I’ve been feeling more and more compelled to write Cora’s heart story. I don’t journal – for various personal reasons – but I don’t want to forget those early details and even the ones we’re experiencing now. So, I’m going to periodically come back and update the story of her birth, her diagnosis, her surgeries…as much as I can remember. If you have no interest in this technical and emotional babble - just ignore this page!
I think the best way to understand what’s going on with Cora’s heart is to first understand what SHOULD be going on. Here is the healthy heart’s flow:
Blood comes to the heart from two large veins, the superior and inferior vena cava. This blood is “blue” or unoxygenated and comes into the right atrim (upper chamber). The tricuspid valve pushes the blood into the right ventricle (lower chamber) where it is then ejected through the pulmonary valve and the pulmonary artery. The pulmonary artery leads the blood to the lungs so the unoxygenated or “blue” blood can get it’s oxygen. Once the blood is oxygenated, this now “red” blood is returned through the pulmonary veins into the left atrium (upper chamber) pushed by the mitral valve into the left ventricle (lower chamber) and then pumped through the aortic valve into the aorta where the oxygen rich blood is circulated to the body.
The above is a HEALTHY heart
This means the heart is positioned in the center of the chest instead of on the normal left side. The word means “heart in the middle of the thorax.
AVSD – Atrial Ventricular Septal Defect *or* Complete AV Canal
This alone is a complex heart defect. It’s best described as a large hole in the middle of the heart. Hopefully, you remember from 9th grade biology that the heart is divided into 4 chambers. Blood flows in one direction through the four chambers with the help of the four heart valves which are called the tricuspid, pulmonary, mitral and aortic valves. The chambers are the left and right atrium – the upper chambers – and the left and right ventricles – the lower chambers. In a healthy heart, the left and right sides are separated by a septal muscle wall and the upper and lower chambers are separated by valves.
Now, with AVSD, there is NO WALL of separation between the left and right chambers, the septal muscle wall is not there. There are other defects that involve a “hole” in the heart such as ASD or VSD – atrial or ventricular septal defects. The heart has a septal wall in place but there is a space or “hole” in the wall that allows blood to flow between the left and right sides of the atrium or ventricles. Sometimes there are “two” holes, one in each chamber. Cora doesn’t have ASD or VSD but rather a complete AVSD meaning that there is no septal wall to begin with.
Additionally, the valve between the atrial (upper) and ventricular (lower) chambers did not separate into separate mitral and tricuspid valves as it should. Instead, she has an over-riding valve that doesn’t close correctly and blood leaks backwards from the ventricular chambers (lower) into the atrial chambers(upper). This “leak” is called insufficiency of the valve or “regurgitation” and has lead to the enlargment of her heart.
PS – Pulmonary Stenosis
This pretty much means her pulmonary artery is too narrow to allow “blue” blood flow to her lungs. Her stenosis is on the severe end meaning she had very minimal blood flowing through that artery at birth and pretty much nothing at all now. The infundular part refers to the TYPE of PS she has. This one is where the muscle that is under the pulmonary valve is thickened and this narrows the outflow tract from the right ventricle.
TGA – Transposition of the Great Arteries
This is another biggie because it involves the two major arteries that move blood in and out of the heart – the aorta and the pulmonary artery. In TGA these two areteries are reversed. The aorta received the oxygen-poor blood from the right ventricle but it’s carried back to the body without receiving more oxygen. Likewise, the pulmonary artery receives oxygen-rich blood from the left ventricle but carries back to the lungs. Very counterproductive!
DORV – Double Outlet Right Ventricle
This a very rare Congenital Heart Defect (CHD). Normally, a ventricle has just ONE outlet and for the left ventricle this is the aorta, for the left it is the pulmonary artery. In DORV, both of these “outlet” blood vessels (the aorta and pulmonary artery) come out of the right ventricle.
SV – Single Ventricle
The normal heart has two ventricles but in Cora’s case, her left ventricle is poorly developed and this condition is called single ventricle or univentricular heart. Single-ventricle heart defects are among the most complex congenital heart problems known. She has two normal atria that open into the ventrical through an AV (atrio-ventricular) valve – the leaky one! This single ventricle connects with the aorta and pulmonary artery.
Overriding Aorta
This basically means that her aorta is malpositioned because it straddles her ventricular septum (or where it SHOULD be) and receives blood from both the left AND right ventricles.
Interrupted Inferior Vena Cava
The Superior Vena Cava brings the majority of blood flow from the upper part of the body, the Inferior Vena Cava brings it from the lower half. Cora’s Inferior Vena Cava does not connect directly to the heart as it should but instead enters the Superior Vena Cava via the azygos vein. No, I don’t have a picture of this and I’m not sure on the pronounciation.
PLSVA – Persistent Left Superior Vena Cava
|
Left Atrial Isomerism
This one I’m a bit fuzzy on the exact definition – it took me several weeks to learn to pronounce it correctly. My understanding is that the left side of her heart is “mirrored” onto what should be the right. In other words, the right side of Cora’s heart has the characteristics of what should be the left side. And her left side looks like a left side. Clear?
Right Aortic Arch
This seems to be just another “backwards” thing she has going on and it’s kinda small potatoes when viewed in light of her other CHD’s (Congenital Heart Defects).
*sigh*
I “think” that’s it but I need to go through some old paperwork to be completely sure. As you can see, it is a true miracle she is alive and with us today.
Next…I’ll tackle the surgeries.
