How common is this?

My daughter was seen by a cardiac specialist in Chicago today, after her ob Dr. was concerned with possible fluid around the babies heart.  It turns out that there was no fluid, but the baby was diagnosed with Congenital Heart Disease.  DTGA.  This disease is when the heart was developing, the Aorta developed on the right side of the heart and the Pulmonary on the left instead of the other way around like it is suppose to be.  The cardialogist stated that until the baby is born, there is no danger.  After the baby is born, within 7-10 days the baby will need to have surgery to correct the arteries.  There is an estimated 85% survival rate with this type of disease.  He stated that this is a rare disease, but almost 10% of his patients have had this same defect.  They are pretty certain that there are no genetic defects tied in, so he is pretty confident as long as everything goes accordingly.  My daughter has to be seen by the pediatric cardialogist once a month to make sure that the area between the left ventricle and right ventricle remains open. I am hoping to find someone that has been through this in hopes of preparing ourselves for the long road ahead.

[Jeffrey R Boris, M.D.]

Dear Shari,

For our other readers, d-TGA is transposition of the great arteries.  You are correct in your description—the aorta arises from the right side of the heart, instead of the left, and the pulmonary artery arises from the left side of the heart, instead of the right.  This leads to 2 separate parallel blood circulation pathways, with one gong to and from the lungs and one going to and from the body.  In contrast, normal circulation is in series.  The “d” has to do with the arrangements of the ventricles and the great arteries.  You didn’t mention if they told you whether the ventricular septum is intact or there is a ventricular septal defect (a hole between the two ventricles), as that can make a difference in both the type and the timing of the intervention.  However, you do mention in the follow-up plan an “area” between the two ventricles remaining open, which suggests that there is a ventricular septal defect, though I’m not sure from your description.  By the way, it’s not particularly rare; the incidence of all congenital heart defects is in 0.8% of all babies born, or 1 out of 125.  Of those, 5-7% of them have d-TGA.  If you think about all the babies born every day, that’s a pretty good sized number.

It actually is a good thing that this was found in utero, because there must be a connection between the two different blood circulations to allow blood with oxygen to get out to the body.  Sometimes, if the connection is small, these kids can get sick, so it is nice to know ahead of time that your daughter needs to be looked after right away.  The heart surgery is performed quite soon, even sooner than 7-10 days at our institution, and the survival is quite good.  After surgery and release from the hospital, your daughter would be seen in the first 7-14 days, then likely again at 2 months after the surgery, then in 6-12 months after that, and likely annually beyond that.  There should not be a residual hole between any of the chambers after surgery.

Overall, the long term outcomes are very good, but not perfect.  This disease is uniformly fatal if it is not surgically repaired, and this is something that the field of cardiology has really done a great job with over the last 20 years.  However, one of the fields in which our cardiologists have done a fair bit of research is into the longer term neurodevelopmental outcomes of these patients.  We do know that a good sized number of these patients have complications of ADD with or without hyperactivity as well as abnormalities of executive function, even if nothing went wrong with their surgery.  Also, since both the great arteries AS WELL AS the coronary arteries (the arteries that feed the heart) need to be moved in the surgical repair, there have been increasing information that the coronary arteries, which are quite small, can have some problems with obstruction.  Both these things mean that your daughter will need lifelong care by a pediatric cardiologist, or an adult congenital cardiology specialist at the right time, to make sure that her heart is functioning well.