Harlie's Heart 101

For those of you more interested, you can read this very long, medical-heavy entry that talks more about Harlie's heart defects and how her heart is different from a normal heart. For those of you less interested, just skip it.

Here is a list of her heart defects:

1. Congenitally Corrected Transposition of the Great Arteries: Her heart was formed in a mirror image. Meaning what's normally on the right side of the heart, is on the left, and what's normally on the left side, is on the right.

2. VSD: Ventricular Septal Defect: An opening between the left and right ventricles which causes the blue and red blood to mix. In a normal heart there is no opening and the blood is completely separate.

3. Hypoplastic Right Ventricle. Her right ventricle is smaller than it should be.

4. She has two small Superior Vena Cavas (SVCs). In a normal heart, there is one, large SVC which carries the blood from your head to the right atrium. It was this defect (combined with defect #3) that prevented the normal "fix" for her CCTGA (called a Double Switch) and lead us to the Fontan.

4. Subaortic Stenosis: A narrowing of the passage way from her right ventricle to the aorta (blood going to the body). They said that her blockage is from tissue that has grown over the passage way.

5. Intermittent Second Degree Heart Block: In most cases of CCTGA, the electrical connections in her heart are compromised. Often times, this requires a pacemaker. They anticipated this at her birth and during her first heart surgery at 4 days old, they placed the pacemaker leads for future connection. It is unknown at this time if she will need the pacemaker to be connected after the Fontan.

The Normal Heart

In a normal heart, the right atrium receives blood returning from the body. There is a "pipe" that carries the blood from your head called the Superior Vena Cava and a "pipe" that carries the blood from the rest of the body called the Inferior Vena Cava. Both of these pipes empty the blood into the right atrium. This blood is low in oxygen (blue).

It flows into the right ventricle, and is then pumped to the pulmonary artery to the lungs. In the lungs it picks up oxygen (red) then flows through the pulmonary veins to the left atrium and into the left ventricle. It is then pumped out of the aorta to the rest of the body.

In Brief:

Blue blood from body (IVC) and head (SVC) go to right atrium, to right ventricle, to pulmonary artery, to lungs, to left atrium, left ventricle, aorta, then body. Repeat. For many, many, many years!

Right now Harlie's blood flows like this:

Blue blood from body (IVC) to left atrium, to left ventricle to right ventricle to aorta, to body (notice NO lungs are mentioned).

And

Blue blood from head (SVC) to lungs, to right atrium, to right ventricle to aorta, to body.

The Bi-lateral, Bi-Directional Glenn

In her 2nd heart surgery (called the Glenn) the surgeon disconnected her SVCs from feeding blood into her left atrium. Then he connected her SVCs to the pulmonary artery, so blood coming from her head would go straight to her lungs to receive oxygen and then would go to her heart to be pumped out to her body.

Basically, in a normal heart there are two ways for the blood to leave the heart - the pulmonary artery (going to the lungs) and the aorta (going to the body) and they leave from two different ventricles. The small ventricle (right) pumps to the lungs and the large ventricle (left) pumps to the body.

In Harlie's heart, there is only one way for the blood to leave her heart - the aorta (going to the body). Because she essentially only has one ventricle because of her large VSD.

The Fontan

In the Fontan procedure, the surgeon will disconnect the IVC (blood coming from her body and feeding into her left atrium) from her left atrium and will connect it directly to her pulmonary artery so that blood coming from her body will go straight to her lungs to receive oxygen, then will go to her heart to be pumped to her body again.

So, her new circulation will go like this:

Blood from body and head will go to pulmonary artery, lungs, right atrium, her one ventricle and out the aorta to her body. Repeat. For many, many years.

The Damus-Kaye-Stansel Procedure (DKS)

The DKS is the creation of a connection (made of donated tissue or Gore-Tex) between her left ventricle to her aorta. It will give the blood another way out of her combined ventricles to the aorta, to her body.

That's it! Got it? Piece of cake.