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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.
Lynne Ramsay, Mother: We left the hospital after he was born, after about three days — normal time. And we had about eight months at home — normal, which was wonderful. And then needed to have his surgery.
J. William Gaynor, MD: Eventually, we're going to go to the Fontan operation. And in the Fontan operation what we do is we connect up the blood flow coming back from the body directly to the lungs. You can do it in one operation, but we've learned that babies do much better if we split it up and do it in two operations.
Thomas Spray, MD: When you have this single ventricle malformation, all the blood that goes to the lungs, comes back to the heart, goes out to the body, and a portion to the lungs again. And the amount that is going to the lungs is an extra amount of blood that the heart has to pump. So eventually, we want to get to a situation where we're separating the circulations again. The second stage operation takes away that extra volume that the heart has to pump, and it does that by getting rid of the shunt, or getting rid of the blood flow to the lungs directly from the heart, and connecting the veins from the upper part of the body directly to the arteries to the lungs.
Peter Gruber, MD: You have to wait until the lungs essentially mature enough that you can provide a different source of pulmonary blood flow.
Thomas Spray, MD: This is called either a Bidirectional Glenn shunt or a HemiFontan operation, half of the Fontan operation.
Unknown Speaker: It's very, very important that the family feels comfortable with you as an individual taking their child from them and that the child recognizes that nothing bad is going to happen to them, that they can do this without being awake for any painful experience.
J. William Gaynor, MD: They'll go to the operating room. The anesthesiologist will put them to sleep. We'll then clean, prepare everything, their chest and abdomen sterilely so that we can do the operation without infection. It's performed through an incision in the front over the breastbone, and we put them on the heart-lung machine, and cool them down to take care of their brain and other organs while we do the repair.
Peter Gruber, MD: We're bypassing the heart and the lungs so that the operative field, the area where we're working, is clean, and we can see what's going on. The heart's generally not beating, although occasionally it is, but at least it's decompressed, there isn't blood flowing through it.
J. William Gaynor, MD: We'll then do the operation, warm them up, bring them off the heart-lung machine. We'll make sure that the heart is working okay, that the blood pressure is okay. That there's enough oxygen in the blood, that there's no bleeding. And then, usually, we leave a couple little tubes inside the heart that let us measure pressures in the heart and give drugs. These come out through the skin. There's also usually two little blue pacing wires which let us change the heart rhythm. We then leave a drainage tube, and once everything's stable, the baby will come back up to the Intensive Care Unit.
Thomas Spray, MD: Once we get to that second operation, the heart is in a much better condition and usually the risk of that surgery is extremely low and the risk after that surgery becomes quite low also of any sudden event. So I always breathe a sigh of relief when we get to that second operation or that second stage procedure because it's a much better physiologic situation for the heart.
Contact the Fetal Heart Program for more information