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Hypoplastic Left Heart Syndrome is a common congenital defect that involves an underdeveloped left ventricle, or pumping chamber.
Thomas Spray, MD: What we want to do after a baby is born is we want to stabilize the child, make sure that there are no other organs involved because there are other associated anomalies in some children. We want to make sure that there are no genetic issues that we need to address, we want to make sure all the other organ systems are as good as they can be and assess them if we have to. And then once that is done then it's more a matter of scheduling the surgery.
J. William Gaynor, MD: For many children with complex heart disease, there's blockage of the blood either going to the body or to the lungs. And there's a blood vessel called the ductus arteriosus which connects those two arteries. Everybody has one and it usually closes after birth.
Sarah Tabbutt, MD: You can keep that blood vessel open with a medication called prostaglandins. And so, therefore, by giving a baby prostaglandins you are actually replicating the same physiology that it had in the uterus when it was very stable to when it's outside the uterus.
J. William Gaynor, MD: So instead of having a child come in critically ill where there was nothing we could do, we can stabilize these children, do diagnostic procedures, then have a stable child who goes for surgery.
Thomas Spray, MD: If you just leave a child with this circulation for a period of time, then they end up going into heart failure because so much blood goes to the lungs in a circle that they end up in heart failure. So you have to walk a balance between the two. You have to have the ductus open and can eventually control how much blood goes to the lungs. That's why the operation is done usually in the first week of life.
Jack Rychik, MD: Through our experience with hypoplastic left heart syndrome in the fetus, we've been able to identify a variety of factors which function as risk factors for outcome for these patients.
J. William Gaynor, MD: One of the things that can cause a real problem is when there's blockage to blood flow from the lungs getting back to the heart.
Thomas Spray, MD: There are rare children who have what's called an intact atrial septum, and these children are the most difficult to deal with with hypoplastic left heart syndrome.
J. William Gaynor, MD: In hypoplastic left heart syndrome, the blood that comes back from the lungs comes to the left side of the heart. But because there's only one pumping chamber it has to cross over to the right side of the heart, inside the heart to be able to pump out. That's normally done through a hole in the wall between the two upper chambers of the heart called the foramen ovale. In some children we see where there's a very tiny hole and the hole is restrictive. Sometimes we can see where there is no hole and the atrial septum is completely closed.
Thomas Spray, MD: This complete closure of the partition between the upper chambers of the heart means that blood that gets back to the left side of the heart can't go anywhere.
Jack Rychik, MD: That results in an increased backup pressure on the pulmonary veins.
Thomas Spray, MD: So right after birth we have to do something very quickly to open up that partition.
Jack Rychik, MD: And the way that's done is by passing a catheter across the atrial septum, blowing a small balloon up--
Jonathan Rome, MD: --and you pull it back very quickly, and you literally rip a hole between the two upper chambers. That allows the blue and the red blood to mix now and then they get--the children become much pinker. They have oxygen in their bloodstream, and they're stabilized so that they can basically survive until they can have surgery.
Thomas Spray, MDAnd that can allow the resistance in the lungs to drop enough to consider the Stage I operation, usually after five or six days.
Jack Rychik, MD: And oftentimes, even when these babies are born and we immediately open the atrial septum, there can already be difficulties with the way the lungs have formed and that can add a risk to these patients. We know also from our experience that if there are any extracardiac abnormalities, besides the heart disease itself, that that can add risk to the reconstruction of the heart in HLHS. And we also know that if there's a known genetic abnormality, that can add risk. And then finally there's the issue of prematurity. Although we're now able to care for babies with HLHS very, very well, that are very small, if there is significant prematurity and immaturity, or lack of normal development of the lungs and other organ systems, that can add significant risk to the overall operation.
Michael W. Bebbington, MD: So if an amniocentesis test hasn't been done, or a chorionic villus sampling test hasn't been done prior to the patient being referred, then we can help to coordinate that either with the referring doctor or we can take primary responsibility for getting that testing done here.
Contact the Fetal Heart Program for more information