A Little Medical Lingo

Things have been going pretty well. Not a whole lot to report from Children’s these days, which is good. We are just preparing for the surgery on Wednesday. I thought I would take a little time to explain the procedure that will take place.
Claire’s official diagnosis is the following…

Cardiologist's drawing from 3-30-10

Unbalanced AV Canal defect: Unbalanced with a single dominant ventricle and a second hypoplastic ventricle. This results in essentially single-ventricle physiology (so what does this mean) she only has the right side of her heart and all of the necessary veins and arteries connect to these two chambers.

Large ASD (atria septal defect): large hole developed between the right and left atriums.

Pulmonary stenosis: shrinking of the pulmonary valve and arteries

Essentially all babies with a single ventricle defect receive the following three surgeries:
1. Shunt placed between dorsal aorta and the pulmonary artery. (typically first week)
2. Glenn Procedure (typically around 6 months)
3. Fontan Procedure (typically around age 2)

Surgery Number 1
The first surgery is used to keep the PDA (patent ductus arteriosus) open to allow just the right amount of blood to travel to the lungs. The ductus arteriosus is a normal part of the circulation of the fetus. It is a blood vessel that connects the two large arteries coming out of the heart, the aorta and the pulmonary artery. After birth, this duct usually closes but little Miss Claire has received prostaglandins to keep her PDA open (Aprostadil is also used in maintaining a patent ductus arteriosus in the newborn. This is primarily useful when there is threat of premature closure of the ductus arteriosus in an infant with ductal-dependent congenital heart disease). The side effects of this medication have been increased temperature and apnea.

We believe that the surgery will take 4-5 hours. A good bit of the first hour will be specific to preparation (getting arterial iv lines, sedation, etc.) The cardiothoracic surgeon will start with a small incision on her right side. They will separate her ribs and gather a pathway to her small little heart. The shunt will be connected between the dorsal aorta and the pulmonary artery. The shunt should provide just enough blood flow to her lungs. This is a fine specific system. We do not want too much blood flow to her lungs, but we want just enough to keep her oxygen saturation within an acceptable range. If there is too much blood flow to the lungs, they can become over filled and cause lung problems. Obviously, if there is not enough blood flow to the lungs, she will not have enough oxygen in her blood to sustain her cells.

Crazy that we have the technology available to perform such surgeries and live in a country that believes all life is valuable.
Given that you now have at your disposal the World Wide Web and can Google any of the above information, I feel obliged to tell you that a lot of the information out there is not super current. Research has made great strides in treating little ones with these disorders. The Frame Family chooses to steal all optimism from the doctors and nurses as they share their positive outlook for our baby girl.

We appreciate your thoughts and prayer.