Coarctation of the aorta
Filed under: Heart & Vascular
Coarctation (ko-ahrk-TAY-shun) 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 occurs,
your heart must pump harder to force blood through the narrow part of
Coarctation of the aorta is generally present at birth (congenital).
Coarctation of the aorta may range from mild to severe, and may not be
detected until adulthood, depending on how narrowed the aorta is.
Coarctation of the aorta often occurs along with other heart defects.
While treatment for coarctation of the aorta is usually successful, it's
a condition that requires careful follow-up through infancy and
The signs and symptoms of coarctation of the aorta depend on its
seriousness. Children with serious aortic narrowing tend to show signs
and symptoms earlier in life, while mild cases may not be diagnosed
Babies with severe coarctation of the aorta usually begin having signs and symptoms shortly after birth. These include:
* Pale skin
* Heavy sweating
* Difficulty breathing
Left untreated, aortic coarctation in babies may lead to heart failure and death.
Older children and adults with the condition often don't have symptoms,
because they tend to have less severe narrowing of the aorta. If signs
or symptoms appear, the most common sign is high blood pressure
(hypertension) measured in the arm. Signs and symptoms may include:
* High blood pressure
* Shortness of breath, especially during exercise
* Muscle weakness
* Leg cramps or cold feet
When to see a doctor
Seek medical help if you or your child has the following signs or symptoms:
* Severe chest pain
* Sudden shortness of breath
* Unexplained high blood pressure
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.
Doctors aren't certain what causes aortic coarctation in most cases. For
unknown reasons, mild to severe narrowing develops in part of the
aorta. Although aortic coarctation can occur anywhere along the aorta,
the coarctation is most often located near a blood vessel called the
ductus arteriosus. The condition generally begins before birth.
Rarely, 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 narrow your aorta, leading
to aortic coarctation. In rare cases, trauma may lead to coarctation of
Coarctation of the aorta usually occurs beyond the blood vessels that
branch off to your upper body and before the blood vessels that lead to
your lower body. This often means you'll have high blood pressure in
your arms, but low blood pressure in your legs and ankles.
Coarctation of the aorta often occurs along with other congenital heart
defects, although doctors don't know what causes multiple heart defects
to form together. If you or your child has any of the following heart
conditions, you or your child is more likely to have aortic coarctation:
* Bicuspid aortic valve. The aortic valve separates the lower left
chamber (left ventricle) of the heart from the aorta. A bicuspid aortic
valve has two leaflets instead of the usual three.
* Ventricular septal defect. In this condition, there's a hole in
the wall that separates the two lower chambers (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. While a baby is still in the womb, 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 valve stenosis. This is a narrowing of the valve that
separates the left ventricle of the heart from the aorta. This means
your heart has to pump harder to get adequate blood flow to your body.
Over time, this can thicken the muscle of your heart and lead to heart
* Mitral valve stenosis. This is a narrowing of the valve that lets
blood flow through the left side of your heart. This means blood may
back up into your lungs, causing shortness of breath or lung congestion.
Like aortic valve stenosis, this condition can also lead to heart
Coarctation of the aorta is also more common in people who have certain
genetic conditions, such as Turner syndrome. Women and girls with this
syndrome have 45 chromosomes, with one missing or incomplete X
chromosome, instead of 46. About 10 percent of women and girls with
Turner syndrome have aortic coarctation.
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.
Complications of coarctation of the aorta include:
* High blood pressure
* Rupture of the aorta
* Premature coronary artery disease — narrowing of the blood vessels that supply the heart
* Weakened or bulging artery in the brain (cerebral aneurysm)
In addition, if the coarctation of the aorta is severe, your heart may
not be able to pump adequate blood to the organs of your body, resulting
in the failure of organs such as your kidneys or liver.
Preparing for your appointment
If you or your child develops signs and symptoms common to coarctation
of the aorta, call your doctor. After an initial examination, it's
likely that the doctor will refer you or your child to a doctor who
specializes in the diagnosis and treatment of heart conditions
Here's some information to help you prepare for your appointment, and what to expect from your doctor.
What you can do
* Write down any signs and symptoms you or your child has had, and for how long.
* Write down key medical information, including any other health
problems and the names of any medications you or your child is taking.
* Find a family member or friend who can come with you to the
appointment, if possible. Someone who accompanies you can help remember
what the doctor says.
* Write down the questions you want to be sure to ask your doctor.
Questions to ask the doctor at the initial appointment include:
* What is likely causing these symptoms?
* Are there any other possible causes for these symptoms?
* What tests are needed?
* Should a specialist be consulted?
Questions to ask if you are referred to a cardiologist include:
* Do I or does my child have coarctation of the aorta?
* How severe is the defect?
* Did tests reveal any other heart defects?
* What is the risk of complications from coarctation of the aorta?
* What treatment approach do you recommend?
* If you're recommending medications, what are the possible side effects?
* If you're recommending surgery, what type of procedure is most likely to be effective? Why?
* What will be involved in recovery and rehabilitation after surgery?
* How often should my child or I be seen for follow-up exams and tests?
* What signs and symptoms should I watch for at home?
* What is the long-term outlook for this condition?
* Do you recommend any dietary or activity restrictions?
* Do you recommend taking antibiotics before dental appointments or other medical procedures?
* Is it safe for a woman with coarctation of the aorta to become pregnant?
* What is the risk that my or my child's future children will have this defect?
* Should I meet with a genetic counselor?
In addition to the questions that you've prepared to ask your doctor,
don't hesitate to ask questions during your appointment at any time that
you don't understand something.
What to expect from your doctor
A doctor or cardiologist who sees you or your child for possible
coarctation of the aorta may ask a number of questions, including:
If you are the person affected:
* What are your symptoms?
* When did you first begin experiencing symptoms?
* Have your symptoms gotten worse over time?
* Do your symptoms include shortness of breath?
* Do your symptoms include headache or dizziness?
* Do your symptoms include chest pain?
* Do your symptoms include cold feet?
* Have you had any muscle-related problems, such as weakness or leg cramps?
* Have you ever fainted?
* Do you have frequent nosebleeds?
* Does exercise or physical exertion make your symptoms worse?
* Have you been diagnosed with any other medical conditions?
* What medications are you currently taking, including
over-the-counter and prescription drugs, as well as vitamins and
* Are you aware of any history of heart problems in your family?
* Do you or did you smoke? How much?
* Do you have any children?
* Are you planning to become pregnant in the future?
If your baby or child is affected:
* What are your child's symptoms?
* When did you first notice these symptoms?
* Is your child gaining weight at a normal rate?
* Does your child have any breathing problems, such as running out of breath easily or breathing rapidly?
* Does your child tire easily?
* Does your child sweat heavily?
* Does your child seem irritable?
* Do your child's symptoms include chest pain?
* Do your child's symptoms include cold feet?
* Has your child been diagnosed with any other medical conditions?
* Is your child currently taking any medications?
* Are you aware of any history of heart problems in your child's family?
* Is there a history of congenital heart defects in your child's family?
Tests 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.
Adults and older children tend to have milder cases and usually appear healthy until a doctor detects:
* High blood pressure in the arms
* A blood pressure difference between arms and legs
* A weak or delayed pulse in the legs
* A heart murmur — an abnormal whooshing sound caused by turbulent blood flow
Tests to 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. A chest X-ray may show an enlarged heart or a
narrowing in the aorta at the site of the coarctation.
* Echocardiogram. Echocardiograms use high-pitched sound waves to
produce an image of your heart. Sound waves bounce off your heart and
produce moving images that can be viewed on a video screen. An
echocardiogram may detect the location and severity of the aortic
coarctation and can show other heart defects, such as a bicuspid aortic
* Electrocardiogram (ECG). An ECG test records the electrical
activity in your heart each time it contracts. For this procedure,
patches with wires (electrodes) are placed on your chest, wrists and
ankles. The electrodes measure electrical activity, which is recorded on
paper or a computer monitor. If the coarctation of the aorta is severe,
the ECG will show that you might have a thickened heart muscle
* 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 your
body. An MRI of your chest will reveal the location of the coarctation
of the aorta and determine whether it affects other blood vessels in
* 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
Treatments and drugs
Treatment options for coarctation of the aorta depend on your age when
you're diagnosed and how narrowed your aorta is. Other heart defects may
be repaired at the same time as aortic coarctation. Treatment
approaches usually consist of surgery or a procedure called balloon
There are several open-heart surgical techniques to repair aortic
coarctation. Your doctor can discuss with you which type is most likely
to successfully repair your or your child's condition. The options
* Resection with end-to-end anastomosis. This method involves
removing the narrowed segment of the aorta (resection) followed by
connecting the two ends of the aorta together (anastomosis).
* Patch aortoplasty. Your doctor may treat your coarctation by
cutting across the constricted area of the aorta and then attaching a
patch of synthetic material 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, the blood vessel that delivers blood to your 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
portions of the aorta.
Balloon angioplasty and stenting
Balloon angioplasty is an option for initially treating aortic
coarctation or for treating re-narrowing (re-coarctation) that has
occurred after surgery. During this procedure, your doctor inserts a
thin flexible tube (catheter) into an artery in your groin and threads
it up through your blood vessels to 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
narrowed part of the aorta open.
Medication isn't used to repair coarctation of the aorta, but it 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 keep the ductus arteriosis open,
providing a bypass for blood flow around the constriction, until the
coarctation is repaired.
The most common long-term complication of coarctation of the aorta is
high blood pressure. Although your blood pressure usually falls 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, but it's possible to have additional surgeries or
procedures to correct the re-narrowing.
Lifestyle and home remedies
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 temporarily raise
your 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 of
the coarctation, 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 location for bacterial growth. Antibiotics had once been
recommended before dental procedures to prevent endocarditis. But
current guidelines suggest that antibiotics likely are helpful only for
people having major dental procedures who are most at risk of a poor
outcome from infective endocarditis. Ask your doctor about the best
approach for you.
Read this article on Mayoclinic.com.
Expand Arrow DownPrevention
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 syndrome,
another heart defect or a family history of congenital heart disease,
early detection can help. Discuss the risk of aortic coarctation with