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Coarctation of the aorta
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Coarctation of the Aorta
What is Coarctation of the aorta?
When the heart is functioning normally, a large artery called the aorta 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 toward 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 toward the abdomen, carrying blood to the lower part of the body
(descending aorta).
Coarctation of the aorta, a congenital (present at birth) defect, occurs when the
aorta narrows or becomes pinched. 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 symptoms a child will experience, 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.
Seventy-five 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 to 8 percent of all children with congenital
heart disease. Boys have the defect twice as often as girls do.
What causes coarctation of the aorta?
Coarctation of the aorta occurs due to improper development of the aorta in the first
eight weeks of fetal growth.
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 by chance, with no clear reason for its
development.
Why is coarctation a concern?
Coarctation of the aorta causes several problems, including the following:
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The left ventricle has to work harder to move blood through the narrowing in
the aorta. Eventually, the left ventricle is no longer able to handle the extra
workload, and it fails to pump blood to the body efficiently. This could lead to
a serious condition called congestive heart failure.
Blood pressure is higher above the narrowing, and lower below the narrowing.
Older children may have headaches from too much pressure in the vessels in
the head, or cramps in the legs or abdomen from too little blood flow in that
region. Also, the kidneys may not make enough urine since they require a
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certain amount of blood flow and a certain blood pressure to perform this
task.
The walls of the ascending aorta, the aortic arch, or any of the arteries in the
head and arms may become weakened by high pressure. Spontaneous tears
in any of these arteries can occur, which can cause a stroke or uncontrollable
bleeding.
There is a higher than average chance of developing an infection in the lining
of the heart or aorta known as bacterial endocarditis.
The coronary arteries, which supply oxygen-rich (red) blood to the heart
muscle, may narrow in response to elevated pressure.
The blood pressure in the pulmonary artery may rise above normal levels, a
condition called pulmonary hypertension.
What are the symptoms of coarctation of the aorta? 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. Each child may experience
symptoms differently. Symptoms may include:
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irritability
pale skin
sweating
heavy and/or rapid breathing
poor feeding
poor weight gain
Mild narrowing may not cause symptoms. 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.
What are the treatments for coarctation of the aorta?
Specific treatment for coarctation of the aorta will be determined by your child's
physician based on:
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your child's age, overall health and medical history
extent of the disease
your child's tolerance for specific medications, procedures or therapies
how your child's doctor expects the condition to progress
your opinion or preference
Coarctation of the aorta is treated with repair of the narrowed vessel. Your child's
coarctation of the aorta may be repaired through a cardiac catheterization procedure
or through surgery in an operating room:
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Cardiac Catheterization — During the procedure, the child is sedated and a
small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin
and guided to the inside of the heart. Once the catheter is in the heart, the
cardiologist will pass an inflated balloon through the narrowed section of the
aorta to stretch the area open. A small device, called a stent, may also be
placed in the narrowed area after the balloon dilation to keep the aorta open.
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Surgical Repair — The surgical repair of aortic coarctation is done through an
incision on the left chest below the armpit. The ribs are spread, the lung
pushed aside, and the aorta exposed near the heart. The aorta is clamped on
either side of the narrowing and the narrow segment with the adjacent ductus
arteriosus is cut out. The two ends of aorta are then sewn together, clamps
are removed, and flow through the aorta is reestablished.
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.
What is the long-term outlook after coarctation of the aorta surgical repair?
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.
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.
Your child's cardiologist may recommend that antibiotics be given to prevent
bacterial endocarditis after discharge from the hospital.
Source: Children’s Hospital, Boston Cardiology Website, Accessible at:
http://www.childrenshospital.org/az/Site520/printerfriendlypageS520P0.html
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