Atrial Septal Defect (ASD)

What is an atrial septal defect?

Anatomy of the heart, normal

An atrial septal defect 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. ASD can be a congenital (present at birth) heart defect, or it can result from the failure of normal postnatal closure of a hole that is present in the heart of every fetus.  

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, and then is pumped out to the body through the aorta.

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.

Illustration of the anatomy of a heart with an atrial septal defect

Atrial septal defects occur in a small percentage of children born with congenital heart disease. For unknown reasons, girls have atrial septal defects twice as often as boys.

Prevention & Risk Treatment

Prevention & Risk Treatment

What causes an atrial septal defect?

The heart is forming during the first eight weeks of fetal development. It begins as a hollow tube, then partitions within the tube develop that eventually become the septa (or walls) dividing the right side of the heart from the left. Atrial septal defects usually 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.

What are the types of atrial septal defects?

There are four major types of atrial septal defects:

  • Ostium secundum atrial septal defect. This is the most common atrial septal defect, affecting over two-thirds of people with atrial septal defects. It is caused when a part of the atrial septum fails to close completely while the heart is developing. This causes an opening to develop in the center of the wall separating the two atria.
  • Ostium primum atrial septal defect. This defect is part of atrioventricular canal defects, and is associated with a split (cleft) in one of the leaflets of the mitral valve.
  • Sinus venosus atrial septal defect. This defect occurs at the superior vena cava and right atrium junction, in the area where the right pulmonary veins enter the heart. As a result, the drainage of one or more of the pulmonary veins may be abnormal in that the pulmonary veins drain to the right atrium, rather than the left atrium. 
  • Coronary sinus atrial septal defect. This defect is located within the wall of the coronary sinus, where it passes behind the left atrium. The coronary sinus carries the blood flow from the heart's own vein, into the right atrium. It is the rarest of all atrial septal defects.

Why is an atrial septal defect a concern?

This heart defect can over time 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. This extra blood passes through the pulmonary artery into the lungs, causing higher amounts of blood flow than normal in the vessels in the lungs.

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 lungs are able to cope with this extra blood flow for a long period of time. In some patients, the extra blood flow eventually raises the blood pressure in the lungs, usually after several decades. This then hardens the blood vessels in the lungs, causing them to be diseased, resulting in irreversible changes in the lungs.

What are the symptoms of an atrial septal defect?

Many children have no symptoms and seem healthy. However, if the ASD is large, permitting a large amount of blood to pass through to the right side of the heart, 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. However, 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.

Diagnosis

Diagnosis

How are arrhythmias diagnosed?

In addition to a complete medical history and physical examination of your child, there are several different types of procedures that may be used to diagnose arrhythmias. Some of these procedures include the following:

  • Electrocardiogram (ECG or EKG). An electrocardiogram is a measurement of the electrical activity of the heart. By placing electrodes at specific locations on the body (chest, arms, and legs), a picture, or tracing, of the electrical activity can be obtained as the electrical activity is received and interpreted by an ECG machine. An ECG can indicate the presence of arrhythmias or other types of heart conditions. There are several variations of the ECG test, including the following:
    • Resting ECG. For this procedure, the clothing on the upper body is removed and small, sticky patches called electrodes are attached to the chest, arms, and legs. These electrodes are connected to the ECG machine by wires. The ECG machine is then started and records the heart's electrical activity for a minute or so. The child is lying down during this ECG.
    • Exercise ECG, or stress test. The child is attached to the ECG machine as described above. However, rather than lying down, the child exercises by walking on a treadmill or pedaling a stationary bicycle while the ECG is recorded. This test is done to assess changes in the ECG during stress such as exercise.
  • Holter monitor. An ECG recording done over a period of 24 or more hours. Three electrodes are attached to the child's chest and connected to a small, portable ECG recorder by lead wires. The child goes about his or her usual daily activities (except for activities such as taking a shower, swimming, or any activity causing an excessive amount of sweating which would cause the electrodes to become loose or fall off) during this procedure. There are two types of Holter monitoring, including the following:
    • Continuous recording
    • The ECG is recorded continuously during the entire testing period.
    • Event monitor, or loop recording
    • The ECG is recorded only when the patient starts the recording when symptoms are felt or when an abnormal rhythm is detected.
  • Holter monitoring may be done when an arrhythmia is suspected but not seen on a resting ECG. Arrhythmias may be short-lived in nature and not seen during the shorter recording times of the resting ECG.
  • Electrophysiologic study (EPS). An invasive test in which a small, thin tube (catheter) is inserted in a large blood vessel in the leg or arm and advanced to the heart. This gives the doctor the capability of finding the site of the arrhythmia's origin within the heart tissue, thus determining how to best treat it. Another procedure called an esophageal electrophysiologic study may be ordered where a soft, thin flexible plastic tube is inserted in the nostril and placed in the esophagus (close to the atria) to provide a more precise ECG recording.
  • Tilt table test. A test recommended for children who have frequent fainting (syncope) episodes. The test displays how the heart rate and blood pressure respond to a change in position--lying down to standing up. During this test, medication may be given intravenously to help prevent a fainting episode once the cause has been identified by the doctor.
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Matthew R.'s Story

"It is overwhelming for any parent with children of any age to have to experience something like this. Now that I am and have gone through the experience I cannot thank Children's National enough for its kindness, professionalism, and knowledge."

Read More of Matthew R.'s Story