Hey all,
First, I'd like to thank all of you who have contributed to my daughter Kacey's efforts for the American Heart Association. Through all of your help, she has raised $1,965 for the American Heart Association! As most of you know, the HeartWalk 2005 is occuring this weekend down in Charleston, SC. My wife, myself and Kacey will be attending this to show our support.
Kacey is just $35 shy of her $2,000 goal, so for any of you who wish to still contribute this week, you can get to her donation site here.
For those of you who wish to read about Kacey's Heart Defect, I've explained it in detail below.
Thank you all again for your support!
-Darin
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DETAILS FOR THOSE INTERESTED
THE HEART
In order to understand the problem and ultimately the "solution", I need to give a quick explanation of how the heart works. Your heart is split into 2 halves, the right half and the left half. Your right side of the heart is responsible for pumping blood into your lungs while your left side of the heart is responsible for pumping blood out to your body. Each half is split into two chambers. The top chamber is the Atrium. This is where the blood pools up and then eventually flows down through a valve into the Ventricle which is the "pump" of the heart half.
Generally, here's how the "flow" of blood goes through the heart. Two veins connect to the right atrium to deliver "blue" blood to the heart from the body. This blood is termed "blue" because it is oxygen depleated blood. One vein is the Superior Vena Cava which returns "blue" blood from your upper body and the other is the Inferior Vena Cava which returns "blue" blood from the lower body. The "blue" blood pools in the right atrium and then flows through, what's called a Tricuspid Valve, into the right ventricle. The tricuspid valve opens to let the "blue" blood into the ventricle, then when the ventricle starts to compress, it closes so the "blue" blood will be forced into the pulmonary artery instead of back into the atrium. The pulmonary artery looks kind of like a T. The bottom of the T is where the blood is coming from the right ventricle and the top splits off into the right and left pulmonary arteries, which go to the right and left lung, respectively. The blood goes into the lungs and flows through the little sacks in the lungs picking up oxygen and then exits the lungs by way of the Pulmonary Veins. The pulmonary veins carry the oxygen rich blood, or "red" blood, into the left atrium, where again, the "red" blood pools up. The left side of the heart has a valve called the Mitral Valve which works just like the Tricuspid Valve on the right side. This time, the Mitral Valve opens and lets the "red" blood flow into the left ventricle and as the ventricle compresses, the valve closes which forces the oxygen rich "red" blood up though the Aorta and out into the body. The "red" blood carries the needed oxygen to the cells throughout the body and returns back through the Superior and Inferior Vena Cava's to start the process all over again.
THE PROBLEM
Kacey has a rare heart defect known as Ebsteins Anomoly. This affects the right side of the heart, which is responsible for pumping blood to the lungs. Basically, in Ebsteins Anomoly, the Tricuspid Valve which seperates the Atrium from the Ventricle is pushed down into the Ventricle making the Atrium larger in size and the Ventricle smaller in size. The degree's of severity (i.e. how far pushed down into the ventricle it is) varies from case to case. In Kacey's instance, the displacement of the Tricuspid valve is severe, making the Ventricle almost non-existant when looking at an echocardiogram (essentially a sonogram/x-ray of the heart). Since the Tricuspid Valve is displaced and not located where it should, this also affects the valves ability to close correctly. In most cases, the valve does not close all the way, so when the right ventricle compresses, some blood is forced back up into the atrium, which is known as "regurgitation". So, not only is the ventricle extremely small to begin with, not all of the small amount of blood which enters into it gets pushed into the pulmonary artery as it should, some of it is regurgitated back into the right atrium. Some of you might be thinking that if the blood gets pushed back into the atrium from the ventricle as well as coming in from both vena cava, wouldn't the right atrium fill with pressure and eventually just "explode"? A legitimate question, however, our bodies have accomodated for such a scenario. Between the right and left atriums there is something called the ASD, or Atrial Septum Defect. This is basically a pressure release valve so if either side of the heart gets too much blood in it, it can "shunt" it from one side to the other. So, in Kacey's case, with blood getting pushed into the left atrium, the "blue" blood shunts over to the left atrium and mixes with the "red" blood. So between not much blood being carried through the lungs to get oxygen and the "blue" blood shunting to the left side and diluting the "red" blood, you can see why she has low oxygen saturation levels.
THE SOLUTION
The generally accepted solution is to perform a Modified Glenn's Procedure when the child is 6 months of age and follow that up with a Fontan procedure when the child is 2-4 years of age. In the Glenn's Procedure, they sever the Superior Vena Cava from the right atrium and "plug" it directly into the Right Pulmonary Artery. A "Modified" Glenn's Procedure, also known as a Bi-directional Glenn's Procedure is where they "plug" the Superior Vena Cava into the "trunk" part of the T of the Pulmonary Artery so that the "blue" blood from the Superior Vena Cava can flow to either the right or the left lung. As you know, the Superior Vena Cava is what is carrying the "blue" blood back from the upper part of the body. So by feeding this vein directly into the Pulmonary Artery, they are essentially bypassing the right atrium and pump all together. This works because the circulatory system is, well, circulatory. When the left side of the heart pumps and pushes blood up into the Aorta, that has a ripple effect through the whole circulatory system because as blood is pushed into the Aorta, the blood that was in the Aorta just get's pushed further along the arteries and then veins until it winds up coming out of either Vena Cava. The Fontan procedure is the same thing, but this time with the Inferior Vena Cava. So once both procedures are complete, the right side of the heart will essentially do nothing. There are other things which may be done during these procedures such as patching the ASD, etc, but the main concept of it is that they re-route the blood directly into the Pulmonary Artery which feeds into the lungs. Apparently the left side of the heart is the one that does most of the work anyway when we are older so the doctor's said if you have to have a problem with your heart, it's better to have it on the right side than the left.
They have been doing these procedures since the 1970's on adults, however, they don't know any long term impact on it since they have not been doing it on infants for that long. The general guesstimate is that it will shave 5-10 years off of the persons life in the end.
CURRENT STATUS
Well, Kacey had her Modified Glenn's Procedure back in March '05 when she was 6 months old. The procedure went well and only had minor complications following the procedure. Immediately following the procedure, Kacey developped a heart arrythmia where her heart would jump up to around 220-250 beats per minute. This happened several times after surgery and they were able to "pace her" out of it. What this consists of is, after surgery, they leave wires attached to the heart which extend out through the incision to the skin. By sending electrical impulses down through these wires, they can force the heart back into rhythm. This is very similar to how a pace maker works, except it's external to the body. They eventually put Kacey on a beta blocker to help prevent this rapid heart beat from occuring. She is still on this beta blocker today and the current plan is to keep her on it until she outgrows the dosage. Then they will put a halter monitor on her for like a week to see if the rapid heart rate returns. If it does, she will be on the beta blocker for the rest of her life. If it does not, then she will no longer need to be on the beta blocker. The other complication which occured was that excess fluid formed around the heart and pooled in the heart sac. This is known as an "effusion". If an effusion grows large enough, it can be problematic because it could compress the heart and minimize the expansion of it during beats. To treat this, first, they had to insert a drain into the heart sac and keep it under constant suction to remove any fluid which forms. Secondly, they put Kacey on a high dosage of anti-inflammatory medication (aspirin) as well as a steroid. Eventually the fluid subsided and the drain was removed.
In addition to the Modified Glenn's Procedure, the surgeon was able to make some modificiations to the Tricuspid valve and the end result was he was able to minimize the regurgitation substantially. Today, Kacey is doing fantastic! She's in the 90+ percentile of height, weight and head size (although, I blame the constant praise of her grandparents for this one ). She is currently maintaining oxygen saturations in the mid 90 percentile and the doctor's are still uncertain if she will need the Fontan Procedure. Although she is still slightly behind in her developmental process due to the set back, she is progressing every day! She's now crawling and is almost able to stand up on her own. You can see her on her website.
First, I'd like to thank all of you who have contributed to my daughter Kacey's efforts for the American Heart Association. Through all of your help, she has raised $1,965 for the American Heart Association! As most of you know, the HeartWalk 2005 is occuring this weekend down in Charleston, SC. My wife, myself and Kacey will be attending this to show our support.
Kacey is just $35 shy of her $2,000 goal, so for any of you who wish to still contribute this week, you can get to her donation site here.
For those of you who wish to read about Kacey's Heart Defect, I've explained it in detail below.
Thank you all again for your support!
-Darin
--------------------------------------------------------------------------------
DETAILS FOR THOSE INTERESTED
THE HEART
In order to understand the problem and ultimately the "solution", I need to give a quick explanation of how the heart works. Your heart is split into 2 halves, the right half and the left half. Your right side of the heart is responsible for pumping blood into your lungs while your left side of the heart is responsible for pumping blood out to your body. Each half is split into two chambers. The top chamber is the Atrium. This is where the blood pools up and then eventually flows down through a valve into the Ventricle which is the "pump" of the heart half.
Generally, here's how the "flow" of blood goes through the heart. Two veins connect to the right atrium to deliver "blue" blood to the heart from the body. This blood is termed "blue" because it is oxygen depleated blood. One vein is the Superior Vena Cava which returns "blue" blood from your upper body and the other is the Inferior Vena Cava which returns "blue" blood from the lower body. The "blue" blood pools in the right atrium and then flows through, what's called a Tricuspid Valve, into the right ventricle. The tricuspid valve opens to let the "blue" blood into the ventricle, then when the ventricle starts to compress, it closes so the "blue" blood will be forced into the pulmonary artery instead of back into the atrium. The pulmonary artery looks kind of like a T. The bottom of the T is where the blood is coming from the right ventricle and the top splits off into the right and left pulmonary arteries, which go to the right and left lung, respectively. The blood goes into the lungs and flows through the little sacks in the lungs picking up oxygen and then exits the lungs by way of the Pulmonary Veins. The pulmonary veins carry the oxygen rich blood, or "red" blood, into the left atrium, where again, the "red" blood pools up. The left side of the heart has a valve called the Mitral Valve which works just like the Tricuspid Valve on the right side. This time, the Mitral Valve opens and lets the "red" blood flow into the left ventricle and as the ventricle compresses, the valve closes which forces the oxygen rich "red" blood up though the Aorta and out into the body. The "red" blood carries the needed oxygen to the cells throughout the body and returns back through the Superior and Inferior Vena Cava's to start the process all over again.
THE PROBLEM
Kacey has a rare heart defect known as Ebsteins Anomoly. This affects the right side of the heart, which is responsible for pumping blood to the lungs. Basically, in Ebsteins Anomoly, the Tricuspid Valve which seperates the Atrium from the Ventricle is pushed down into the Ventricle making the Atrium larger in size and the Ventricle smaller in size. The degree's of severity (i.e. how far pushed down into the ventricle it is) varies from case to case. In Kacey's instance, the displacement of the Tricuspid valve is severe, making the Ventricle almost non-existant when looking at an echocardiogram (essentially a sonogram/x-ray of the heart). Since the Tricuspid Valve is displaced and not located where it should, this also affects the valves ability to close correctly. In most cases, the valve does not close all the way, so when the right ventricle compresses, some blood is forced back up into the atrium, which is known as "regurgitation". So, not only is the ventricle extremely small to begin with, not all of the small amount of blood which enters into it gets pushed into the pulmonary artery as it should, some of it is regurgitated back into the right atrium. Some of you might be thinking that if the blood gets pushed back into the atrium from the ventricle as well as coming in from both vena cava, wouldn't the right atrium fill with pressure and eventually just "explode"? A legitimate question, however, our bodies have accomodated for such a scenario. Between the right and left atriums there is something called the ASD, or Atrial Septum Defect. This is basically a pressure release valve so if either side of the heart gets too much blood in it, it can "shunt" it from one side to the other. So, in Kacey's case, with blood getting pushed into the left atrium, the "blue" blood shunts over to the left atrium and mixes with the "red" blood. So between not much blood being carried through the lungs to get oxygen and the "blue" blood shunting to the left side and diluting the "red" blood, you can see why she has low oxygen saturation levels.
THE SOLUTION
The generally accepted solution is to perform a Modified Glenn's Procedure when the child is 6 months of age and follow that up with a Fontan procedure when the child is 2-4 years of age. In the Glenn's Procedure, they sever the Superior Vena Cava from the right atrium and "plug" it directly into the Right Pulmonary Artery. A "Modified" Glenn's Procedure, also known as a Bi-directional Glenn's Procedure is where they "plug" the Superior Vena Cava into the "trunk" part of the T of the Pulmonary Artery so that the "blue" blood from the Superior Vena Cava can flow to either the right or the left lung. As you know, the Superior Vena Cava is what is carrying the "blue" blood back from the upper part of the body. So by feeding this vein directly into the Pulmonary Artery, they are essentially bypassing the right atrium and pump all together. This works because the circulatory system is, well, circulatory. When the left side of the heart pumps and pushes blood up into the Aorta, that has a ripple effect through the whole circulatory system because as blood is pushed into the Aorta, the blood that was in the Aorta just get's pushed further along the arteries and then veins until it winds up coming out of either Vena Cava. The Fontan procedure is the same thing, but this time with the Inferior Vena Cava. So once both procedures are complete, the right side of the heart will essentially do nothing. There are other things which may be done during these procedures such as patching the ASD, etc, but the main concept of it is that they re-route the blood directly into the Pulmonary Artery which feeds into the lungs. Apparently the left side of the heart is the one that does most of the work anyway when we are older so the doctor's said if you have to have a problem with your heart, it's better to have it on the right side than the left.
They have been doing these procedures since the 1970's on adults, however, they don't know any long term impact on it since they have not been doing it on infants for that long. The general guesstimate is that it will shave 5-10 years off of the persons life in the end.
CURRENT STATUS
Well, Kacey had her Modified Glenn's Procedure back in March '05 when she was 6 months old. The procedure went well and only had minor complications following the procedure. Immediately following the procedure, Kacey developped a heart arrythmia where her heart would jump up to around 220-250 beats per minute. This happened several times after surgery and they were able to "pace her" out of it. What this consists of is, after surgery, they leave wires attached to the heart which extend out through the incision to the skin. By sending electrical impulses down through these wires, they can force the heart back into rhythm. This is very similar to how a pace maker works, except it's external to the body. They eventually put Kacey on a beta blocker to help prevent this rapid heart beat from occuring. She is still on this beta blocker today and the current plan is to keep her on it until she outgrows the dosage. Then they will put a halter monitor on her for like a week to see if the rapid heart rate returns. If it does, she will be on the beta blocker for the rest of her life. If it does not, then she will no longer need to be on the beta blocker. The other complication which occured was that excess fluid formed around the heart and pooled in the heart sac. This is known as an "effusion". If an effusion grows large enough, it can be problematic because it could compress the heart and minimize the expansion of it during beats. To treat this, first, they had to insert a drain into the heart sac and keep it under constant suction to remove any fluid which forms. Secondly, they put Kacey on a high dosage of anti-inflammatory medication (aspirin) as well as a steroid. Eventually the fluid subsided and the drain was removed.
In addition to the Modified Glenn's Procedure, the surgeon was able to make some modificiations to the Tricuspid valve and the end result was he was able to minimize the regurgitation substantially. Today, Kacey is doing fantastic! She's in the 90+ percentile of height, weight and head size (although, I blame the constant praise of her grandparents for this one ). She is currently maintaining oxygen saturations in the mid 90 percentile and the doctor's are still uncertain if she will need the Fontan Procedure. Although she is still slightly behind in her developmental process due to the set back, she is progressing every day! She's now crawling and is almost able to stand up on her own. You can see her on her website.