic coarctation. Your doctor can discuss with you which type is most likely to successfully repair your condition. The options include:
- Resection with end-to-end anastamosis. This method involves removing the narrowed segment of the aorta (resection) followed by connecting the two ends of the aorta together (anastamosis).
- Patch aortoplasty. Your doctor may treat your coarctation by cutting across the constricted area of the aorta and then attaching a synthetic patch to widen the blood vessel. Patch aortoplasty is useful if the coarctation involves a long segment of the aorta.
- Left subclavian flap angioplasty. A portion of the left subclavian artery, which delivers blood to the left arm, may be used to expand the narrowed area of the artery.
- Bypass graft repair. This technique involves bypassing the narrowed area by inserting a plastic tube called a graft between the ascending and descending portions of the aorta.
Balloon angioplasty and stenting
Balloon angioplasty is an option for treating aortic coarctation or for treating re-narrowing (re-coarctation) that has recurred after surgery. During this procedure, your doctor inserts a thin flexible tube (catheter) into an artery in your groin and threads it up toward your 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. In some cases, a mesh-covered hollow tube called a stent is inserted to keep the constriction in the aorta open.
Medication
Medication isn't used to repair coarctation of the aorta, but may be used to control blood pressure before surgery. Although repairing aortic coarctation improves blood pressure, many people will still need to take medication even after a successful surgery. Babies with severe coarctation of the aorta may receive a drug called prostaglandin E. This drug helps keeps the ductus arteriosis open, providing a bypass for blood flow around the constriction, until the coarctation is repaired.
After treatment
The most common long-term complication of coarctation of the aorta is high blood pressure. Although your blood pressure usually lowers after the aortic coarctation has been repaired, it may still remain higher than normal. Occasionally, the segment of the aorta that has been repaired will become weak and bulge (aortic aneurysm) and may eventually rupture. In some cases, the coarctation will recur, possibly even years after treatment.
Prevention
Coarctation of the aorta can't be prevented, because it's usually present at birth. However, if you or your child has a condition that increases the risk of aortic coarctation, such as Turner's syndrome, another heart defect, or a family history of congenital heart disease, discuss your risk of aortic coarctation with your doctor.
Self-care
Although coarctation of the aorta may be repaired, the condition requires careful follow-up throughout adulthood to help prevent complications and to monitor for recurrences. Here are a few tips for managing your condition:
- Get regular exercise. Regular exercise helps to lower blood pressure. Talk to your doctor about whether you need to restrict certain physical activities, such as weightlifting, which may place a strain on your heart because it raises blood pressure.
- Consider pregnancy carefully. Before becoming pregnant, talk to your doctor to determine if you can undergo pregnancy safely. Women with aortic aneurysms due to coarctation of the aorta, even after repair, are at high risk of aortic rupture during pregnancy and delivery. If you have had aortic coarctation repair and are considering becoming pregnant, careful management of your blood pressure is important to help keep you and your baby healthy.
- Prevent endocarditis. Endocarditis is an inflammation of the inner lining of the heart or of its structures, caused by a bacterial infection. People with coarctation of the aorta are at risk of this infection because the abnormal tissue at the site of the coarctation is a good habitat for bacterial growth. Talk to your doctor about taking antibiotics before certain dental and medical procedures to reduce the risk of infection.
