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.
Its 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.
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.