Newbie - Looking to understand son's condition!

[MistieC]

Hi! My son is 2 months away from turning 14 yrs old. Last summer (2011) we took him to the dr because he fainted and a couple of times he was starting to get dizzy. He is a very health kid. He is hardly ever sick even if there is an illness going around school he very rarely gets it. The cardiologist ran a bunch of test and found that he has low blood pressure and is on medication now. However because of the echo that was done he found that he has BAV and a dilated aortic root. I've tried researching this on the web but most of what I found has to do with Marfan Syndrom. He does not have this.

My questions are... How large is a moderately dilated aortic root? I'd like to know if there is a range I can gage the sizes. Meaning how big is mild, moderate, and large enough to have surgery?... Hunter is also on a low blood pressure medication and the dr is talking about putting him on a beta blocker at his next appointment in Nov. Is this mediction going to interfer with his low blood pressure meds because beta blockers a supposed to lower blood pressure?... And, what is an average age for children to have to have their valve replaced?

My son's dr is leaving Texas Childrens and we have been given a new dr so as of right now I don't have anyone to ask these questions and I know the dr's are very busy anyways... I just worry about him and I'm glad I came across this website. Thanks, Mistie

 

[ElectLive]

Hello Mistie and welcome. Aortic root size, and what is classified as moderately dilated, depends on many factors (such as age, sex, and body size) including your son's own individual "normal" , so it is hard to provide a definitive answer to your question. However, for general reading about normal aortic root size ranges, please see this post (#22) and thread: http://www.valvereplacement.org/forums/showthread.php?40337-aortic-root-4-2-(no-BAV)-growth-rate&p=523778&highlight=#post523778. Sorry, but you will have to do a little math, though!

Keep in mind that most of what you read here as far as aortic root size will be relative to adults, although that post I linked does at least include a chart in a broad children and adolescent (under 20) age group. Aside from size, other factors enter into the surgical evaluation process, such as growth rate, location of dilation, symptoms, other conditions, and/or valve related issues. All that being said, I'm actually going to give you a number (but I've done my best to convince you that you should ignore it!): a very common surgical threshold for aortic repair in BAV patients is now 5.0 cm. But again, it's not nearly that simple, and very often another size may be used. Finally, one other thing to mention relative to size...echo measurements aren't necessarily definitive either, so repeated measurement over time could provide a better gauge for the severity of the dilation than a single echo.

Beta blockers are very commonly prescribed in those with dilated aortas as a means to impede dilation. Obviously, with your son's already low blood pressure, it's an important conversation to have in more depth with his doctor. The goal of the beta blocker approach is lower than normal blood pressure and pulse but generally only to the extent that the patient is able to tolerate...ie not have other problems like excessive dizziness.

There really is no average age for BAV replacement, it can happen at any point in time depending on the individual patients. There are broad patterns, though, and those who develop aortic aneurysms seem to more often fall into one of the earlier age groups (20s and 30s). But again, no way to know for sure, so it's just important to monitor over time. Some with BAV never need surgery, many not until much much later in life.

Best wishes to you and your son and be assured that whatever happens moving forward, aorta repair and valve replacement are extremely safe at whatever age they occur, should that in fact be necessary one day. This community is full of success stories of amazing recovery and return to completely normal (athletes included) lifestyles. There are also some great parents on here who will be able to more directly relate to your experience.

 

[MistieC]

ElectLive, Thank you for the information. In the first 6 months after he was diagnosed we had an echo ever 2 months and a MRI at the 6th month and another echo at a year to give use a base line to work with to see how fast it is growing bigger. He has to have an echo every year as well as a MRI every other year. I trust the dr but when I ask for the size it measures I've only been told that it is moderately dilated. I know he is probably trying now to scare Hunter but I feel I need to know. At the next visit I am going to talk about the beta blockers and the size. Again thank you for the information... MistieC

 

[kfay]

Mistie, Some of us have found it helpful to have copies of every test that is done so that we have the "numbers" in front of us and it is much easier to compare yourself, visit to visit, how he is doing. You have a right to those records, all you need to do is request them. The echos will be more specific, and although they usually say whether or not it is moderate, severe, etc., there will actually be measurements on there that you can get a sense of the size of things. Good luck and I hope it will be many, many years before your son needs any intervention.

 

[MistieC]

kfay, Thank you... I did not even think about asking for a copy of his records.

 

[ramjet]

MistieC,

It can be very frustrating, having been in your sons position, I was diagnosed almost 30 years ago, and have outlived two cardiologists and one GP, and boy, do you think any two specialists actually agree on anything...no way, and then start talking to surgeons and the opinions continue to vary....let alone what family friends and others who are well meaning have to say....

Its more than just aortic roots size, there are lots of things they consider when determining the best option for treatment and the timimg of treatment...not the least of which are the severity of symtoms and abnormal vital signs. Fortunately I was symptom free (no shortness of breath or chest pain, or lethargy etc) until this year, and my pulse and blood pressure have always been normal thankfully. Once I started to find it hard to exercise, and started to become breathless when exerting myself, they did another cardiac echo, and an angiogram (because I was nearly fifty and they like to know what the hearts blood vessels are like before they put someone in my age group and avbove on heat-lung bypass during surgery), and they looked back over all my previous cardiac echo test results, and they could see that my aortic root had not yet started to dialate, but my LVEDV (left ventricular end diastolic volume)...(basically the amount of blood that was sneaking back into my left ventricle due to the leaky bicuspid aortic valve and making my left ventricle start to stretch) was getting worse, and the hearts ejection fraction was falling (amount of blood pushed out with each contraction)...I didn't have any aortic valve stenosis to speak of and the decision to replace my leaky aortic valve was made before it got any worse...so I lived a normal life with a bicuspid aortic valve for nearly 49years without any real issue, and if not for a routine echo done by a thorough old GP, I would never have known about it.

You son is a little different in that he has become symptomatic already (ie feinting I think you said), and low blood pressure and his heart is starting to dilate. Beta blockers are not likely to be prescribed if his blood pressure is low. If the aortic root dilaes too much more it may mean that the surgery is more extensive (ie aortic valve replacement AND an root replacement), or other symptoms can develop, so you will need to be guided by a cardiac surgeon who is very experienced and specialises in replacing aortic valves in "young adults" .
Based on trends in measurements done by cardiac echo, vital signs, symptoms, LVEDV, aortic roots size, amount of regurgitation (backflow) or stenosis (obstruction to flow), they use dopler measurments to calculate blood velocity, and they measure heart chamber size, and blah blah...everyone is slightly different, and it all depends on each persons individual situation as to how and when they manage the valve....a bicuspid aortic valve is one of the most common genetic heart disorders, and you need to ask questions, keep test results, and take notes, becasue one dctor will say something and another will contradict that opinion sometimes...oh the joy of dealing with doctors who are too smart for their own good sometimes