Ashley's Heart Conditions

We've been keeping a pretty accurate log of everything that's been done for Ashley since she was first admitted to the hospital. We figure it might come in handy should any complications arise when she's older, and her present condition is all a distant memory. As part of that record, the following is what we know so far about Ashley's heart issues. As she develops, most of these issues will repair themselves, but a few of them she'll have to live with for the rest of her life.

1. Coarctation of the Aorta. A coarctation is a fancy word for a narrowing along the aorta. Ashley had surgery to repair the narrowing in Edmonton. It was bypass surgery, so the surgeons had to stop her heart and then snip the narrow part of her aorta out through an incision between her ribs, under her left arm. They then reattached the two pieces of aorta together in a way that is supposed to inhibit the formation of scar tissue, which can cause major problems in the future if left unchecked. There is no sign of scar tissue, and her arch looks really good on all the echos she's had. The surgeons did an excellent job, and she probably won't require any further surgeries on that particular part of her heart.

2. Parachute Mitral Valve. A normal mitral valve is connected to the bottom of the left ventricle by two muscles. Ashley's is only connected by one muscle, which makes it resemble a parachute. It functions well as it is, and isn't causing any problems with her circulation at all, so she'll probably just live with it for the rest of her life, and never know the difference.

3. Bicuspid Aortic Valve. A normal aortic valve has three flaps of tissue that close up to prevent back flow into the left ventricle. Ashley's only has two flaps, but they close up tightly and show no signs of leakage, so it's just another anomaly that she'll live with for the rest of her life. My Dad also has a bicuspid aortic valve, and has suffered no ill effects because of it throughout his life - unless it's related to the telling of really corny jokes. Oh please don't let it be so.

4. Ventricular Septal Defects (VSD's). These are basically holes in her heart. She has two of them between her right and left ventricle walls. They cause a distinct murmur when you listen to her heart, which is the sound of blood passing through the holes, and mixing in ways that it really shouldn't. These holes were not repaired at the time that her coarctation was removed, as they require open heart surgery to fix. The hope is that these holes will close up by themselves as Ashley's heart grows and more tissue is formed. If by the time she is 6 months old they have not closed up, she will undergo open heart surgery and they will be patched up manually. We're really hoping and praying that that procedure will not have to be a part of her future. It's pretty invasive, there's a lot of risks involved, and the recovery time isn't short, or pleasant.

5. Pulmonary Artery Band. To help with the closing of the VSD's, the surgeons placed a band around Ashley's pulmonary artery. The band restricts the amount of blood leaving the right ventricle and out to the lungs, which increases the pressure in the right ventricle. The increase of pressure is supposed to limit the amount of blood flowing through the VSD's from the left ventricle into the right, giving the VSD's a better chance of closing up by themselves. If this works, Ashley will still have to have another surgery in 6 months to remove the band, but they'll probably be able to go in through her existing incision, and they won't require her heart to be stopped to do it.

Ashley's left function of her heart is still weaker than it should be, but the 4 medications she's on are supposed to help her with that. As she grows, her heart will naturally get stronger as well, and she'll be able to function properly without the aid of medications or oxygen.

A lot of Ashley's condition and treatment still seems like a bit of guess and test work, and I have a feeling there are some mysteries yet to be solved. I've been doing a bit of research on the heart lately, and the other day I came across something called Shone's Syndrome. Shone's Syndrome is a combination of 4 different heart defects all occurring all at once, 2 of which we already know that Ashley has (the coarctation and the parachute mitral valve). The other two defects are a little more subtle, and potentially could have been overlooked, so I'm going to bring it up with our cardiologist the next time we see her. I guess one thing I've found through all of this, is that it doesn't hurt to do a little investigating of your own, and to involve an educate yourself as much as possible.