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How the heart works with an atrial septal defect

An atrial septal defect (ASD) allows freshly oxygenated blood to flow from the left upper
chamber of the heart (left atrium) into the right upper chamber of the heart (right atrium). There,
it mixes with deoxygenated blood and is pumped to the lungs, even though it's already
refreshed with oxygen.

If the atrial septal defect is large, this extra blood volume can overfill the lungs and overwork the
right side of the heart. If not treated, the right side of the heart eventually enlarges and weakens.
If this process continues, the blood pressure in lungs may increase as well, leading to
pulmonary hypertension.

Atrial septal defects can be several types, including:

Secundum. This is the most common type of ASD, and occurs in the middle of the wall between
the atria (atrial septum).
Primum. This defect occurs in the lower part of the atrial septum, and may occur with other
congenital heart problems.
Sinus venosus. This rare defect usually occurs in the upper part of the atrial septum.
Coronary sinus. In this rare defect, part of the wall between the coronary sinus — which is part
of the vein system of the heart — and the left atrium is missing.
Risk factors
It is unknown but congenital heart defects appear to run in families and sometimes occur with
other genetic problems, such as Down syndrome.

Some conditions that have or that occur during pregnancy may increase risk of having a baby
with a heart defect, including:

Rubella infection. Becoming infected with rubella (German measles) during the first few months
of your pregnancy can increase the risk of fetal heart defects.
Drug, tobacco or alcohol use, or exposure to certain substances. Use of certain medications,
tobacco, alcohol or drugs, such as cocaine, during pregnancy can harm the developing fetus.
Diabetes or lupus. If the mother is having diabetes or lupus, that may be more likely to have a
baby with a heart defect.
Obesity. Being extremely overweight (obese) may play a role in increasing the risk of having a
baby with a birth defect.
Phenylketonuria (PKU). If the mother have PKU and aren't following your PKU meal plan, it may
be more likely to have a baby with a heart defect.
Complications
A small atrial septal defect may never cause any problems. Small atrial septal defects often
close during infancy.

Larger defects can cause serious problems, including:


Right-sided heart failure
Heart rhythm abnormalities (arrhythmias)
Increased risk of a stroke
Shortened life span
Less common serious complications may include:

Pulmonary hypertension. If a large atrial septal defect goes untreated, increased blood flow to
your lungs increases the blood pressure in the lung arteries (pulmonary hypertension).
Eisenmenger syndrome. Pulmonary hypertension can cause permanent lung damage. This
complication, called Eisenmenger syndrome, usually develops over many years and occurs
uncommonly in people with large atrial septal defects.
Treatment can prevent or help manage many of these complications.

Atrial septal defect and pregnancy


Most women with an atrial septal defect can tolerate pregnancy without any problems. However,
having a larger defect or having complications such as heart failure, arrhythmias or pulmonary
hypertension can increase risk of complications during pregnancy. Doctors strongly advise
women with Eisenmenger syndrome not to become pregnant because it can endanger their
lives.

The risk of congenital heart disease is higher for children of parents with congenital heart
disease, whether in the father or the mother. Anyone with a congenital heart defect, repaired or
not, who is considering starting a family should carefully discuss it beforehand with a doctor.
Some medications may need to be stopped or adjusted before you become pregnant because
they can cause serious problems for a developing fetus.

Prevention
In most cases, atrial septal defects can't be prevented. If any mother is planning to become
pregnant, schedule a preconception visit with health care provider. This visit should include:

Getting tested for immunity to rubella. If mother is not immune, ask physician about getting
vaccinated.
Going over current health conditions and medications. Its need to carefully monitor certain
health problems during pregnancy.
Reviewing mother family medical history. If mother have a family history of heart defects or
other genetic disorders, consider talking with a genetic counselor to determine what the risk
might be before getting pregnant.
Diagnosis
Your or your child's doctor may first suspect an atrial septal defect or other heart defect during a
regular checkup if he or she hears a heart murmur while listening to the heart using a
stethoscope.
If your doctor suspects you or your child has a heart defect, the doctor may request one or more
of the following tests:

Echocardiogram. This is the most commonly used test to diagnose an atrial septal defect. Some
atrial septal defects can be found during an echocardiogram done for another reason.

During an echocardiogram, sound waves are used to produce a video image of the heart. It
allows your doctor to see your heart's chambers and measure their pumping strength.

This test also checks heart valves and looks for any signs of heart defects. Doctors may use this
test to evaluate your condition and determine your treatment plan.

Chest X-ray. An X-ray image helps your doctor to see the condition of your heart and lungs. An
X-ray may identify conditions other than a heart defect that may explain your signs or
symptoms.
Electrocardiogram (ECG). This test records the electrical activity of your heart and helps identify
heart rhythm problems.
Cardiac catheterization. In this test, a thin, flexible tube (catheter) is inserted into a blood vessel
at your groin or arm and guided to your heart. Through catheterization, doctors can diagnose
congenital heart defects, test how well your heart is pumping and check the function of your
heart valves. Using catheterization, the blood pressure in your lungs also can be measured.

However, this test usually isn't needed to diagnose an atrial septal defect. Doctors may also use
catheterization techniques to repair heart defects.

Magnetic resonance imaging (MRI). An MRI is a technique that uses a magnetic field and radio
waves to create 3-D images of your heart and other organs and tissues within your body. Your
doctor may request an MRI if echocardiography can't definitively diagnose an atrial septal
defect.
Computerized tomography (CT) scan. A CT scan uses a series of X-rays to create detailed
images of your heart. A CT scan may be used to diagnose an atrial septal defect if
echocardiography hasn't definitely diagnosed an atrial septal defect.
Treatment
Many atrial septal defects close on their own during childhood. For those that don't close, some
small atrial septal defects don't cause any problems and may not require any treatment. But
many persistent atrial septal defects eventually require surgery to be corrected.

Medical monitoring
If your child has an atrial septal defect, your child's cardiologist may recommend monitoring it for
a period of time to see if it closes on its own. Your child's doctor will decide when your child
needs treatment, depending on your child's condition and whether your child has any other
congenital heart defects.
Medications
Medications won't repair the hole, but they may be used to reduce some of the signs and
symptoms that can accompany an atrial septal defect. Drugs may also be used to reduce the
risk of complications after surgery. Medications may include those to keep the heartbeat regular
(beta blockers) or to reduce the risk of blood clots (anticoagulants).

Surgery
Many doctors recommend repairing an atrial septal defect diagnosed during childhood to
prevent complications as an adult. Doctors may recommend surgery to repair medium- to
large-sized atrial septal defects. However, surgery isn't recommended if you have severe
pulmonary hypertension because it might make the condition worse.

For adults and children, surgery involves sewing closed or patching the abnormal opening
between the atria. Doctors will evaluate your condition and determine which procedure is most
appropriate. Atrial septal defects can be repaired using two methods:

Cardiac catheterization. In this procedure, doctors insert a thin tube (catheter) into a blood
vessel in the groin and guide it to the heart using imaging techniques. Through the catheter,
doctors set a mesh patch or plug into place to close the hole. The heart tissue grows around the
mesh, permanently sealing the hole.
This type of procedure may be performed to repair only the secundum type of atrial septal
defects. Some large secundum atrial septal defects may not be able to be repaired through
cardiac catheterization, and may require open-heart surgery.

Open-heart surgery. This type of surgery is done under general anesthesia and requires the use
of a heart-lung machine. Through an incision in the chest, surgeons use patches to close the
hole. This procedure is the preferred treatment for certain types of atrial septal defects (primum,
sinus venosus and coronary sinus), and these types of atrial defects can only be repaired
through open-heart surgery.

This procedure may also be conducted using small incisions (minimally invasive surgery) for
some types of atrial septal defects.

Doctors use imaging techniques after the defect has been repaired to check the repaired area.

Follow-up care
Follow-up care depends on the type of defect and whether other defects are present. Repeated
echocardiograms are done after hospital discharge, one year later and then as requested by
your or your child's doctor. For simple atrial septal defects closed during childhood, only
occasional follow-up care is generally needed.

Adults who've had atrial septal defect repair need to be monitored throughout life to check for
complications, such as pulmonary hypertension, arrhythmias, heart failure or valve problems.
Follow-up exams are typically done on a yearly basis.

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