Congenital Heart Disease

Somewhere between 30 and 60% of children with Down syndrome have congenital heart defects, with ventricular septal defects and complete atrioventricular septal defects being the most prevalent. Other newborns with Down syndrome are diagnosed with tetralogy of Fallot or patent ductus arteriosus.

Because young children with Down syndrome develop early increases in pulmonary vascular resistance, there is less left to right intracardiac shunting in the newborn period than there will be later in infancy. For this reason, neonates with significant septal defects may show no signs of congestive heart failure or respiratory involvement right after birth. These babies can seem healthy during their first 8 months, and may even seem to be getting better. Unless the defect is corrected surgically, however, the infants may be undergoing significant pulmonary vascular changes that have lifelong consequences.

It’s extremely important, therefore, that all babies with Down syndrome be evaluated by a pediatric cardiologist and have an echocardiogram during their first three months of life. In some cases, children with mild congenital defects are monitored regularly and the defects resolve without surgery. Other children need intensive medical intervention in the form of diuretics, digitalis, and other cardiac medications. And about half the children with septal defects require open heart surgery to repair the defect and prevent further cardiopulmonary problems. The good news is that these children generally do well with the surgery and go on to live healthy lives.

For more indepth info about ventricular septal defect and congenital heart disease, please see the RnCeus.com interactive course: Ventricular Septal Defect: Effects, Assessment & Treatment.

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If babies with Down syndrome do well during their first eight months of life, we can safely surmise that they have no congenital heart disease.
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Nurses caring for infants with Down syndrome should be aware of the early symptoms of heart disease in babies:
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Vomiting and poor weight gain may be signs of early heart disease in babies with Down syndrome.
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When working with children who have valvular disease, it’s important to determine the physician’s recommendations for prophylactic treatment for subacute bacterial endocarditis (SBE) and counsel the parents when indicated. SBE prophylaxis involves preventive antibiotic therapy before dental cleanings and other invasive procedures.

Clearly, nurses can play many roles in the lives of young children with Down syndrome and congenital heart disease. Encouraging parents to follow health screening guidelines, teaching them to administer medications and other treatments as needed, and providing peri-operative care related to open heart surgery are expectations of nurses in a variety of settings.