On January 3, 2011, we headed down to Rady's Childrens Hospital so that Maren could see Dr. El Said and so we could conference regarding the results of the December 8, 2010 MRI. Although we had been told the results were very positive, we really wanted to talk directly with Dr. El Said about the numbers and details of the MRI, and find out what she envisioned as Maren's treatment plan going forward. As discussed below (skip to conclusion if you like) the bottom line is no surgery this year, and if Maren stays on her current super healthy healing path, she will require only catheter surgery one or two times in the foreseeable future.
MEDICAL DETAILS:
-Oxygen Levels: After getting Maren's height: 31.25 inches, weight: 10.2kg, they took a reading of Maren's oxygen and it was 94/95. This is pretty amazing, as she had been 90 just 6 months ago. (Normal is 97-99).
- Right Ventricle Size: Dr. El Said told us that the MRI showed that the ventricle has grown "significantly". Her determination of its growth is somewhat subjective because this was Maren's first MRI. Thus, there is no baseline MRI result for comparison. (Recall, the MRI is the only test that provides an accurate reading of the volume of the ventricle.) Dr. El Said's determination that the right ventricle has grown significantly is premised in large part on her piecing together all the information she has about Maren's heart including oxygen levels, her generally excellent health, as well as the MRI results. Equally important in assessing Maren's right ventricle is a direct comparison of the volume of the right ventricle with the volume of the left ventricle. Assuming the left ventricle is normal, which Maren's appears to be, the two ventricles should be about equal in size. In Maren's case, her right ventricle appears to be about 2/3 the size of her left ventricle. In sum, this is all excellent news according to the doctors.
- Leakage: Heart valves help to insure blood flows only one direction. A valve that is not working properly will have some leakage/regurgitation where a small amount of the blood flows backwards. The backward leakage in Maren's pulmonary valve and her tricuspid valve is rated +1. This rating is excellent for a girl like Maren. These are issues that could become pronounced over time, but right now, this is very close to normal, and is something the doctors will just continue to keep an eye on. In fact, the leakage at the pulmonary valve helps to increase the right ventricle size, and so has had a side benefit at this point.
-Right Outflow Tract/Pulmonary Flow: In addition to a small right ventricle, another concern has been stenosis of the pulmonary artery where the conduit of blood from the right ventricle through the pulmonary valve is potentially narrow and obstructed so that blood does not flow well. We learned that Maren has only minor issues with this right outflow tract obstruction. The gradient across her pulmonary was around 12mmHg, with normal being 6mmHg, where problems arise in the high 20s. Last October, when Maren had a catheter surgery to reballoon /open her pulmonary valve, this same gradient measurement was in the 30s. Along this line of reasoning and observation, Dr. El Said says Maren's pulmonary artery is not too narrow.
- Tricuspid Valve Z Score: Maren's tricuspid valve Z Score came in as a -0.08 today. This is great news, as tricuspid valve size is a very important indicator of the potential for a PAIVS baby like Maren to see ventricle growth. The tricuspid valve sits at the entrance to the right ventricle. Its performance is critical to getting proper blood flow through the right ventricle. Studies have shown a relationship between tricuspid Z scores and the recovery of PAIVS babies. Thus, many hospitals set up treatment plans based in large part on the tricuspid valve Z score. Most say that a Z score between 0 and -2 is mild, a Z score between -2 and -4 is moderate, and -4 and below is severe. So to see Maren's Z score close to 0 is pretty exciting.
- PDA Stent: According to the tests, the stent they inserted in May 2009 as a "cheat" connection is still open, with some flow. But the doctors seem to think this is not a concern, it will close naturally in time, and that Maren does not actually need the stent at all at this point.
-Atrial Septal Defect: Adding up all of the above information, the outlook for Maren was amazing today. The doctor told us that her immediate focus is on the hole in Maren's heart between the right and left atrium ( known as an atrial septal defect or "ASD"). This extra connection has never been even a blip on the radar in view of our legitimate concerns about right ventricle overhaul, valve replacements, 1.5 heart plans, etc. This ASD is apparently giving a slight right to left shunting of blood and ultimately they will want to close it via a catheter surgery. Our doctor thinks this could be done now, but she is reluctant to change anything, since Maren is doing so well.
CONCLUSION:
In sum, Dr. El Said said she did not need to see Maren for another year! Of course, we told her that was ridiculous, and convinced her we should come back in 8 months. Long term, if Maren can stay on her current track, we are looking at catheter surgery in the next year or two to close the hole in her heart (ASD), and at the same time close the PDA stent. After that, she said Maren will at some point need a pulmonary valve replacement, but the timeline for that procedure covers a very very broad spectrum. Thus, some patients may need a new valve as a teenager, some not until their 30s.
At the moment Maren is on the two ventricle heart plan with no open surgical intervention. Based on where she started, from the right ventricle being categorized as moderate to severe with a low Z score of -4 and a prognosis of a possible Fontan surgery, and now looking at her present plan of no surgery at all, well, this is all pretty amazing. I will have to check my stats, but I feel like the probablility of this recovery path was under 10 per cent. Of course it is all guess work. So we will see what comes next, but today sure seems like a good day.
