Looking for Advice - 10 Year Old Son - BAV and Moderately Enlarged Aorta

[hopefulmama]

Hi All,

I am so happy that I have found this forum. My son is 10 and has BAV. The BAV was discovered when he was born due to a heart murmur. The heart murmur was gone not long after birth. So we were lucky to have had that murmur to alert us to bigger issues! In April 2013 his pediatric cardiologist reported that his aorta was mildy dilated and started him on Losartan. At that time his BP was 108/73 and AAO was 2.91cm with a Z-score of 3.43. I took him back November 2013 and here are his measurements: BP 121/77:; annulus 14.7, sinus of valsalva 2.28cm, ST junction - 7.95CM and AAO 2.63. Essentially the Dr. said it was unchanged. I took him in recently, one year later and there has been a "big change." His BP was 121/74, annulus 2.14 cm, sinus 2.64, ST junction is 2.0 cm and AAO is 3.15 with a z score of 4.22. He has restricted him from playing football or wrestling and increased his Losartan dosage. The dr's do not think he has Marfan's - no apparent physical signs. My father dies of a brain aneurysm at 52, his father died unexpectedly at 38 they said from heart attack, but that would have been in 1915. There are lots of cardiac issues on both sides of our family. I am concerned and feel like I need more information. The cardiologist suggested that we wait 6 months and then do another Echo. He also said that he would order a cardiac MRI now if we wanted to. I consulted with his Pediatrician - who is researching to try and find the "mecca" for children's aorta issues. I don't know if I should get the MRI now? Go to Cleveland clinic for another opinion? Texas Childrens, etc? It is hard not to worry and I think it is really tough for my son to process. It is plenty difficult for adults to manage the stress and anxiety related to this type of thing, I can't imagine how it would feel for a 10-year old. I would like to get him into some type of support group for kiddos facing these things - to help deal with the feelings and anxiety , but don't know of any where we are in Oklahoma City. Part of the problem is that they don't really give you any data points to work from - I have no idea what to anticipate or expect in the future. So sorry for this long-winded post....just need some advice - what to do now (MRI, 2nd opinion, etc) and where to get my son the emotional support he needs. Thanks so much!

 

[pellicle]

Hi

welcome to the forum.

I guess I should start my reply to say that I'm responding from the perspective of your son as I was diagnosed early in life too. I think that I said some things which may be of use to you in this thread over here

anyway up front I think that always more information is better to have than less, so yes, if you can afford it get some scans done and get some other opinions. I do however think that an intelligent and experienced mecial practitioner can determine a lot from just listening.

I had my first surgery at about 10 and I was diagnosed with a murmor at about 5.

Now importantly what I think will be the hardest is to not make your kid feel that he's in cotton wool, recognise that he's perhaps going to try things you may not (he's a boy right?) and will push boundaries. I think that its important to not disrupt natural development by making him feel like he's to be kept in a bottle. I know it was something my mum did to an extent, and that I saw oter peoples mum (other kids at the clinics) do to a greater extent.

I was glad that mum let me do the things I did (although don't get the idea that it was without a fight). Your son is another human first and foremost, and your son second. I think its important to instill feelings of self determination in kids and not take over their lives. My views of things over the decades has shown that path to lead to conflict.

I don't feel qualified to answer any of the medical questions as I believe (despite knowing quite enough) that the interpretation of that data is best left to the medical professionals. If your "gut" tells you that their diagnosis is wrong (well, because say he's getting sicker) then do go with your gut to another professional, but talking in terms of enlargement sizes, valve ejection fractions and diameters is strictly stuff for people who work with those numbers all the time IMO.

Aside from that if there is any question you'd like to ask me about my growing up and surgeries (I've had three and I'm 50 now) please do ask.

Best Wishes

 

[Duffey]

Our soon to be six year old grandson is being followed by a pediatric cardiologist due to an enlarged aorta that was diagnosed along with a BAV at birth. Our son has a BAV, my valve was replaced due to a stenotic BAV, and my father died from a BAV, so our family is familiar with this issue. Your son's pediatric cardiologist seems to be recommending a course of action (another echo at 6 months) to determine a rate of growth for his aorta , and I would be reassured by his offer to do the MRI at your request. Have you looked at any of the congenital heart defect internet forums to see what's offered in terms of support? There is an Adult Congenital Heart Association, www.achaheart.org, which offers helps to young adults that have undergone surgery at children or teen-agers as they transition into the next stage of adulthood. I believe you can find a wealth of information there. Best wishes and please keep us informed going forward.

 

[AZ Don]

Hi,
Welcome to the forum. I'm assuming the test results posted above were taken via echocardiogram. It is my understanding that the test is highly dependent on operator expertise and it is not unusual for results to vary from one test to another, even when there is no change. This may be especially true if the test is done in a different facility or by a different technician. For measuring the aorta, a CT scan (not generally recommended for children due to radiation) or an MRI is more accurate. The CT and MRI are more expensive so generally not done unless recommended based on findings from the echo.

Second opinions are generally a good idea though sometimes different perspectives lead to more confusion rather than less. It may give you some peace of mind to consult with an expert. Texas Children's Hospital is actually the 2nd ranked Cardio hospital for children in the country, and ranked well ahead of Cleveland Clinic (for children): http://health.usnews.com/best-hospitals/pediatric-rankings/cardiology-and-heart-surgery. When/if the time comes for surgery it is nice to have a good Dr and a good hospital, but for a second opinion you may only really need a good Dr. While rankings of top Dr's doesn't guarantee a Dr's expertise it is still the best way I know of to find a highly experienced Dr. Castle Connolly ranks Dr's by specialty nationwide, for a small fee: http://www.castleconnolly.com/. Perhaps you can find a top ranked Dr. closer to home.

Unfortunately congenital heart defects are not so uncommon among children. There is a support group for such children and their families called Mended Little Hearts. They have a chapter in Oklahoma City: https://mendedlittlehearts.gnosishosting.net/Groups/Home/LifelineOC. I don't know much about this group but maybe they can be helpful.

Sometimes I think the families of those with such heart issues suffer more than those with the actual heart issue. That is probably especially true when the patient is a child. The important thing to remember is that issues with the aortic valve and the aorta can be fixed. This is not true for many diseases. As someone that has a BAV and has had surgery for an aortic aneurysm, it really wasn't as bad as I would have expected. It was much less painful than my knee surgery (which had some complications).

Good luck

 

[hopefulmama]

Thank you all so much for this information, it is so very helpful! One more question - is the decision to perform surgery based on the AAO measurement and not the Z score? Thank you!

 

[francie12]

I have been away from the forum for a long time, but your post caught my eye because my son was diagnosed at age 11 with BAV, Mod AV Regurgitation and a Mod Dilated Asc AO. We have been on the journey for 11 yrs, and son is 22 now. I decided in the beginning, and I'm really glad I did, that I would not make too much of my son's condition when talking to him. He has gone to his yearly echos, and I answered his questions honestly, with a brief, casual and confident air (he had few), but mostly we just left it alone. The consequence is that my son at 22 has a very practical, unworried and commonsense attitude about it. He says he will do what needs to be done when the time comes, but doesn't need to be concerned about it in advance. Each child is different though, and different ones need different things, of course.

I was very happy when my son had a cardiac MRI to confirm echo results, which they reassuringly did, so you may want to take advantage of that. Second opinions do not hurt, and we actually ended up changing cardios in the early stages to one that was a better fit. I'll never forget how happy I was to find this forum 11 years ago! What a comfort and source of information it was. Best of luck to you and your son.

Wanted to add that we also started to follow up with the Pediatric Genetics and Connective Disease clinic at the local teaching hospital (U of Iowa Hosp and Clinics), and they have helped us to feel that the situation is being well-covered.