Case in Progress
Cameron at 4 months
Cameron is a 4 month old little girl with Down Syndrome. Approximately 40-45% of children with Down Syndrome are born with heart defects.
In Cameron's case she was diagnosed with endocardial cushion defect and small patent ductus arteriosis, (PDA). The PDA closed over several days following her birth. Due to the endocardial defect Cameron has been taking lanoxin and furosemide to help her heart to contract with more force and function more efficiently.
Cameron is scheduled for surgery at Childrens Hospital Los Angeles to repair the endocardial defect on May 27, 1998. She will be 5½ months old. We will keep you posted on the progress and her family.
Before her surgery let's review the heart and specifically why it must be repaired.
When a baby has a heart defect, there may be a hole in the walls between the chambers. The result is that too much blood is pumped to the lungs and not enough blood is pumped to the rest of the body. Consequently, the body does not get enough oxygen-carrying blood. In addition, because a hole can disrupt the flow of blood within the heart and allow blood to pool, this condition can raise the risk of serious cardiac infections.
There are several types of heart defects. The most common in babies with Down syndrome is called an atrioventricular canal defect or AV canal, as it is commonly called. It is also sometimes referred to as an endocardial cushion defect. An AV canal is a large hole in the center of the heart. This means that the walls between the two upper chambers (the atria) and the two lower chambers (the ventricles), as well as the valves between them, may be deformed. This large opening in the center of the heart allows the red oxygenated blood to mix with low-oxygen blood and return to the lungs. This forces the heart to pump an extra amount of blood to the lungs. This extra effort in turn causes the heart to enlarge. In addition, the body receives less oxygen since it receives red blood that is mixed with low-oxygen blood.
Because of the high volume of blood that is pumped to the lungs, high blood pressure may occur there, resulting in damage to the lungs and blood vessels. Surgical repair of an AV canal usually restores blood circulation to normal.
Heart defects are serious problems. In their most severe form. they can threaten your baby's life soon after birth and require emergency corrective surgery. Even defects that do not need immediate surgery can drastically shorten life if left un-treated. If, because of an un-treated heart defect, excess blood is continually pumped to the lungs, high pressure in the blood vessels in the lungs (called pulmonary hypertension) will result. Over time, the blood vessels will become scarred, and, if not treated soon enough, will eventually income so narrow that not enough blood can reach the lungs. This condition is fatal.
Babies with heart defects may show symptoms of what is called "heart failure." Heart failure does not mean the heart is stopping, but rather that it cannot keep up with the body's needs. The clearest symptoms are poor feeding, a change in color during feeding, poor growth, and labored breathing. Sometimes the baby's skin may turn blue (cyanosis), especially during times of feeding or physical exertion. Other signs include accelerated breathing and frequent upper respiratory infections. Children with heart failure need to be very closely monitored by their doctors, parents, and teachers.
There are two ways heart defects are treated by doctors. First, drugs are used to treat minor defects and to help babies with more serious defects survive until they are ready for surgery. This is what Cameron's physician is doing with the lanoxin and furosemide.
Second, open heart surgery may be necessary to correct a defect. The decision to operate to fix a heart defect depends on multiple factors, including complexity of the defect, any related problems, the child's age and weight, and psychological and social considerations. Following successful surgery, an improvement in heart function, growth, and development can be expected. Cameron's family was told there is approximately 95% survival rate following surgical repair of AV canal.
Fortunately, children with Down Syndrome and heart defects can he successfully treated early in life, resulting in increased longevity and improved quality of life. Today, the prognosis for most babies is greatly improved.
For more information
National Down Syndrome Congress
Canadian Down Syndrome Society
Babies with Down Syndrome -- A New Parents' Guide,
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