My daughter is 23 months old and was recently dx with Aortic root dilation. She has shones complex and had a repair done at 3 weeks old for a large VSD, 2 ASD's, PDA, and a coarc. She has a BAV and Parachute MV. She did not cooperate well for the ECHO so they are repeating next week, sedated, to get a better look at the valves but they did see the dilated aortic root. The cardio wanted to wait until we see the valves to give me more info but said it is at 3.8 SD. Her BSA, if I calculated correctly, is at .47. She is 84.45 cm and 8.87kg. They are also putting in a gtube next week for FTT as she has no interest in food. Can anyone tell me what we may be looking at as far as intervention? At her last ECHO, a year ago March, there was no dilation and only trivial regurg on the mitral valve. There was no stenosis on either valve. Could the feeding issues be heart related at this point?
Without evaluating your daughter, I cannot say for sure if her feeding issues are related to her heart. I would need more information and would need to see her. However, I can say that aortic root dilation is definitely associated with bicuspid aortic valve (BAV). The intervention that would be done would be surgery to reduce the size of the aortic root. However, the timing of this does not sound like it would be just now. The size of 3.8 standard deviations above the mean (also known as a Z score of +3.8) is certainly enlarged, but is likely not an indication for surgery just yet. In adult-sized patients, we wait for a diameter of approximately 4.5 cm with BAV for intervention. This may be more associated with an absolute dimension and not with the actual Z score, as from a physics standpoint, the likelihood of aortic rupture is greater with that size. It will certainly need to be observed over time to ensure that it isn't getting too big. There is some preliminary research looking at some medications for aortic dilation in Marfan syndrome. It is unclear if thsee would work in BAV patients, although the mechanism of dilation is felt to be similar. You may discuss this with your cardiologist for further details.
Thank you Dr. Boris! Are you referring to the use of losartan? We have not yet discussed any Beta blockers or other meds with cardio. Would you be considering the start of meds at this size? Her blood pressure remains stable at 89/40 something? at her last visit. She is not taking anything as of yet.
The plan was to do the echo when she was sedated for the gtube placement but they have decided to wait and do them separately as the propothol used for GI will not last long enough for the full echo. It has been postponed to the 28th at which time I will be able to discuss everything at length with her own cardio.
I believe her cardio did her residency at CHoP....I feel very confident in her abilities. But it is hard as a parent to wait with not enough information and "Dr. Google" at everyone's fingertips. I found the following article incredibly informative as it pertains to children and adolescents and the normal values of the aorta.
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