Articles from prior issues of The Advocate

March/April 2000

Congenital Heart Defects Impact Thousands Of Children Nationwide
Colorado Alone Sees 2500 Cases Annually
by Lisa Martin, Texas DDS

REGINALD WASHINGTON, M.D. SPOKE AT THE NADE Conference on the functional impact of congenital heart disease in children. Dr. Washington began by giving some statistics. He reported that 2500 children are born with congenital heart defects each year in Colorado. Ventricular septal defects account for 28% of these; atrial septal defects, 10%; and other defects, 15%. This 15% contains approximately 250 diagnoses. These are the most difficult cases to handle in disability caseloads. As technology progresses, the medical community is saving more and more patients but are creating more and more disabled individuals. In cases of coarctation of the aorta, the prognosis is extremely variable depending on the individual. Coarctation of the aorta is causes an hour-glass shape narrowing of the aorta. In coarctation of the aorta, the heart is usually enlarged and there is severe aortic stenosis. The diagnosis depends on the degree of obstructions. The prognosis also depends on the decision to operate, the age of child, etc. Some surgeons will just cut out the obstructions. Severe obstructions will need a bypass graft. However, as the child grows, the graft would need to be replaced. Therefore, this procedure has to be repeated as the child gets older. In the 1970s, children born with coarctation were likely to die. The prognosis is much better now. Transposition of the great arteries (TGA) patients, with an intact ventricular septum, usually die shortly after birth if the defect is not detected quickly. These are the blue babies. There was a 100% mortality rate of these infants 30 years ago. Today, a balloon technique has been developed to tear between the chambers. Other new techniques now allow these patients to live until 20 to 30 years old. However, these patients will need transplantation to live beyond this time frame. In many cases the doctor will stabilize the child and then perform surgery to correct the vessel placement. The greatest challenge for surgery in these infants is that the heart is the size of a walnut. Therefore, this is a very intricate procedure. However, the prognosis after surgery is very good. Tetralogy of Fallot includes a narrowing of the pulmonary artery outflow tract with a hole between the ventricles. These babies can be very variable in their presentation. Usually the child will have a boot-shaped heart on chest x-ray. The lung artery is also extremely small. If the child is in extreme need of surgery a shunt will be inserted. If the baby's condition is less serious, the physician may decide to manage the child on medication until it gets older and surgery can be done to patch the defect. Hypoplastic left heart syndrome is a congenital heart defect where the child is born without the left side of the heart. These children literally have just has half a heart. What can be done for these children? A Norwood procedure is being done at a hospital in Philadelphia. This is a three stage procedure and is claimed to have a 90% survival rate. In the first stage, the surgeon takes the two vessels and combines them. In the second stage, they take the blood from the upper body and direct it to the pulmonary artery. Stage three takes the blood from the lower body and direct it to the pulmonary artery. Some heart centers will do this procedure, others will do a heart transplant. Still others will tell the parents that the child has a major congenital defect that is not survivable and that the parents should withdraw life support. Even if transplanted, the child will need another transplant as the heart wears out. This is one of the reasons why some doctors recommend removal of life support. Twenty years ago none of these children would have survived. Now those that have surgery have a fairly good survival rate for five years. How is a Disability Examiner to know if a child is disabled from a congenital heart defect? Dr. Washington suggested that the examiner contact the treating cardiologist on these types of cases. An examiner needs to know if any surgical procedures have been performed and how often the child is seen in follow-up, i.e. once a year, monthly, weekly. Current testing will tell the examiner how the child is currently functioning. These tests include X-rays, EKGs, echocardiograms, and angiograms. We must consider that the child maybe better, but is not completely without problems. Dr. Washington states that considering our children are now more obese and less fit than 10 years ago, everyone needs to be more active. Cardiac patients are being “saved” in record numbers, but we must consider who will care for this child as he/she gets older. Children with congenital heart disease can be physically active, but the activity must be safe. They will require limits. The cardiologist or pediatrician should be documenting the records adequately. These records should give a good indication as to the functional abilities of the child.