Our Teddy Bear's Journey

Theodore was born with renal failure. This is his story.

Congenital Heart Defect Awareness Week

on February 9, 2015

This is CHD Awareness Week.

Why, you might wonder, is Sarah putting this on Teddy’s blog?

That’s right. Remember that routine echocardiogram in January? We didn’t find heart wall thickening like we were looking for. We found a heart defect instead.

Teddy has a heart defect called Bicuspid Aortic Valve. (Sometimes it’s called Bicuspid Aortic Disease, BAD, lol.) Your aortic valve (between your heart and your aorta – the giant artery leading from your heart to the rest of your body) is supposed to be tricuspid – with three flaps. Teddy’s has two flaps. So it doesn’t open all the way (making the valve area narrower) and it doesn’t close all the way, either.

Associated with this, he also has stenosis (narrowing/hardening) of his artery, as well as regurgitation (blood flows backwards). It all means that his heart has to work a lot harder than it should.

There’s also a risk of other effects such as aneurysms in the aorta, in the brain, and in the renal veins, as well as renal cysts (which I find interesting). They think that BAV might be related to an underlying connective tissue disorder, as well. And the risk of aneurysms, as well as aortic dissection, isn’t necessarily related to the degree of complications from the valve. (In other words, a REALLY bad valve doesn’t seem to be any worse than a just sort of bad valve.) Another complication is infective endocarditis (infection of the lining of your heart), which is an interesting one because being a transplant recipient puts him at a higher risk of endocarditis,  as well.

This is a relatively common heart defect. The BIG problem is if you have this defect and don’t know it – a good chunk of athletes who keel over suddenly, for example, are found to have BAV or related issues. If you do know you have it, you can monitor for aneurysm development (at least in the aorta and renal veins), and for the stenosis or valve function to get bad enough to require surgery – hopefully BEFORE something happens.

Chances are good that Teddy will need open heart surgery at some point in his life. Chances are also good that this won’t be during his childhood. Most likely not until he’s a teenager or later.

We won’t see cardiology until April, at which point we should have more information. I think it’s a good sign that they didn’t need to see us immediately. 🙂

And because I like to give people more information than they probably want, here are a few scholarly articles on the subject:

This is a general overview of Bicuspid Aortic Disease, with a few pictures.

 

And this:

BAV occurs in 1% to 2% of the population, compared with 0.8% for all other forms of congenital cardiac disease combined. Given that serious complications will develop in ≥33% of patients with BAV, the bicuspid valve may be responsible for more deaths and morbidity than the combined effects of all the other congenital heart defects.7 Although patients with BAV may go undetected or without clinical consequences for a lifetime, the vast majority will require some intervention, most often surgery. The important clinical consequences of BAV disease are valvular stenosis, regurgitation, infective endocarditis, and aortic complications such as dilation and dissection.

http://circ.ahajournals.org/content/106/8/900.full

 

 

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