Atrial Septal Defect

What is an atrial septal defect?

Normal Heart
(click to enlarge)
The normal heart has two sides, the left and the right, which are separated by a
muscular wall called the septum. Each side of the heart also has two parts -- an upper
chamber called an atrium, and a lower chamber called a ventricle. Atrial septal defect
(ASD), a congenital (present at birth) defect, occurs when there is an opening in the
atrial septum, or dividing wall between the two upper chambers of the heart, known as
the right and left atria.

Normally, oxygen-poor (blue) blood returns to the right atrium from the body, travels
to the right ventricle, then is pumped into the lungs where it receives oxygen.
Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left
ventricle, then is pumped out to the body through the aorta. See About the Heart and
Blood Vessels and Anatomy and Function of the Heart Valves for more information
on how the heart normally works.
An atrial septal defect allows oxygen-rich (red) blood to pass from the left atrium
through the opening in the septum, and then mix with oxygen-poor (blue) blood in the
right atrium. An ostium secundum, an opening in the middle of the atrial septum, is
the most common type of ASD.
Atrial septal defects occur in 5 to 10 percent of all children born with congenital heart
disease. For unknown reasons, girls have atrial septal defects twice as often as boys.
What causes an atrial septal defect?
Atrial septal defect (ASD)
(click to enlarge)
The heart is forming during the first eight weeks of fetal development. It begins as a
hollow tube, then partitions develop within the tube that eventually become the septa
(or walls) dividing the right side of the heart from the left. Atrial septal defects occur
when the partitioning process does not occur completely, leaving an opening in the
atrial septum.

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 atrial septal defects occur
sporadically (by chance), with no clear reason for their development.
Why is an atrial septal defect a concern?
This heart defect can cause lung problems if not repaired. When blood passes through
the ASD from the left atrium to the right atrium, a larger volume of blood than normal
must be handled by the right side of the heart, causing the right side to become
overworked and enlarged. Extra blood then passes through the pulmonary artery into
the lungs, causing higher pressure than normal in the blood vessels in the lungs, a
condition known as pulmonary hypertension.
A small opening in the atrial septum allows a small amount of blood to pass through
from the left atrium to the right atrium. A large opening allows more blood to pass
through and mix with the normal blood flow in the right heart. The larger the volume
of blood that goes to the lungs, the higher the pressure.
The lungs are able to cope with this extra pressure for a while, depending on how high
the pressure is. After a while, however, the blood vessels in the lungs become
diseased by the extra pressure. If this combination of lung disease and damage to the
right side of the heart is severe, it could lead to an inability of the heart to pump
effectively, called congestive heart failure.
What are the symptoms of an atrial septal defect?
Many children have no symptoms and seem healthy. If the ASD is large and permits a
large amount of blood to pass through to the right side of the heart, however, the right
atrium, right ventricle and lungs will become overworked, and symptoms may be
noted. The following are the most common symptoms of atrial septal defect. Each
child may experience symptoms differently. Symptoms may include:
 child tires easily when playing
 fatigue
 sweating
 rapid breathing
 shortness of breath
 poor growth
 frequent respiratory infections

The symptoms of an atrial septal defect may resemble other medical conditions or
heart problems. Always consult your child's physician for a diagnosis.
How is an atrial septal defect diagnosed?
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 opening from
the left side of the heart to the right.
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 the 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. Other tests are needed to help with the diagnosis, and
may include the following:
 Chest X-ray — A diagnostic test that uses invisible electromagnetic energy
beams to produce images of internal tissues, bones and organs onto film. With
an ASD, the heart may be enlarged because the right atrium and ventricle have
to handle larger amounts of blood flow than normal. Also, there may be
changes that take place in the lungs due to extra blood flow that can be seen on
an X-ray.
 Electrocardiogram (ECG or EKG) — A test that records the electrical
activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias)
and detects heart muscle stress.
 Echocardiogram (echo) — A procedure that evaluates the structure and
function of the heart by using sound waves, recorded on an electronic sensor,
that produce a moving picture of the heart and heart valves. An echo can show
the pattern of blood flow through the septal opening and determine how large
the opening is, as well as how much blood is passing through it.
 Cardiac Catheterization — A procedure that gives very detailed information
about the structures inside the heart. Under sedation, a small, thin, flexible
tube (catheter) is inserted into a blood vessel in the groin and guided to the
 inside of the heart. Blood pressure and oxygen measurements are taken in the
four chambers of the heart, as well as in the pulmonary artery and aorta.
Contrast dye is injected to more clearly visualize the structures inside the
heart. If the echocardiogram has provided enough information, this procedure
is often not needed to evaluate ASD.
 Cardiac Magnetic Resonance Imaging (MRI) — A non-invasive test that
uses three-dimensional imaging technology produced by magnets to accurately
determine blood flow and functioning of the heart as it is working.

What are the treatments for atrial septal defect?

Specific treatment for ASD will be determined by your child's physician based on:
 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 disease to progress
 your opinion or preference

Ostium secundum atrial septal defects may close spontaneously as a child grows.
Once an atrial septal defect is diagnosed, your child's cardiologist will evaluate your
child periodically to see if it is closing on its own. Usually, an ASD will be repaired if
it has not closed on its own by the time your child starts school, to prevent lung
problems that will develop from long-term exposure to extra blood flow. The decision
to close the ASD also may depend on the size of the defect. Treatment may include:
 Medical Management -- Many children have no symptoms and require no
medications, however some children may need to take medications to help the
heart work better, since the right side is under strain from the extra blood
passing through the ASD. Medication that may be prescribed includes the
following:
o Digoxin - a medication that helps strengthen the heart muscle, enabling
it to pump more efficiently.
o Diuretics - the body's water balance can be affected when the heart is
not working as well as it could. These medications help the kidneys
remove excess fluid from the body.

 Infection Control — Children with certain heart defects are at risk for
developing an infection of the inner surfaces of the heart known as bacterial
endocarditis. It is important that you inform all medical personnel that your
child has an ASD so they may determine if the antibiotics are necessary before
a procedure.
 Cardiac Catheterization — If the defect is large and your child develops
severe symptoms, or the defect seems to be causing injury to the aortic valve,
your child's cardiologist may recommend cardiac catheterization treatment or
surgery immediately. One recent development allows treatment through
cardiac catheterization. A patch shaped like an umbrella is closed (like a
closed umbrella) and inserted into the damaged area through a small tube,
called a catheter. The umbrella patch is then opened to cover the hole. This
method requires a small incision, avoiding the need for open-heart surgery.
 Surgical Repair — The surgical closure of an ASD is carried out through an
incision in the middle of the chest. A heart-lung machine is used to do the
 work of the heart while the heart is cooled, stopped, emptied and opened
through the right atrium. The hole in the wall between the right and left atrium
is closed with stitches if it is small, or, if too large, with a patch of thin leather-
like material called pericardium, which makes up the sac covering the heart.
The right atrium is then closed and the heart is restarted as the heart-lung
machine is withdrawn. Advancements in cardiovascular surgical repair include
minimally invasive cardiac surgery.

What is the long-term outlook after ASD surgical repair?

The majority of children who have had atrial septal defect surgical repair will live
healthy lives. Your child's cardiologist may recommend that your child take
antibiotics to prevent bacterial endocarditis for a specific time period after discharge
from the hospital.
Consult your child's physician regarding specific outlook for your child.
What is the latest research on ASD?
Complementing the Cardiovascular Program of Children's Hospital Boston is the
nation's most intensive clinical and basic research program focused on pediatric heart
disease. Children's is a world leader in opening new avenues of "translational
research," bringing laboratory advances to the bedside and doctor's office as quickly
as possible. All senior medical staff members of the Cardiovascular Program
participate in clinical research activities.

Children's Hospital Boston has pioneered interventional catheterization repair of

many types of congenital heart defects, including atrial septal defect. In many cases,
this eliminates the need for open-heart surgery, allowing less pain, reduced chance of
infection and shorter recovery time.