Single Ventricle Malformations: Joseph's Story Video Series

Transcript: Single Ventricle Surgery: First Stage Repair

Transcripts in This Video Series
Joseph's Story What Are Single Ventricle Malformations? Stabilizing Your Baby After Delivery Single Ventricle Surgery: First Stage Repair Single Ventricle Surgery: Second Stage Repair Single Ventricle Surgery: Third Stage Repair Life After Single Ventricle Surgery

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Thomas Spray, MD: The various surgical options for single ventricle depend, initially, on how much blood flow there is to the lungs. And is there obstruction to the body?

Jack Rychik, MD: The strategy for single ventricle can really be distilled down to a plumbing problem.

Thomas Spray, MD: This is not an operation that creates a normal heart.

Jack Rychik, MD: We can't fix the heart.

J. William Gaynor, MD: We cannot make another pumping chamber.

Thomas Spray, MD: What we do, surgically, is rearrange things, close holes, make connections--

J. William Gaynor, MD: --so that you can have blood going to the lungs and blood going to the body with only one pumping chamber.

Thomas Spray, MD: But that isn't the same as a normal heart.

Jack Rychik, MD: By rerouting the plumbing we normalize the circulation.

J. William Gaynor, MD: And that procedure that does that is called the Fontan operation.

Jack Rychik, MD: And that operation is common amongst all the single ventricle patients. What is different between these various categories is what is done in the first few days of life.

Peter Gruber, MD: It really boils down to how you're going to get blood flow in an unobstructed fashion without any resistance to either the body or the lungs.

Jack Rychik, MD: In Category I where there's obstruction of blood flow to the body one will have to go — undergo a Norwood-type operation to allow for unobstructed blood flow to the body.

Thomas Spray, MD: And the principles of that operation are to connect everything so that the one good pumping chamber has to pump to the body. So you have to connect the arteries to the lungs and the body in such a way that all the blood goes out to the body without any obstruction.

Peter Gruber, MD: The aorta is usually too small. So we correct that by putting a patch on to enlarge it.

J. William Gaynor, MD: And that's done by connecting the pulmonary artery, which comes from the right ventricle, to the aorta and then usually putting a patch on to finish the connection of those two blood vessels and enlarge the aorta.

Thomas Spray, MD: In addition, you have to prevent blood from backing up inside the left side of the heart, so you have to cut out the partition between the two upper chambers of the heart. And then the third principle of the Norwood operation, or the first stage operation, is to provide some restricted blood flow to the lungs.

Peter Gruber, MD: What we call pulmonary blood flow. And we do that by placing a shunt, or a small tube, from one of the systemic arteries, or arteries that goes from the heart to the body, to the pulmonary artery.

Thomas Spray, MD: That is enough flow that you can get enough oxygen in the bloodstream but not so much flow that you have resistance problems. Because that resistance is what prevents you from doing any further surgery.

J. William Gaynor, MD: In other forms of single ventricle, such as tricuspid atresia or pulmonary atresia, you have blockage of blood going to the lungs.

Jack Rychik, MD: If one falls into the category which is obstruction of blood flow to the lung, then one will need a shunt.

Peter Gruber, MD: So if the pulmonary artery is too small, or it doesn't form, or one of the valves that leads to that artery doesn't form, then you need to supply blood flow to the lungs. And we usually do that through the formation of a shunt.

Thomas Spray, MD: The idea being to control how much blood goes to the lungs but allow blood to get to the lungs to mix in the heart.

Jack Rychik, MD: In Category III, if nature has allowed for such, if there's adequate blood flow to the body and to the lung —

J. William Gaynor, MD: You may have a single ventricle with no blockage.

Thomas Spray, MD: There are children who have a type of single ventricle where there's unrestricted blood flow to the lungs and no obstruction to the body. In that situation, if you were just to leave that after birth, these children would get more and more flow to the lungs because of the resistance dropping and then they go into heart failure. As that happens, the body tries to protect itself by creating a high resistance in the lungs and that, of course, is the worst thing you can have for single ventricle, because it prevents you from doing the final operation, the Fontan operation.

Robert Shaddy, MD: One thing that we can offer to infants is the put a band around the pulmonary artery, which is just a ligature that is placed around the pulmonary artery and then tightened down and allows then restriction of blood going to the lungs, so that babies get enough blood and have adequate oxygenation, but not so much that we flood the lungs or damage the lungs because of excessive pulmonary blood flow.

Jonathan Rome, MD: The banding procedure to limit blood flow to the lungs is a surgical procedure. Although people have started developing bands that are adjustable, they're still typically put in by surgery.

Thomas Spray, MD: There are some children who have what is basically a perfect balance between the arteries to the lungs and the arteries to the body in such a way that they have no obstruction of flow to the body, but they've controlled how much blood goes to the lungs with some narrowing at some point in the pulmonary artery.

Sarah Tabbutt, MD: And that group of patients can actually have their first heart surgery when they're older, usually around 4 to 6 months of age.

Lynne Ramsay, Mother: He predicted that Joseph probably would not need that first surgery.

Anthony Ramsay, Father: Because of his makeup the blood was actually mixing very well. We actually didn't have to go through the first stage operation.

Sarah Tabbutt, MD: That group will still require two heart surgeries to get through the pathway, a palliation for single ventricle. But they're lucky. They get to miss the neonatal procedure.

Jack Rychik, MD: So, therefore, there are either two or three surgeries that are necessary depending on the category that you fall into.