Heart problem in fetus

[csutherland]

Anyone have any experience/information on a condition called Double Outlet Left Ventricle? My niece & her husband are expecting their 1st baby in Aug. They just found out today that their baby boy has this condition and will have to be delivered in St Louis or Kansas City so he can be taken immediately to surgery.
From the research I've done this is a very rare condition and I can't find info on how successful surgery will be and possible outcomes.

 

[kfay]

Lynlw is going to be the one who is going to have the most information on this. She has some great links to info on congenital heart diseases. I don't know anything about this particular defect, but, if I were pregnant with a child that had a major defect that was going to require surgery soon after birth, I'd have to give serious consideration to travel to have the baby at a center that has the most experience dealing with that. That is what is going to give the child the best chance at surviving.
Here is the list of the best pediatric heart centers: http://health.usnews.com/best-hospi...d=All&city=City&state=&zip=ZIP+Code&x=46&y=24

Best of luck to your neice and her baby.

 

[Lynlw]

Hi, Sorry to hear about your neice's and her baby. One good thing to remember is they have really come a long way in treating "heart babies". Unfortunately, off the top of my head I can't think of any friend of mine who child has Double outelt LEFT ventricle, I know a few Right ventricle, (DORV) so I Can't be as much help as I wish I could.

The best place I know of to learn about the different CHDs and the different surgeries with easy to understand text and great diagrams as well as alot of great info about the different tests and everything that goes along with having a "Heart Baby"is the Book "its My heart" from the Childrens heart Foundation. They don't have any hard copies right now (and haven't for a while but you can read the whole thing online in pdf. HOPEFULLY this will take you to the table of contents directly http://www.childrensheartfoundation.org/publications/its-my-heart/english
Some times I have problems with direct links to the book, so if they don't work just go their main site http://www.childrensheartfoundation.org/ and on the home page under for recently diagnosed is a link to the book.
One thing that may be tough, is until the baby is born and they can do an echo on the baby, things/diagnosis can always change, so it is hard to know for sure what the surgery will be. What they "normaly" do for the main CHD might change if there are other CHDs like VSDs or pulmonary stenosis and from MY experience, most babies with COMPLEX CHD usually have more than the main one. (for example Justin's main CHD is transpostion of the great vessels, the normal repair is they switch the pulm artery and aorta, but since he also had pulmonary stenosis he wasn't a candidate for the switch and instead neeeded a completely different repair. (Rastelli) Also it sometimes gets a little confusing in the beginning, because they do the same surgery/surgeries for a few different CHDs. Like Justin had a Rastelli, but they usually do that for double outlet Right ventricle (and some DOLV). Also so your familiy isn't blindsided, MANY of the repairs for the more complex CHDs involved more than 1 surgery in the beginning often they will need 2 or 3 stage surgeries. I might be wrong, but from a few things I've been reading, it looks like many of the repairs for left ventricle are the same surgeries as they do for the right ventricle. (which you can find alot more info on)
BUT even IF your neices baby has to have a couple surgeries, the odds for most of even the most complex CHD is they will do very well and live long happy lives.

 

[csutherland]

Thanks, Lyn. I was able to follow the link to the site you recommended. It was great EXCEPT that even with a search of the site there were no results for double outlet LEFT ventricle, it's so rare. I love the last sentence of your post, though. Thanks for the encouragement.
As a side note, after looking through all those different anomalies that can occur in the heart it's truly amazing that so many are "normal!" We tend to just take it for granted that everything's OK inside us.

 

[Lynlw]

Thanks, Lyn. I was able to follow the link to the site you recommended. It was great EXCEPT that even with a search of the site there were no results for double outlet LEFT ventricle, it's so rare. I love the last sentence of your post, though. Thanks for the encouragement.
As a side note, after looking through all those different anomalies that can occur in the heart it's truly amazing that so many are "normal!" We tend to just take it for granted that everything's OK inside us.

When Justin was a baby and I was walking around CHOP and beside the entire heart floor, I saw so many other congenital problems I was amazed to at how it really is a miracle the vast majority of babies are completely healthy.
From what i've been reading,like http://www.springerlink.com/content/k57275645t2324x4/fulltext.pdf?page=1 only a very small % of the double outlet ventricles are left but it seems that depending on the other CHDs the basic surgeries are the same (usually a fontan or Rastelli) so that should give you a little more hope to know many kids do very well with those surgeries
From the link above (which s not a full article, just the free part)
Opinion statement
Double-outlet left ventricle (DOLV) is a rare congenital cardiac malformation in
which both great arteries originate entirely or predominantly from the morphologic
left ventricle. DOLV occurs most commonly in the form of atrial situs solitus with
atrioventricular (AV) concordance. Ventricular septal defect (VSD) is most commonly
subaortic, and 90% of these patients have associated pulmonary stenosis. In most
cases, there is a right anterior position of the aorta relative to the pulmonary artery.
Most patients with DOLV will have pulmonary outfl ow obstruction and present soon
after birth with cyanosis. DOLV with two well-developed ventricles is conventionally
treated surgically by VSD closure, closure of the native left ventricle–to–pulmonary
outfl ow, and placement of a right ventricle–to–pulmonary artery extracardiac
conduit. However, pulmonary root translocation may be used as an alternative to
bi ventricular repair of DOLV. Patients with DOLV and functionally single-ventricle or
AV valve atresia will require a Fontan procedure.


The 1st surgery, involing the conduit is either a Rastelli or modified Rastelli (which Justin has and he turned 22 April 12th )