The Fontan

I have had a request to explain what the Fontan is, so if you're interested, here ya go.
The Fontan is the third and final stage of Olivia's repairs.  No, this does not mean she is fixed.  Her heart will always only be half and she will always have struggles.  We are planning on transplant.  The only question is when.  The three OHS (open heart surgeries) she requires will keep her going until her heart is too tired.  This could be anywhere from next year to early teens to 15 years down the road when they can grow her a custom made heart.  The last is what we are hoping for.

Pictured above is what Olivia's heart was when she was born compared to a normal heart.  Her LV is practically non-existent, it's so small.  Her mitral valve (the opening between the LA and LV was atretic (aka deformed) and that is probably what caused her LV to not form properly.  

So, as you can see, in a healthy heart, the left ventricle pumps oxygenated blood throughout the entire body while the right ventricle pumps blue blood to the lungs to be oxygenated.  Since Livie doesn't have a LV, her right ventricle has been made to do all the work.  A high pressure job for the low pressure side.  In case that doesn't sound scary to you, it is.  This is why she WILL need a transplant.  That RV won't be able to hold out forever.

The above picture is what her heart looked like after her first OHS.  She was six days old and this was the most complex of the three procedures.  Four separate things occured during this operation.  1. Donor tissue was used to expand the aorta.  2. the atrial septum was removed (the wall between the RA and LA) so that blood could mix.  This is why Liv's sats are supposed to be 75-85 and not higher like a  healthy heart.  3.  her pulmonary arteries were disconnected with a homograft patch on the PDA.  4.  A sano shunt was placed.  Above, it shows the blalock taussig shunt.  It's entirely up to the surgeon as to whether the BT shunt or sano is needed.  A lot of times it just depends on where they trained but Dr. Kaza just told us he would decide when he was in there and could see what would be the best fit.  Livie's sano went from the left side of her pulmonary arteries (basically by where the homograft patch is pictured) and went straight into her right ventricle.  Because of her sano, Livie's heart squeezes diskinetically (meaning just a little funky) because of scar tissue.  Also, her LPA (left pulmonary artery) isn't growing, also because of scar tissue.  This is easily fixed with a stent, so if I post xrays and you see a mesh lookin' kinda thing, that's her stent.  Imagine doing all that on a heart the size of a walnut!

Ahhhh the Glenn. Our saving grace.  Livie was NOT stable after her first surgery and the Glenn fixed that.  This one was much simpler.  The sano was removed and the superior vena cava was attached to the right pulmonary artery so blood from her upper extremities could flow passively to her lungs.  BTW the pulmonary arteries carry blood to the lungs.  So you can kind of see how gravity just carries blood from her head and such to the lungs and the workload is greatly reduced on the RV

Ah, the fontan.  The "F" word.  This could be the best thing in the world for Livie but can also be the worst.  There is no guarantee that her body will be happy with this change but we are hopeful.  So, for this one, a conduit is connects to the RPA (right pulmonary artery) with a fenestration between it and the right atrium. And it connects to the inferior vena cava.  She will have this conduit for life and it is BIG.  We got a little piece of the gortex tubing from her sano so maybe we'll get some from this and we'll post a pic.  For the first time, her blood will not be mixed and we should see sats in the 90's for the first time since she was born.  More blood will be going to the lungs which can cause those effusions I mentioned before.  If her body doesn't like this, she could go into heart failure.  There are also rare side effects whose only cure is transplant.  We're hoping to avoid those side effects but you never really know.  Oh, and also this conduit sits above her liver and can cause issues with blood pooling in the liver.  So, yeah.  Lots to worry about.  But also lots to be hopeful for which is what I'm trying really hard to hold on to.

So, if you ever doubted that I was smart, ta da!  I am one of the smartest history graduates when it comes to the functions of a hypoplastic heart.