Evan 101.

I’ve been asked many times what exactly is “wrong” with Evan’s heart.

His defects are diverse, complicated, rare.

Understanding his defects in a quick 5 minute conversation is really impossible.

It took me a while to learn his heart.  I had to learn what normal looked like in order to learn all of Evan’s “special.”

It also took me awhile to decide if I was going to post this.  To see all that needs repair makes me incredibly sad and I still feel guilt in that maybe something I did or didn’t do made him this way. And seeing all of it…it will be overwhelming to many of you…

I just need everyone to promise to see EVAN…not his heart…when you see him or meet him.  It doesn’t define him in any way…it’s just a part of who he is.

But, to really grasp the “biggness” of what we are about to do for Evan in Boston, I have to try and explain what Evan’s heart looks like and how it is so different from what a “normal, healthy” heart is supposed to look like.

I’m doing this because I get so many emails and messages from friends, family and strangers of people who know “so and so” who had heart surgery and is doing super.  Or messages about how excited people are because Evan’s heart will be “fixed.”

I know I flippantly call this surgery a “repair” to “fix his heart”.  Once you see how much “work” his heart needs – you will hopefully understand the magnitude of what is about to happen to our boy and how much of his heart will need to be monitored for the rest of his life.

The difference with this “repair” in Boston and what Dallas proposed is that the surgeon in Boston will give Evan as close to what a normal heart should look and function.  It will however, require him to touch and manipulate parts of his heart that normally should not be touched or manipulated.  There will not be a inch of Evan’s heart that won’t be handled during this surgery.

Dallas – proposed a temporary fix – which would ultimately lead to transplant.  How long that would be…we just would never know.

That being said – the Boston repair is young.  And the outcomes are relatively good. But, there are still patients who DO end up with transplant.  But, it is our hope that Evan will be able to handle the new circulation and not need a transplant in the future.  He will – however – need intervention again in the future – a heart catheterization and even another open heart surgery.

I just need to reiterate, a transplant is something every complex congenital heart patient is always told is a possibility.  That’s just the lay of the (heart)land.

I know some of you – my doctor friends in particular – have a lot of questions.  I plan on doing an FAQ about the surgery and our plans.  But for now…take a potty break and grab some coffee – you are about to get schooled on what an incredibly special heart my son has.

 

Here’s normal:

Capture

Normal heart – (blue blood – de-oxygenated) enters the Right atrium via the Superior Vena cava and the inferior vena cava

–          Blue Blood flows from the right atrium into the right ventricle via the tricuspid valve (valves help prevent backflow)

–          Blue Blood is then forcefully pushed out the right ventricle through pulmonary artery to the lungs

–          Blood will get oxygenated in the lungs

–          Blood returns to the Left Atrium via the pulmonary veins (blood is now oxygengated – RED blood)

–          Red blood flows from the Left Atrium (LA) to the Left Ventricle

–          Red blood is forcefully pumped out of the LV to the body via the aorta

Here’s Evan’s heart:

The areas with the “NO” sign marked through are areas where a defect is present.

Evan's Heart

 

 

Evan’s heart

1 – NO septum – he has large holes in the middle of his heart so while he has a left and right side of his heart, the has holes and very little differentiation between the atrium and ventricles – he has a large chamber where oxygenated blood and deoxygenated blood flow

2a, 2b – because of the large holes in his heart and where the walls of hit heart did not form, he does not have these key valves for the right and left atrium to ventricles  has a common AV (atrioventriular valve) valve into the large chamber (he lacks the tricuspid valve and mitral valve)

3 – his pulmonary artery is very narrowed – called stenosis – above, below and right where the opening is.  This makes it very hard for blood to be pushed into the lungs from the right side of his heart. This is also referred to at the Right Ventricular Outflow Tract.  This causes pressures in his heart – namely the right side to be higher and cause the heart to work harder.

4 – the aorta is responsible for oxygenated blood to the body.  His curves to the right instead of the left.  And because of the large, single chamber of his heart, it currently leaves from the Right Ventricle instead of the Left ventricle.

5 – Superior vena cava – this allows blood to return to the heart – Evan has 2 of these – called bilateral SVCs.

6 – Inferior vena cava – this allow allows blood return to the heart – Evan does NOT have an inferior vena cava.  He has flow through his liver which allows blood to return to the heart via other routes.  This puts pressure on his liver.

7a, 7b – Pulmonary veins – they are not in the correct place – called Total Anomalous Pulmonary Venous return

8 – his heart is what is called dextrocardic – it sits in the right side of his chest instead of his left.

 

  Evan’s planned repair

1 – septate the heart.  The surgeon will use synthetic material to make right and left ventricles to make very clear pathways for de-oxygenated blood (Right side) and oxygenated blood (left side) to go.

 2a, 2b – Dr. Del Nido will split the common AV valve into right and left valves – tricuspid and mitral , respectively.

3 – his valve will be replaced with what is called a pulmonary conduit with either a human donor valve, synthetic, or animal valve. The plan currently is to use a human donor valve called a homograft.

4 – once the heart is septated – the surgeon will move the aorta to the left side of the heart so when the heart pumps, it will pump from the Left ventricle as in a normal heart.

5 – he will “unhook” the current connections to his pulmonary arteries that were established by our Dallas team.  Because the blood flow through the pulmonary artery is how the blood will get to the lungs.

6 – nothing – but with the additions of the repairs above – there will be less stress on the liver

7 – when the surgeon septates the heart, he will do so in a way that the veins will go to the right place in his heart.

8 – just adds a level of complexity and difficulty for the surgeon.  Nothing to be done…just make sure when Evan says the “Pledge of Allegiance” he puts his left hand over his right sided heart….

 

There you have it.

Now…that you know his heart.

Remember, that he’s mine…my heart no matter how imperfect it may be.

And pray for him even more fiercely than you could ever imagine.

 

Comments

  1. Sending lots of prayers and good vibes and positive thoughts to your amazing boy, and to all of you!

  2. My daughter Matilda died of CHD (rare variety of pulmonary atresia) January last year after only three days outside the womb. My heart breaks over and over again every time I read about someone else having to fight that same awful battle. Praying that you would get to keep your Evan for many, many more years.

  3. Just came by your blog by accident when another mom (who has a special needs child, although not heart related) shared your recent blog post about wanting to just be mommy. Does your baby have heterotaxy syndrome? With some of his defects, I just wondered. My daughter, Harper, has heterotaxy syndrome that resulted in multiple heart defects. We are deciding on where to have her procedures done so I am just curious about exactly what led to your decisions. Thanks!