Chaseton’s story.
Chaseton came into this world on September 23rd at 10:34 pm in a natural
water birth. At birth he weighed 7.15 pounds and was 20.5 inches long.
After passing all of his post-birth tests, he greeted all four of his grandparents
along with his two aunts with wide eyes and a studious expression. After all
the visitors departed, Chase spent the night with his parents Sammy and
Brandon
Around 8 am, Chaseton’s pediatrician came in for a
routine visit. While listing to his heart the doctor noticed a murmur and
took Chase back to the nursery for a closer examination. It was quickly
determined that Chase had issues with his heart and needed care beyond the
hospital’s ability to provide. An ambulance with a team who specializes in
the transportation of infants was called to move Chase to the hospital in
Macon, GA. After further testing in Macon, it was decided that Egleston
Hospital in Atlanta was the best place to treat his problems. After a short
19-hour stay in Macon, Chase was transported to Atlanta.
Once Chase arrived in Atlanta, we received the first clear picture of
exactly what was wrong with his heart. Chase is suffering from Transposition
of the Great Arteries, (TGA) along with a defect in his ventricular septum
(VSD).
What does that mean, you ask?
I will try give an oversimplified explanation of a rather complex problem.
Transposition of the Great Arteries (known from now on as TGA) is a lot
like it sounds. In a normal heart the pulmonary artery is attached to the
right ventricle and pumps oxygen-depleted blood up to the lungs. The aortic
artery is connected to the left ventricle and pumps oxygen-rich blood coming
from the lungs out to the rest of the body. In TGA, those two arteries are
switched. The pulmonary artery is connected to the left ventricle and is
pumping oxygen-rich blood from the lungs back into the lungs. While the
aorta artery is pumping oxygen-depleted blood coming from the body back out
to the body again. This is not compatible with life.
Fortunately for Chase, he also has a VSD – a small hole in the wall between
the left and right ventricles. This hole is allowing oxygen-rich blood from
the left ventricle to enter the right ventricle, mix with the oxygen
depleted blood, and to be pumped out of the aorta to the rest of his body.
This mixture is directly responsible for keeping Chase with us.
The fix:
The first option for repairing the TGA and VSD is an operation called the
Jatene Procedure, where the two arteries are cut just above the valve
leading into the heart and sewn back into their correct position. There is
more to the operation but that is the basics. The prognosis for full
recovery from the Jatene Procedure is very good; 98%-99%.
While studying Chase’s latest echocardiogram the lead surgeon discovered a
third problem called Pulmonary stenosis. Pulmonary stenosis is a partial
blockage or obstruction in the pulmonary artery. Sometimes that can be
fixed during the Jatene procedure. In Chase’s case the obstruction is below
the pulmonary valve and can not be cleared. While a small blockage in the
pulmonary artery is not a severe problem, if they had performed the
operation that valve would have been attached to the aorta. A small blockage
in the aorta is not a good thing at all. The lead surgeon made the decision
that Jatene Procedure was no longer a viable option.
Now what?
There are two other surgical procedures that can fix the problem. The first,
most commonly implemented fix, is called the “Rastelli Procedure”. Imagine
the wall between the two ventricle as a wall between two equal size rooms
with a door that is not supposed to be there. The first step of the
operation is to move the wall back into the right ventricle enough to expose
the opening of the aorta. Then they seal the hole in the wall with a patch.
So now you have two rooms, one slightly larger that the other, with no door
between them. The aorta is now located in the left ventricle. The next step
is to detach the pulmonary artery, increase its length with a donated vessel
(that will make it long enough to be moved over to the right ventricle)
where it will be attached. The survivability of the Rastelli procedure is
about the same as the Jatene Procedure. There are, however, some added
long-term complications. First, the vessel used to lengthen the Pulmonary
artery will not grow with the rest of Chase’s body; eventually it will have
to be replaced with a larger one. The second is that in some patients the
patch used on the ventricle wears out and must be replaced. There are also
some patients who end up needing a pace maker later in life.
The second option is called the “Nikaidoh Procedure”. It is a relatively new
procedure that is not yet widely implemented. First they cut the pulmonary
artery and remove the pulmonary valve. Next, they remove the aortic artery –
keeping the valve attached. Then they place a patch of pericardium from the
sac around the heart to both close the VSD and to widen the vessel that
flows out of the left ventricle. This widening is just like if you cut open
a letter “o”–so it looks like a backwards “c”–and then sewed it onto a
letter “l” to form a letter “D. Then they insert the aortic artery in the
hole left by the pulmonary valve and reattach it. The tricky part is that
all the coronary arteries that feed blood to the heart are attached to the
aorta and when the aorta is moved it can add strain to those vessels. If
that is the case then each of the coronary arteries must also be relocated.
Next, they cut a slit into the narrow part of the pulmonary artery and sew
in a patch of pericardium in the shape of a triangle and sew it into the
slit with the base of the triangle at the narrow point of the artery. ^ This
helps to make the artery more uniform in size. Then the pulmonary artery is
attached to the right ventricle as it would be in a normal heart. After
Chase has grown to his adult size a replacement pulmonary valve would be
inserted using a heart catheter. The long-term affects of this procedure are
not as clear as they are for the Rastelli. There are no donated parts so
every thing will grow at the same rate. Early studies seem to show that
there are fewer long-term consequences from this procedure…but the
Nikaidoh procedure is more complicated and invasive.
What procedure will we use and when will it be done?
Now that is the big question. Neither procedure can be performed until Chase
is older. Figure 6-12 months old. As to what procedure will be performed,
the parents do have some say in the matter but would have to “shop around”
to find a surgeon that will performed the Nikaidoh. The hospital we are at
now is one of the top in the nation and they have only done it a few times.
(The number “4” comes to mind but I’m not sure that is correct.)
What happens now?
For the next few weeks (and that is a very flexible number) Chase will stay
in the hospital, in the CICU. (Cardiac Intensive Care Unit) They will slowly
remove him from his respirator. If he handles that well (he is doing very
well at this time) they will move him to the “step down” unit. It is just a
little less than Intensive care. If he shows no further complications,
eventually he will be sent home to grow up enough to allow one of the fixes
to be implemented. Sammy and Brandon will receive extensive training on the
care of a cardiac infant (or “heart baby”) and he will be watched closely by
his cardiologist in Macon, GA.
The Story of Chaseton. 