Tetralogy of Fallot Video Series

Transcript: Preparing for your Baby's Delivery

Transcripts in This Video Series
Diagnosis What is Tetralogy of Fallot (TOF)? How TOF Develops Preparing for your Baby's Delivery TOF Surgery Life After TOF Surgery

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Peggy McCann, RDCS: A lot of times our families come in, and they're just conscientious of getting the heart adequately diagnosed. And what happens is once they're evaluated by our team, by our group of physicians and the nurse coordinator, they're given a little bit more information. And they're strongly encouraged to have other testing done and that might be an amniocentesis with a full genetic workup for a 22Q Deletion. They might have a fetal MRI. They might have a more involved fetal ultrasound.

Jack Rychik, MD: Tetralogy of Fallot is a relatively common form of congenital heart disease, and it can be associated with other forms of congenital abnormalities.

Elizabeth Goldmuntz, MD: At this point specifically what we know about the genetic basis of Tetralogy of Fallot is that about 7% of those children will have Trisomy 21. So they have a complete extra copy of a chromosome 21. We also know that about 15% of those children will be missing a piece of chromosome 22, now called the 22Q11 Deletion Syndrome.

Jack Rychik, MD: This abnormality can result in the presence of Tetralogy of Fallot. And it can also result in some other types of anomalies including cleft lip or palate, abnormalities of speech or phonation, and in some patients, even development of learning differences or learning difficulties.

Elizabeth Goldmuntz, MD: So you can see that the genetic basis of these disorders can encompass either complete change in chromosome number, like having a complete extra copy of a chromosome. It can be a missing part or a duplicated part, extra part of a chromosome, or it can be a small change in a specific and single gene.

Peggy McCann, RDCS: It's one thing for us to say, "We can fix the heart." But it's another thing to say, "Sure, we fixed the heart, but there's all these other questions that remain to be answered."

Jack Rychik, MD: We treat Tetralogy of Fallot in the same way in these patients that have these various other associated anomalies, but certainly, it places things in a little bit of a different context. Some of the patients who have these associated anomalies may require additional care and intervention in the Intensive Care Unit. There might be a need for a bit more attention or perhaps a bit of a longer stay when these children undergo these various surgeries.

Peggy McCann, RDCS: So it's important to know if there's other anomalies because it gives a more comprehensive picture to the parents of what this baby's going to behave like postnatally.

Thomas Spray, MD: Babies with Tetralogy of Fallot often do extremely well after birth.

Jack Rychik, MD: Most can safely deliver through a vaginal means. When delivered, however, and when separated from the placenta, no longer receiving oxygenated blood from the mother, we now place the task of oxygenation upon the lungs of the newborn.

J. William Gaynor, MD: If they have enough blood flow at the time of birth so that they don't need an urgent operation, we'll have an elective operation somewhere between 2 and 6 months of life.

Thomas Spray, MD: And those babies are just like any other baby. The issues really are feeding and growing.

Sarah Tabbutt, MD: Because, technically, for the surgeon, they can do it when the baby's first born. It's really no different than doing it when the baby's 3 months of age. It's just for us taking care of the baby in the ICU. Your hospitalization is much shorter if you've already been at home. You've learned how to eat. You've gone through that newborn period. Then, if you come back when you're 3 to 4 months of age for surgery, your recovery time in the hospital is very short, as compared to if you're a newborn.

Thomas Spray, MD: Nowadays, because surgery has developed so much, we can repair this heart defect very routinely somewhere between 2 and 4 months of age. So that's when we now electively repair Tetralogy of Fallot.

Peggy McCann, RDCS: In patients with a big VSD, with serious overriding of the aorta, and seriously stenotic pulmonary valve. We know that, unfortunately, that patient isn't going to have the liberty of waiting for, like, say a repair at 3 to 6 months.

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.

Jonathan Rome, MD: About half of the children who are born with Tetralogy of Fallot have a more complicated form of tetralogy, where not only is there a hole between the two pumping chambers of the heart and not only is there a narrowing going out to the lungs, but actually there's no connection at all between the heart and the blood vessels in the lungs. And that's called Tetralogy of Fallot with pulmonary atresia.

J. William Gaynor, MD: Obviously, if we know there's a complete blockage, or pulmonary atresia, we know the baby will not have blood flow to the lungs after birth, if the ductus closes. So we have to initiate prostaglandin therapy to keep the ductus open.

Jack Rychik, MD: When the ductus arteriosus remains open, that can provide an additional source of blood flow to the lung and, therefore, allow a period of stability and adequate oxygenation until the baby makes its way to surgery.

Sarah Tabbutt, MD: Sometimes they're born with a diagnosis where we're not sure if they're going to need surgery in the newborn period and so those babies are often started on a prostaglandin medication in the delivery room, which stabilizes the baby so that if there's any problems, they won't occur in the delivery room. And we bring the baby to the ICU, and we stop the prostaglandins. And we let the baby transition to being a baby separate from being in the uterus, and we watch very carefully and see how the child does. And for some of those children they need the prostaglandins restarted because, in fact, they do need to have their surgery before they go home and for other children they're actually okay and they can go home and come back at a later date.

J. William Gaynor, MD: So not every baby who has a prenatal diagnosis of Tetralogy of Fallot will need an immediate urgent operation. If possible, if they have adequate pulmonary blood flow, we will let them--we like to let them grow for a couple months before they have the surgery. So they would be born, they would come to the Cardiac Intensive Care Unit. They would have an echo to confirm the diagnosis. We would watch them to be sure that they had enough blood flow to the lungs, then let them start to eat, and hopefully go home, and come back for an elective operation at 2 to 3 months of age.