The Cardiologist

October 23rd, 2014 we were scheduled with the cardiologist. We were there early and have had time to think about the news we learned about the day before. We had been searching the internet to find out what HLHS actually means, and it was not good news. When we first came in we were sent in to do a very thorough ultrasound on the baby's heart. The ultrasound took about an hour and then we waited to meet with the cardiologist that were looking at the results before she came in to talk to us. When she came in she brought a couple of drawings of hearts. First he described how a regular heart works and then she described how a heart with HLHS looks. Our sons left ventricle was almost nonexistent and his aorta is underdeveloped and very narrow. Children with HLHS often have an opening between the atria, an atrial septum defect that makes it possible for oxygenated blood to pass through the atria from the left atrium to the right atrium and then down to the right ventricle that will pump the blood up through the pulmonary artery. Our son does not have an atrial septum defect and without oxygen in the blood a fetus cannot survive. Our son’s heart had done something amazing by creating its own vein from the left side into the right atrium so the oxygenated blood comes in to the right side, it is called a decompression vein. Isn’t that pretty amazing? The heart can do some mysterious things sometimes. If the heart had not created this alternative route for the blood it would have been very likely that our little boy had not made it this far. The cardiologist was going through treatment options for this condition and they are going to perform three open heart surgeries. The first procedure is called the Norwood procedure and will be performed within the baby’s first 24 hours. The second surgery (Glenn) will take place when the baby is 6-9 months old and the third surgery (Fontan) when the baby is 18-36 months old. These are very complicated surgeries on a heart the size of a baby’s fist and veins thin like spaghetti. What these surgeries will do is re-plumb everything to the hearts right side so the right side will pump blood out to the body and also pump oxygen into the blood, which is done by the left side in a normal heart. These surgeries must happen in three steps as I mentioned and does not fix the heart, but it makes it function with a lot of extra work for the right side. About 60% of babies with HLHS survives all three surgeries and can live a relatively normal life with some physical limitations. The oldest patients with HLHS are in their 30’s because these surgeries has only been around for that long. But science is always moving forward, so we have to stay optimistic.