The risk of a potential heart defect is quite high for newborns with Down syndrome. According to the American Heart Association, less than one percent of newborns suffer from heart defects. However, for newborns with down syndrome the incidence rises to 45%, making heart defects a serious health concern and common occurrence.
What is a Septal Heart Defect?
The most common type of heart defect for children with Down syndrome is a septal defect. To better understand a septal defect, it is important to know that the human heart is divided into four distinct chambers. The right and left atrium are the upper two chambers of the heart and the right and left ventricle are the lower two chambers of the heart. Septal defects occur when the walls between the chambers of the heart do not fully develop leaving gaps or holes in them. The size of the hole can vary significantly, from a very small hole to a complete absence of the septum.
Symptoms of a Septal Heart Defect in an Infant with Down Syndrome
The symptoms resulting from the septal heart defect depend largely on the size of the defect. Most infants with Down syndrome do not exhibits symptoms of a heart defect until several weeks of age. Symptoms of a heart defect include: difficulty feeding, sweating while feeding, poor weight gain, and labored breathing. Infants with Down syndrome that have heart defects often become tired and fall asleep while feeding. Feeding is exhausting for them. In an infant with Down syndrome that suffers from a heart defect, the chest often rises and falls dramatically as they take a breath. Infants with heart defects expend a lot of calories doing simple activities like eating, sleeping and breathing. Because they often can not consume enough calories to keep up with the extra work load from the heart defect they experience poor weight gain or are diagnosed with "failure to thrive."
The Physician's Role in Treating a Septal Heart Defect and Common Medications
Infants with suspected heart defects should be evaluated by a physician who will check the infants weight, heart rate, breathing, and listen for sounds of a heart murmur, or shunting. The physician that suspects a heart defect will refer the child to a pediatric cardiologist that can perform an echocardiogram to determine if the child with Down syndrome does indeed have a heart defect. If a heart defect is confirmed a cardiologist may opt to prescribe a medication that will help the heart do it's job. This is often done to see if the defect will correct itself, or to help the child reach an age to withstand surgery. Lanoxin and Lasix are two commonly prescribed drugs that help the heart do it's job and rid the lungs of excess fluid.
Surgery for Septal Heart Defects in Children with Down Syndrome
Because the septal defects of children with down syndrome rarely heal themselves, the majority of infants diagnosed with a heart defect eventually undergo surgery. Infants have a better chance of surviving the surgery if the child is at least three months of age. For this reason, pediatric heart surgeons commonly rely on the prescription medications listed above as a method of postponing the surgery until the child reaches three months of age.
Pulmonary Hypertension in Children with Down Syndrome that Suffer from Heart Defects
However, postponing the surgery until the child is three months increases the risk of the child developing pulmonary hypertension and occlusive pulmonary vascular disease, or OPVD. Pulmonary hypertension occurs in children with heart defects because the heart allows to much blood to flow to the lungs. To reduce the flow of blood, the pulmonary blood vessels constrict. Over time the tissue of the blood vessels with thicken. This can lead to an unusual blood flow pattern within the heart, bypassing the lungs and circulating deoxygenated blood throughout the body. The infant with Down syndrome that experiences this may exhibit cyanosis, or a blue coloring of the skin. Often cyanosis is most noticeable in the finger tips and nails. Children with Down syndrome that have been diagnosed with heart defects, tend to develop serious pulmonary hypertension complications at an earlier age than their peers, with life-threatening pulmonary conditions found in infants as young as six months of age. Heart defects should be surgically correct prior to six months when possible.
Advances in surgical procedures have made the repair of a septal defect in children with Down syndrome very successful. The repair is done by patching the hole with a piece of synthetic surgical material, such as Dacron. The success rate is quite high, with over 90 percent of patients having no complications from the surgery. Infants with down syndrome undergoing septal defect repair are often released from medical care within one to two weeks and require only occasional cardiac follow-up.
Sources:
Mecial and Surgical Care for Children with Down Syndrome: A Guide for Parents by Philip Mattheis, MD, Susan Schoon Eberly, MA, and Janet Williams, PhD [Woodbine House, 1995]
Heather Schulte - Heather Schulte is a freelance writer based in the Midwest. She writes web content for a variety of publications such as Suite101, eHow, ...
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