Posted by Claudia Grazioso on April 14, 2011
Any birth defect is terrifying for parents, and if it requires surgery, it is even more so. If you’re child is born with Hypoplastic Left Heart Syndrome, he or she will need surgical intervention. Take a deep breath, and be strong. As surgical procedures have improved, the prognosis for children born with this frightening birth defect has also improved.
HLHS is a congenital heart defect in which the left side of the heart has not fully developed and is unable to pump blood to the body. For a while, the right side of the heart will compensate and attempt to perform all of the circulatory functions, but surgery to basically build the left side of the heart and connect it to the right side is essential.
The surgery usually happens in three stages. The first step is sometimes known as the Norwood Operation, and in the most basic terms, the doctor is rebuilding the aorta and connecting it to the right ventricle. This operation is usually performed within the first few weeks of life.
The second stage is sometimes referred to as the bi-directional Glenn or Hemi-Fontan procedure, and it connects the major vein carrying blood blue to the blood vessels that lead to the lungs, so that the blood can get oxygen. This surgery is usually performed when the baby is 4 to 12 months old.
The last step in the surgical process to the repair Hypoplastic Left Heart Syndrome is the Fontan procedure, and it is usually performed after the child is 18 months old. And in this procedure, the rest of the veins that carry blue blood are connected to blood vessels that go to the lungs.
Sometimes certain hospitals or doctors will opt for a heart transplant, but that is not the common choice as hearts for infants are rare.
The causes of HLHS are not known. But if you have one child born with HLHS, you are at increased risk for having another child with it. Signs to watch for are cyanosis, or a bluish tinge to your child’s skin. It is normal for a child who is cold to have a bluish tinge on their feet and hands, but if you see it on their chest, abdomen, lips or tongue, you should read it as a possible sign that they are not getting enough oxygen. Additional symptoms are a poor pulse rate, shortness of breath, cold hands and feet and rapid breathing.
HLHS is a serious birth defect, but it is important to remember that survival rates are going up as surgical techniques improve. Many children survive this birth defect, but you should be watchful for complications that can arise. Your child may tire easily after exercise, experience arrhythmias, edema and blood clots. For these reasons, it is very important to see a cardiologist for ongoing care.