Coarctation of the aorta is a congenital (present at birth) heart defect involving a narrowing of the aorta. The aorta is the large artery that carries oxygen-rich (red) blood from the left ventricle to the body. It is shaped like a candy cane, with the first section moving up towards the head (ascending aorta), then curving in a C-shape as smaller arteries that are attached to it carry blood to the head and arms (aortic arch). After the curve, the aorta becomes straight again, and moves downward towards the abdomen, carrying blood to the lower part of the body (descending aorta).
The narrowed segment called coarctation can occur anywhere in the aorta, but is most likely to happen in the segment just after the aortic arch. This narrowing restricts the amount of oxygen-rich (red) blood that can travel to the lower part of the body. Varying degrees of narrowing can occur.
The more severe the narrowing, the more symptomatic a child will be, and the earlier the problem will be noticed. In some cases, coarctation is noted in infancy. In others, however, it may not be noted until school-age or adolescence.
Thirty percent to 40 percent of children with coarctation of the aorta also have a bicuspid aortic valve—a valve that has two leaflets instead of the usual three.
Coarctation of the aorta occurs in about 6 percent to 8 percent of all children with congenital heart disease. Boys have the defect twice as often as girls do.
Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Most of the time this heart defect occurs sporadically (by chance), with no clear reason for its development.
Coarctation of the aorta causes several problems, including the following:
Symptoms noted in early infancy are caused by moderate to severe aortic narrowing. The following are the most common symptoms of coarctation of the aorta. However, each child may experience symptoms differently. Symptoms may include:
Mild narrowing may not cause symptoms at all. Often, a school-aged child or adolescent is simply noted to have high blood pressure or a heart murmur on a physical examination. Some may complain of headaches or cramps in the lower sections of the body.
The symptoms of coarctation of the aorta may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.
Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the obstruction in the coarctation segment of the aorta. Symptoms your child exhibits will also help with the diagnosis.
A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to your child's heart and lungs, and make other observations that help in the diagnosis. The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have. Diagnostic testing for congenital heart disease varies by the child's age, clinical condition, and institutional preferences. Some tests that may be recommended include the following:
Specific treatment for coarctation of the aorta will be determined by your child's physician based on:
Coarctation of the aorta is treated with repair of the narrowed vessel. Several options are currently available.
Some infants will be very sick, requiring care in the intensive care unit (ICU) prior to the procedure, and could possibly even need emergency repair of the coarctation. Others, who are exhibiting few symptoms, will have the repair scheduled on a less urgent basis.
After surgery, infants will return to the ICU to be closely monitored during recovery.
While your child is in the ICU, special equipment will be used to help him or her recover, and may include the following:
Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.
Your child will be kept as comfortable as possible with several different medications; some of which relieve pain, and some of which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.
After discharge from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while, and these will be explained to you. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.
Most children who have had a coarctation of the aorta surgical repair will live healthy lives. Activity levels, appetite, and growth should eventually return to normal.
Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis before major surgeries or procedures, such as dental cleaning.
As the child grows, the aorta may once again become narrow on occasion. If this happens, a balloon procedure or operation may be necessary to repair the coarctation. Evaluation with MRI is generally recommended. If an aortic aneurysm or dissection is suspected, computed tomography (CT scan) may also be performed to further evaluate the anatomy before deciding on treatment options.
Blood pressure management is very important. Often, the blood pressure in the child is elevated after aortic coarctation repair. In that case, medications may be prescribed to help lower the child's blood pressure.
Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout the individual's lifespan.
Consult your child's physician regarding the specific outlook for your child.
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