Pediatric Heart Expert Forum
l-TGA
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Questions in this forum are answered by pediatric cardiologists, cardiothoracic surgeons and anesthesiologists from The Children's Hospital of Philadelphia. This forum is for questions and support about pediatric heart problems, symptoms and topics such as heart murmurs, palpitations, fainting, chest pain, congenital heart defects (including management and intervention), fetal cardiology, adult congenital cardiology, arrhythmias and pre-participation athletic screening.

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l-TGA

My daughter is pregnant with her first child and we learned our grandson has I-TGA. My daughter is due in June 2012. After digesting all that we have been given to us we are now looking for best outcomes. We have been on line searching for information to find out more information about surgery outcomes (Double Switch), who has been successful, who has done the most, etc. We have also been trying to find answers to know when it is best to have the surgery or not have the surgery. As one parent wrote in 2009 on this blog, was very well put... Trying to make a decision to have surgery or not is a very difficult one as you do not want to put your child who may appear healthy knowing his little heart needs help.

We have seen a very fine pediatric cardiac surgeon in our state after we received the diagnosis to come and talk with us, but at that time we were so overwhelmed with the news we could not ask all the questions. He did let us know they do not have to make a decision immediately but if surgery is what they want to do, to do it before he is 5 years of age.

We need guidance... How do we locate information on surgeons or institutes for whom how have done this surgery, their statistics and outcomes, and rankings of success?

Also, is there any information that helps a new parent make a decision if is surgery is the right thing to do?  What symptoms should you always consider surgery or when do you say no to surgery.

What would you do if this was your child?  I am not looking for absolute decisions but information and some  guidance.

We are praying our little boy will be born healthy and normal, that his heart problem will correct itself.
He is a special little boy in our hearts and he will bless many who will come in contact with him.

Tags: L-TGA, cctga
773637_tn?1327450515
Dear MamawR,

For our other readers, l-transposition of the great arteries (L-TGA, or "corrected transposition") occurs when there is a right atrium feeding into a left ventricle which then goes to the pulmonary artery.  As well, the left atrium sends blood into a right ventricle which goes then out to the aorta and the body.  Everything is in the right place EXCEPT for the ventricles, which are in an "inverted" position.  The probelm with patients with this heart is that the right ventricle was never meant to work as a systemic ventricle, pumping systemic-level blood pressures.  Thus, over time, the right ventricle fails.

The double-switch operation is one in which baffles are placed within the atria to direct the blood from the atria to the correct ventricle (i.e. right to right and left to left).  The second part of the double-switch occurs with a switching of the great arteries (pulmonary artery and aorta) as well as the coronary arteries, so that the redirected blood goes to the correct location.

So, how do you choose a surgeon for this?  The short answer is that it's REALLY difficult.  This is not a common diagnosis, so it is not a frequently performed surgery at many places.  It may be that there are some surgical centers that are referral centers, as they do more of these than others.  However, in total, they are rare.  And, since they are rare, these are surgical procedures whose outcomes are not widely reported.  I would recommend having the pediatric cardiologist do the footwork here, and find the center(s) that do more of these.  In the absence of that, do remember that it's not just the surgeon but also the entire team, including the intensive care unit afterwards.  This means that cardiac ICU outcomes matter, as well.

In these patients, sa you have already been advised, it is best to not wait for symptoms.  If you get to the point where the cihld is symptomatic, the surgical outcome will be worse, as there is less "cardiac reserve" to be able to tolerate the surgery.

Unfortunately, this is not a cardiac condition that will correct itself.  All that can be done at this point is to hope that there was an error on the fetal echo.  That said, the likelihood of that is low if it was done by a pediatric cardiologist who is experienced and skilled with fetal echocardiography.  There are patients w/ l-TGA that are able to go a number of years prior to requiring surgery.  I think that we don't really know exactly what the appropriate time to do the intervention is.  There are those who say it should be done before age 5 years.  However, there are those who would say that it should be done when the right ventricle just starts to have problems, and that may not occur until years later.  The bottom line here is that we have little data on what the best thing is to do.
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