The Diagnosis

Lisa with Abigail her second night at Primay Children's.

At long last, here’s the diagnosis as it was explained to Quinn and I at that first evening. I will speak in very crude, elementary, possibly anatomically incorrect language, which is how I understand this stuff. The heart, though made up of multiple chambers, is essentially divided into two halves: the right and the left chambers. Each chamber has a main artery—the pulmonary for the right and the Aorta for the left. Those chambers and their corresponding arteries serve the following purposes. Blood flows into the right chamber from the body and the brain. The blood that flows into the right chamber is relatively blue and oxygen depleted, and it comes into the chamber for the purpose of receiving oxygen. The right chamber then pumps its newly received un-oxygenated blood into the Pulmonary Artery, which takes the blood into the lungs where it receives its much wanted oxygen. From there, the now red and oxygenated blood travels into the left chamber of the heart, which then pumps its newly received blood into the Aorta. The Aorta then takes the blood and ships it out to the rest of the body, delivering freshly invigorated, oxygenated blood to all of the other organs and extremities. Without that oxygenated blood, we simply cannot survive.

Abigail’s problems start with the two main arteries—The Pulmonary and the Aorta (the two super highways of blood transport in the body). In Abigail’s heart, the plumbing is simply backward. The Pulmonary Artery is linked with the left chamber and the Aorta is linked with the right—just the opposite of what they should be. The right chamber is getting new blood from the body, and then pumping it out through the Aorta, which then sends that same “blue” blood back into the body without having gotten any oxygen from the lungs. You all know that my children bleed blue, but Abigail is bordering on fanaticism! The left chamber is sending blood through the Pulmonary Artery into the lungs, and then, getting it right back into the left chamber, only to send it right back in to the lungs. So, the oxygenated blood is flowing in circles, not directly getting back into the body, where it needs it.  This condition is called Transposition of the Great Arteries.

Fortunately, there’s a hole—rather, a valve opening— in the wall the separates the top of the two chambers that allows blood to flow between the right chamber and the left chamber. Thus, some of the highly oxygenated blood from the left chamber is getting mixed with the bluer blood from the right chamber, and that is how Abigail is getting some oxygenated blood into her body. That hole or valve typically closes within weeks of the birth, but right now it remains open, and right now, it is saving Abigail’s life.

Here’s how they will solve the first and largest problem—the fact that the Aorta and Pulmonary Artery are linked to the wrong chambers. They will actually go and move the arteries into the opposite chamber. During that process, they must be extremely careful not to damage the coronary arteries, which come off the Aorta and supply the heart organ itself with needed blood. They will have to locate the coronary arteries, cut them out cleanly, and then re-attach them to the heart and Aorta which will have been moved the left chamber. In most cases they cut a hole around the coronaries, being careful not to actually cut the coronaries themselves.

The second problem that Abigail faces is that there are multiple holes in the septum, the wall that separates the two chambers.  (We later learned that her septum had the appearance of swiss cheese).  There was one very large hole in particular that was close to the great arteries, the position of which makes a possible repair technically complicated.  Also, for some reason this hole does not aid much in the mixing of the blood that the top hole facilitates. This hole has to be patched.

The third problem is that the Aorta narrows significantly as it gets further from the heart. That narrow section of the Aorta--ac condition called coarctation--could restrict blood flow to the body. That narrow section will need to be removed, and then the surgeons will connect the two large sections of the Aorta together.

Two Procedures to Fix the Problem: From Salt Lake to Palo Alto

Prior to open heart surgery, the cardiologists will perform a procedure known as a catheterization. They will insert a catheter of some kind into her groin, through an artery where it will travel up into her heart. The purpose of the procedure is twofold: (1) to inject die into various places in the heart, which will allow them to take clear pictures (I assume with an X-Ray machine) of a few things such as the exact placement of the coronaries, and (2) to widen (and keep from closing) that all important hole that is allowing the blood to mix in the upper chambers. They will enter the heart through the right chamber, pass through the “good” hole into the left chamber, inflate a balloon at the end of the catheter, and then pull it back through the hole, which will essentially rip open a larger hole in the chamber wall, allowing for a more free flow and mixing of the blood between both chambers. This is sort of a temporary fix that will ensure higher oxygen saturation levels until she can have her second procedure, open heart surgery.

The cardiologists at Primary Children’s told us that heart surgeons here have a vast amount of experience in dealing with each of these three individual issues, and that if Abigail’s heart had just one of these complications then they would feel comfortable performing the surgery to fix the problem.  However, the level of complexity to the open heart surgery rises exponentially when all three conditions exist simultaneously which makes the surgeons here think that they aren’t qualified to perform the surgery. In particular, the placement of the large hole in the septum and its proximity to the great arteries will make any repair that much more technically difficult.

The former senior thoracic surgeon at Primary Children’s, a highly regarded and experienced surgeon in such matters was recently diagnosed with pancreatic cancer, and is no longer working. He would have had the ability and expertise to perform the operation, but he is no longer working. Therefore, Primary Children’s is going to send Abigail to Stanford Medical Center in Palo Alto, CA, where they have two surgeons with enough experience in the handing these simultaneous issues. So, sometime in the near future, Abigail will be loaded into her own private jet and flown to Palo Alto for heart surgery (I am purposely not adding up the cost of all of this, as at this point I have no idea what my insurance will and will not cover, because at the moment, that is irrelevant—I will be in debt for life if necessary to attend to Abigail’s heart—does anyone know a good loan officer?).

At the moment, we’re waiting for the first procedure that should be performed this afternoon. It was scheduled for yesterday afternoon, then bumped to last night, then bumped to this morning, and now it has been bumped again till this afternoon. The good news is that that means that she’s fairly stable; the bad news is that the longer they wait to perform this procedure the longer that they will likely wait for her to be transported to Stanford. I guess that God knows that I need to continue to learn patience.