I'll start simple. The heart has 4 chambers: 2 upper ones called atriums and 2 lower ones called ventricles. Don't lose me here...In a normal heart, blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs, then blood returns to the left atrium from the lungs, passes into the left ventricle, then is pumped through the aorta out to the body.
Because Sofia was born without a tricuspid valve, blood can't get from the right atrium to the right ventricle and to the lungs as it should. No blood to the lungs? Houston, we have a problem. Essentially, there were walls where valves should have been. And surgeons cannot put a valve where there is a wall. So because blood can't travel through these valves because she doesn't have them, a surgical repair needed so that both a baby's body and lungs can get oxygen. This is usually staged in 3 surgeries. (It is kind of a moot point that she has pulmonary atresia in addition to the tricuspid atresia because she without the tricuspid valve, they would be bypassing the right ventricle anyway). She is referred to as a single-ventricle patient because she only has a functional left ventricle. There are several types of single ventricle anatomies and tricuspid atresia is just one of a few. And there are different variations of tricuspid atresia too.
In her first surgery that she had at 4 days old, she had a BT shunt put in which allowed her lungs to get oxygen. A connection was made, which allowed some of the blood traveling through the aorta towards the body to "shunt" through this connection and flow into the pulmonary artery to receive oxygen. This is a temporary fix which allowed her to grow before having her next surgery. A baby will eventually outgrow the shunt because it is made of Gortex and does not grow with the body.
Her second surgery, called the Glenn, was done at 5 1/2 months old. Babies are more stable after the Glenn because the surgery reduces the left ventricle’s workload and sets the stage for the Fontan Procedure to come. In her Glenn surgery, they took down the shunt and and replaced it with different connection to the pulmonary artery. They basically took her superior vena cava (the large vein that returns oxygen-poor blood from the head and arms back to the heart) and surgically connected it to the right pulmonary artery so that blood can get to the lungs to receive oxygen.
Is anyone still with me? We're almost there...
This final operation, the one she is having in May, is call the Fontan (named after the surgeon who invented it). The principle of the procedure is that it's not necessary to have a ventricle that pumps to the lungs so long as the lung arteries have a low resistance, are well developed and are of good size, and so long as the single ventricle fills at a low pressure. Surgery involves directly connecting the returning blue blood into the pulmonary arteries. This can be done in a few different ways, and Sofia will have what is called the extra-cardiac Fontan, involving a synthetic Gortex tube called a conduit that will be big enough for her to live with as an adult. She will also be pink after the surgery because she will have more oxygen rich blood where she needs it and her pulse ox will be near normal. I should also mention that these surgeries have been modified over the years to get the best outcome, so a Fontan isn't the same in all T.A. patients.
Because her blood will be passively getting to her lungs, not pumped by the right ventricle, like yours and mine, she needs to be on a thinner (right now just aspirin) to prevent clotting. Aspirin is the only medication she is on. She is at risk for developing arrhythmias (abnormal heart rhythms) that could have to be treated with medication or pacemaker down the road. We pray that everything continues to go smoothly for her. We are thankful that she has a functional left ventricle which was designed to pump to the body. Some babies that are born with only a working right ventricle (opposite of Sofia's and is called hypoplastic left ventricle), which was only designed to only pump to the lungs,, can have more difficulties and their first surgery is generally more complicated.
There you have it. Modern medicine and surgery. Please don't ask me to repeat this without my notes.
http://www.childrenshospital.org/az/Site508/mainpageS508P4.html
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