Also known as the Rashkind procedure, a balloon atrial septostomy is a catheter-based procedure that widens an existing hole in the wall (septum) between the two upper chambers of the heart (atria). This hole, which can be present in infants at birth, is called an atrial septal defect (ASD). By widening the defect, oxygen-rich and oxygen-poor blood can mix more freely.
Under normal circumstances, oxygen-rich and oxygen-poor blood should not mix. However, babies born with certain heart defects do not receive enough oxygen-rich blood. As a result, the infant tends to have a bluish tint (cyanosis) to the skin, lips, fingernails and other parts of the body. This condition is often called blue baby. A successful balloon atrial septostomy is a nonsurgical procedure that widens the ASD in order to improve the availability and circulation of oxygen-rich blood until all the heart defects present can be surgically repaired. As a result of the balloon atrial septostomy, the baby’s color returns to a more natural, healthier tone.
About balloon atrial septostomy
Also known as the Rashkind procedure, a balloon atrial septostomy is a catheter-based procedure that widens a heart defect called an atrial septal defect (ASD), which is a hole between the two upper chambers of the heart (atria). An ASD is a congenital heart defect, meaning it is present in babies at birth. The procedure is performed when the infant also has one or more additional heart defects that result in abnormal circulation throughout the heart. In these cases, the ASD is often necessary for life because it provides a pathway for oxygenated blood to be pumped out to the body. Thus, the Rashkind procedure is a palliative procedure, or a temporary solution to improve circulation until underlying cardiac defects can be repaired.
When a balloon atrial septostomy is successful, the baby will no longer have a bluish tint (cyanosis) to its skin, lips, fingernails and other body parts, a condition often called blue baby.
In general, a balloon atrial septostomy is performed on infants who are 2 or 3 weeks old when they have been diagnosed with one or more congenital heart defects, such as:
Transposition of the great arteries. A heart defect in which the aorta (the main artery carrying oxygen-rich blood to the body) and the pulmonary artery (the main artery carrying oxygen-poor blood to the lungs) are in reversed positions.
Total anomalous pulmonary venous return (TAPVR). A heart defect in which the pulmonary veins between the lungs and the heart are not connected properly. As a result, oxygen-rich blood returning from the lungs to the heart is detoured through the bad connection and mixed with oxygen-poor blood coming back to the heart from the rest of the body.
Double outlet right ventricle. In this defect, both the great vessels (e.g., aorta and pulmonary artery) arise from the right ventricle.
Pulmonary valve disease. Any disease that causes significant obstruction of the pulmonary valve, which lies between the right ventricle and the pulmonary artery. For example, pulmonary atresia, in which the leaflets of the pulmonary valve are fused, might require a balloon atrial septostomy.
A balloon atrial septostomy may not be performed on infants over the age of 1 to 2 months because the muscular wall (septum) between the atria may have become too thick for the balloon. For these infants, another procedure called blade atrial septostomy may be used to enlarge the tiny hole in the septum between the atria. The physician may sometimes need to perform a septostomy to create a hole in the first place to improve cardiac circulation until the other heart defect(s) can be repaired.
History of balloon atrial septostomy
First performed by Dr. William Rashkind in 1966, balloon atrial septostomy was the first catheter-based procedure to prove useful in the survival rate of infants with congenital heart disease. Rashkind was treating a newborn infant diagnosed with transposition of the great arteries (TGA). Much of the blood circulating through the patient’s body was oxygen-poor, resulting in a bluish tint (cyanosis) to the skin, lips, fingernails and other areas of the body. This condition is often called blue baby.
Rashkind discovered that the baby also had a small atrial septal defect (ASD) through which life-sustaining, oxygen-rich blood was mixing with oxygen-poor blood and being circulated throughout the body. Rashkind correctly predicted that he could improve the patient’s condition by using a balloon-tipped catheter to widen the ASD and increase the volume of oxygen-rich blood being pumped into the body. He inserted the catheter through a blood vessel in the baby’s body and guided it all the way to the ASD. Once the catheter was in place, he inflated the balloon at the tip of the catheter to widen the ASD and then withdrew the balloon. The procedure successfully stabilized the patient until surgery could be performed to repair the TGA. Since that initial procedure, thousands have been performed around the world.
Before, during and after
A parent or legal guardian will need to sign a consent form that authorizes the procedure. Immediately before the procedure, the infant may need to be restrained for his or her own safety because too much movement during the procedure could complicate the process or injure the infant. Parents should be prepared to see their infant crying and resisting the procedure.
The balloon atrial septostomy is a catheter-based procedure. During the procedure, the area where the catheter is to be inserted is first numbed with a local anesthetic. The physician will then make a cut (incision) at that area. Guided by images created by a fluoroscope (a type of real-time x-ray), the physician inserts the catheter through the blood vessel and guides it all the way up to the atrial septal defect in the left atrium. A balloon at the tip of the catheter is then inflated to a diameter of about 15 millimeters and then pulled through the ASD and into the right atrium. The catheter is then withdrawn and the procedure is complete.
With the septal hole now enlarged, the infant’s cyanosis should be partially relieved, and the patient should be able to receive enough oxygen until surgical repair of the other congenital heart defects. The enlarged atrial septal defect will also be closed when it is no longer necessary.
Potential risks with balloon atrial septostomy
The ideal result of a balloon septostomy is a wider atrial septal defect (ASD) that improves circulation until all the heart defects present can be surgically repaired. As a result, the baby’s color returns to a healthier tone. Balloon septostomy is usually a safe procedure, and has been made even more reliable in recent years with advancements in catheter design and techniques. However, there are several complications that very rarely occur:
Premature and abnormal heart rhythms (arrhythmias), such as atrial flutter or atrial fibrillation.
A balloon-fragment embolism, in which the balloon breaks apart and pieces of it travel through the bloodstream and become lodged in a blood vessel. Depending on the size of the fragment and where it is lodged, this could lead to serious consequences such as heart attack or stroke.
Treating pulmonary hypertension
Balloon atrial septostomy is a procedure usually associated only with treating congenital heart defects in newborns. This is because over the normal course of development, the muscular wall (septum) between the atria generally becomes too thick for a balloon to be used.
A variation of balloon atrial septostomy, known as a graded balloon dilation atrial septostomy, has been used in adults to treat pulmonary hypertension. In this procedure, the physician initially uses a needle to create a tiny hole in the septum between the atria. The abnormally increased pressure in the right side of the heart caused by pulmonary hypertension allows blood to flow from the right atrium to the left atrium, creating a right-to-left shunt. Larger balloons are successively used to widen the hole. This procedure relieves the pressure on the right side of the heart, thereby hopefully preventing right-sided heart failureheart failure.
However, early studies have shown a high rate of mortality associated with the procedure, making it difficult to predict which patients will benefit. It is recommended that patients seek a physician and medical facility with a great deal of experience in balloon atrial septostomy in adults. Also, studies have found that older patients and those with impaired kidney function may not be good candidates for balloon atrial septostomy.
Questions for your doctor
Preparing questions in advance can help individuals to have more meaningful discussions with their physicians regarding their conditions. Parents may wish to ask their doctor the following questions about their child’s heart condition and balloon atrial septostomy:
What type of heart defect does my child have?
Is my child a candidate for balloon atrial septostomy?
At what age should the procedure be performed?
Where will the procedure be performed?
What are the risks associated with the procedure?
What is the recuperation from this procedure?
How will we know if the procedure is successful?
What will be done if the procedure does not correct the defect?
Will my child need additional surgeries any other defects?
What are the chances I will have another child born with a heart defect?
How might this procedure affect my child later in life?