Tricuspid Valve Atresia with Malposed Great Vessels
I’m going to memorize that at some point I’m sure.
So we finally were able to meet with Dr. Roten. She was able to see that there were four chambers,
but the Great Vessels had basically switched up so that the right ventricle didn’t really increase in size because of the way it all worked they weren’t being used. So, four chambers but the right ventricle is basically useless.
The left ventricle is strong, which Dr. Roten said is a GOOD thing. The road ahead is long, but before you get too worked up, Amanda and I are feeling good about where we are at right now.
So how do we fix this? Well, it’ll never be perfect but the idea is that Rhett will have (3) surgeries before the age of 3.
The first surgery will be when he is a few days to a week old. The second around four months old. The third around 3 years old which will hopefully be the last one.
Amanda will still deliver vaginally if everything goes according to plan and then we will have just a minute or so for her to hold him and take a few pictures. After that he will be loaded onto his cadillac (the nurse called them that) and they will roll him across the skybridge (from Harris Downtown) to Cook Children’s and he’ll be taken to his NICU room which will be ours for a few days until he is taken to the CVICU (Cardiovascular ICU) where he will have his first surgery which is called The Norwood Procedure. In one of these two rooms is where I’ll be sleeping the first night but I’m not sure.
In tricuspid atresia, there’s no tricuspid valve so no blood can flow from the right atrium to the right ventricle. As a result, the right ventricle is small and not fully developed. Survival depends on there being an opening in the wall between the atria (atrial septal defect) and usually an opening in the wall between the two ventricles (ventricular septal defect). As a result, the venous (bluish) blood that returns to the right atrium flows through the atrial septal defect and into the left atrium. There it mixes with oxygen-rich (red) blood from the lungs. Most of this poorly oxygenated mixture goes from the left ventricle into the aorta and on to the body. The rest flows through the ventricular septal defect into the small right ventricle, through the pulmonary artery and back to the lungs. Often in these children it’s necessary to do a surgical shunting procedure to increase blood flow to the lungs. Some children with tricuspid atresia have too much blood flowing to the lungs. They may need a procedure (pulmonary artery banding) to decrease blood flow to the lungs. Other children with tricuspid atresia may have a Fontan procedure. In this, a connection is created between the right atrium and pulmonary artery. The atrial defect is also closed. This eliminates the cyanosis but, without a right ventricle that works normally, the heart can’t work totally as it should.