TYSON MATTHEW KOTTELENBERG
"The Lord is faithful to all His promises, and loving toward all He has made." Psalm 145: 13b
This blog is about our nine year old son Tyson. Tyson was born with serious complex congenital heart defects, (Hypoplastic Right Ventricle, Tricuspid Atresia, Coarctation of Aorta, Transposed Great Arteries, with VSD and ASD.)
In short, the right side of his heart is completely under-developed (he has half a heart,) his main arteries are mixed up, and his aorta is narrow. He has undergone 3 open-heart surgeries and 5 heart cath procedures to try to 'repair' his heart. Sadly, Tyson has also been diagnosed with pulmonary vein stenosis, a fatal disease in the veins of his left lung. Typically, having the 3 palliative surgeries buys children with single ventricle hearts many years before eventually needing a transplant. But because of the narrow pulmonary veins, this increase in pressure is causing his blood to shunt the opposite way across his Fontan fenestration and surgeons are not able to close the fenestration. As a result, he remains on coumadin and is heading down the transplant road faster than we'd originally anticipated. He's still doing AMAZINGLY well all things considered. We entrust our dear son into the hands of God, knowing that in all things God works for the good of those who love Him!
The MRI will give a good picture of his pulmonary veins and will be able to tell us how well the sutureless repair worked and how it's holding up, or if the stenosis has reoccurred.
The answer as to whether or not this applies to Tyson is very complex. This study assumes that pulmonary vein stenosis is caused by a build-up of cells lining the veins. In some patients, that is true. However, for patients with complicated heart disease, such as Tyson’s, there are other mechanisms which are more likely.
1. The lung arteries are small and underdeveloped, and hence the reduced flow into the lung and out of the lung means that the pulmonary veins are also small and underdeveloped (not likely to be helped by these meds).
2. The pulmonary veins are stretched by other structures or scarred from surgery (again, not likely to be helped by these meds).
I have had patients with the pulmonary vein “disease” which they are interested in, but I think it unlikely that Tyson has this kind of problem and would be unlikely to benefit from these meds (but would have all of the risks). I think we should wait and see what we find on the cath and MRI first, and then decide. I do know these people from Boston.
Dr. Brian McCrindle.
Anyway, Brian and I are both happy to hear that the cause of Tyson's pvs isn't 100% necessarily from an unwanted build-up of cells. We went down to the records department on Wednesday and received copies of ALL Tyson's reports from echos, ecgs, heart catheterizations, MRIs, chest x-rays, etc. I just really want to understand what exactly we're dealing with here. We learned that both his left pulmonary artery and his left pulmonary veins are about 1/3 of the size of his right artery and veins, and he has only 18% blood flow from this left lung. (This was as of his pre-Fontan cath report.) This is substantial. I know that the cardiologist is concerned about this, as he writes in his clinic reports to our family doctor. We also have copies of all those letters too, so we know exactly what we're having to deal with. Now we just have to wait and see what is going to be done about it.
Prayer Request: Please pray that Tyson's pulmonary veins are ok, that the surgery done to repair them was sufficient for him, that the function of his left lung will improve, and that there is no further re-stenosis in the veins. Please also pray for patience for Brian and me as we await his upcoming cath date. Here we go again :(