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Single ventricle malformations are a group of congenital heart defects in which one of the heart’s pumping chambers (ventricles) develops improperly and cannot effectively circulate blood. In this video series, you'll learn how experts in the Fetal Heart Program at The Children's Hospital of Philadelphia diagnose and monitor single ventricle malformations before birth, allowing effective treatment to begin right after delivery.
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.
Jack Rychik, MD: We have identified some risk factors in patients who have single ventricle type of heart disease.
J. William Gaynor, MD: Certain forms of single ventricle may not do as well as others.
Jack Rychik, MD: Since one has to utilize the well-functioning ventricle to do the job of delivering blood flow to the body, if that single ventricle in some way is not functioning well or, if there's leakage of the valve that leads into it, that could be a significant risk factor for reconstruction.
Peter Gruber, MD: Many times there are other associated genetic abnormalities that we have to look for carefully. And these sorts of genetic abnormalities would provide incremental or increased risk for the operation itself.
Jack Rychik, MD: If the babies are immature, premature, that could add significant risk.
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.
Jack Rychik, MD: The obstruction of blood flow coming out of the lung, that we know, can result in poor development of the lungs and can add risk as well.
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.
Contact the Fetal Heart Program for more information