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Infant Toddler Development Training
Module 6, Lesson 5

Congenital Heart Disease

The normal heart has four chambers that pump oxygenated blood from the lungs to all parts of the body and return blood that contains carbon dioxide from the tissues back to the lungs for excretion. This process is necessary for cell maintenance and repair.

family with strollerInfants may be born with a defect of the heart. Some common defects include a Ventricular Septal Defect (VSD), Patent Ductus Arteriosus (PDA), Tetralogy of Fallot, coarctation of the aorta, Atrial Septal Defect (ASD), pulmonary stenosis, transposition of the great arteries, and hypoplastic left heart syndrome. Less common is a defect where an infant is born with only two or three chambers in the heart rather than four.

The following few sections will provide a detailed discussion of defects of the heart. The ITDS needs basic information about these conditions.

    • Endocardial Cushion Defect (Atrioventricular or A-V Canal Defect) is a large hole in the center of the heart where the wall between the upper chambers join the lower chambers. The defect also involves the heart valves. Instead of two separate valves, there is a single valve. The defect causes a mixing of oxygenated blood with un-oxygenated blood. Blood is pumped back into the body without an adequate supply of oxygen. The condition may result in high blood pressure and heart value regurgitation (blood leaking backward from the lower to the upper chambers).

      Infants with this condition, which is common in Down syndrome, usually have an enlarged heart. They breathe rapidly and have upper respiratory infections. They tend to have poor appetites and are undernourished. Surgery is required to correct the defect. Even after sugery, the child needs to be followed by a cardiologist. A child with an erndocardial cushion defect needs prophylactic antibiotics prior to dental work to prevent infection.

    • Ventricular Septal Defect (VSD) is the most common heart lesion. This is a hole in the wall between the ventricles, the two bottom pumping chambers. Some occur in isolation and some occur in combination with other conditions. VSDs usually do not present symptoms at birth. If the defect is large, it can result in congestive heart failure by 6 - 8 weeks of age because the blood is shunted from the left to the right ventricle producing an overload on the right side of the heart. The condition can result in a failure of the heart to pump enough blood into the tissues. Fluid becomes backed up in the heart and also into the lungs causing difficulty breathing.

      baby sleepingMany small VSDs spontaneously close and only present with a murmur. Moderate to large defects may need surgical correction. However, non-invasive procedures for repair are more often used today including "stents" and "umbrella patches". If treated promptly, there is minimal to no long-term sequalae. Infants are the most ill when they are very young and must be under the care of a pediatric cardiologist who will assist the family with nutrition and monitor appropriate activity levels.

What medical professions will be available to the ITDS for consultation when the ITDS is working with a child who has VSD?

When should developmental stimulation activities be initiated by the ITDS for a child who has a VSD?

    • Patent Ductus is the persistent patency of the blood vessel connecting the aorta and the pulmonary artery that normally closes at birth. However, for some infants it remains open and can cause congestive heart failure. A medicine, indomethacin, may be used to facilitate the closing of the patent ductus. If the defect does not respond to this medicine, then surgery may be required and usually effectively corrects the problem.
    • Tetralogy of Fallot is a combination of four lesions: a VSD, pulmonary stenosis, an overriding aortic valve, and right ventricular hypertrophy. The condition causes cyanosis (blueness due to lack of oxygen in the tissues). The infant might need a shunt to improve oxygenation. Other surgery to repair the defect is delayed until the infant is a little older.

What considerations for intervention would the ITDS need to consider for a child who has a Tetralogy of Fallot?

  • Coarctation of the Aorta or narrowing of the aorta, when present in combination with other heart problems or when severe can present in the newborn period with cyanosis and heart failure. However, it usually presents later with a murmur and discrepant blood pressures when the blood pressure is taken in both arms and legs of the child. The condition causes hypertension. Surgical repair is determined based on the location of the lesion and whether or not other anomalous structures are present.
  • Atrial Septal Defects (ASD) often go undiagnosed because everyone is born with a small opening between the two upper chambers of the heart, a patent foramen ovale that closes right after birth when the patent ductus closes. However, some reopen or stay open. In addition, there can be defects in other places in the atrial septum (between the two top chambers of the heart). These defects can usually be closed with a procedure called an "umbrella" patch.
  • mother feeding babyPulmonary Stenosis is a narrowing of the pulmonary valve. This valve allows blood to flow from the right ventricle of the heart to the pulmonary artery so that the body's blood can be re-oxygenated. When the opening is too narrow, the body does not receive enough oxygenated blood to maintain cellular function. Pulmonary stenosis is a congenital defect and can often be corrected with surgery. Severe pulmonary stenosis causes a child to be very ill.
  • Transposition of the Great Arteries is when the aorta and the pulmonary artery come out of the wrong ventricles of the heart. The blood that is returned to the body is not re-oxygenated and the baby appears very blue. The oxygenated blood that should go to the body is returned to the lungs. The baby is given a medicine called “prostaglandin” to keep the ductus open so that the body receives at least a small mixture of oxygenated blood with the blood that is pumped from the heart. Surgery is required to correct the defect.
  • Hypoplastic Left Heart Syndrome is very rare, but is very serious. The whole left side of the heart is underdeveloped. This includes the aorta, the aortic valve, the left ventricle and the mitral valve. An infant who has this rare condition often dies in the first week of life. The treatment for the condition is a heart transplant.

mother holding newborn babySome heart defects are treated with patching, a micro-surgical procedure that has reduced the risk to a child for open-heart surgery. However, open-heart surgery is still required in some circumstances. For a very small number of children, the only option for treatment is a heart transplant. The outcome of a heart transplant can be quite good if the threat of immunologic rejection is managed. To learn more about these and other heart conditions of infants and toddlers, the ITDS can access information from the Texas Heart Institute


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