For our other readers, PDA is patent ductus arteriosus, which is a blood vessel that all babies have in utero which typically closes after birth. “Coarc” is coarctation of the aorta, a narrowing of the main artery that takes blood from the left side of the heart to the body. Coarctation can cause severe obstruction to blood flow. A-V canal, or atrioventricular canal, is a hole in the central portion of the heart. Subaortic stenosis is a fibrous or muscular obstruction of blood flow out from the left ventricle to the aortic valve across the left ventricular outflow tract (LVOT). TEE is short for transesophageal echocardiogram.
There are a lot of confounding problems going on with your son, both with the heart as well as with other non-cardiac systems, so it is very difficult to say what is happening here. As well, I don’t have all of the information that I would want to be able to get a sense of what is going on with your son’s multiple problems. There are numerous possibilities, therefore. One thing that is concerning is that the apnea and hypoxemia during the sleep study may be associated with obstructive sleep apnea, a condition that is often seen in patients with Trisomy-21. If it is prolonged, it can cause pulmonary hypertension, or high blood pressure across the lungs. It can cause some of the concerns that you list. Certainly, neurocardiogenic syncope has been seen in children at this age, although based on what you are saying, this may be less likely. Overall, I agree with your getting a second opinion if you are not getting adequate answers to what is happening with your son’s events, including both pediatric cardiology and neurology.
Sad to say...my son's events have turned into heart failure. LV is stiff, right heart pressure is elevated, gradient is in 60's, aortic valve is tethering, he now has pulmonary hypertension, anemia has developed and we know is sleep apnea is severe. Chest x ray showed signs of heart failure as well as blood work. Moving to a university hospital, Monday, to get workups from specialists he is familiar with as well it is a transplant center just in case. 2 yrs ago, I sent info to CHOPS and they said his next step is 2 prothestic valves since Dr Mee had opened the outflow tract the best that anyone could do already. Is there any new discoveries or any new ways to deal with the muscle structure attached to papillary muscles going into LVOT? What is fastest way to get an opinion from CHOPS? Just in case I do want to go up to a bigger center. He would have to move from the hospital to there. I won't get to speak to the surgeon here for a week.
The better way to get a second opinion after your information has been sent is to be seen for a complete clinical evaluation. I'm not sure that a smarter opinion can be offered any faster without being less complete. There is something to be said for having the patient and family in front of you, being able to ask questions, to perform an examination, and to have the test results that you need to be able to create a complete picture.
It unfortunately is too late for another complete evaluation he will be ready for surgery next week when the surgeon makes it back into town. Looks good outwardly, but more things are skewing out of balance in his systems. Wish he had more time. Thank you so much for your time. If you do get info from our group on Tyler, there is some info given back to you guys 2-3 yrs ago. Thank you
A related discussion, thank you