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BAV, AS, AI, aortic dialation and LV enlargement
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BAV, AS, AI, aortic dialation and LV enlargement

Ok- Here is the rundown. My son is eight years old. He has a Bicuspid Aortic Valve, Moderate Vavular Aortic Stenosis, Severe Aortic Insufficiency, Moderate Aortic Dialation and Mild Left Ventricle Enlargement. He has been symptomatic for a couple of years controlled with 2.0 digoxin twice daily and 2.5 lisinopril twice daily. His energy level  took a market decline in August. His peak velocity was measured in February as 3.7 and when we measured again in September (after the decline) it was 4.4.
I am trying to gauge when we should do the valve replacement. His cardiologist originally said it would be within a few  months (back in September) and I haven't heard anything since. He is scheduled for a stress test in January- was originally scheduled in October but declined to participate. I am unsure what to think since January would be past the "few months" when he should have surgery.
I also have a tendency to like the Ross procedure, but the surgeon at my insurance approved hospital doesn't perform it. He recommends a mechanical valve stating that the surgery with a mechanical valve is easier. I'm not sure I agree with having a mechanical valve when coumadin/warfarin therapy can cause bone density issues in pediatrics. What are your thoughts on the different procedures for a child of eight?
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Dear delvalle6: if your son has a bicuspid aortic valve with severe aortic regurgitation and left ventricular dilation, either with or without aortic stenosis, then surgery will likely be necessary and a valve replacement will be likely, as your cardiologist has stated.  Although the Ross procedure has the advantage of the patient not needing anti-coagulation, there are some long term issues such as: 1) potential early “failure” of the transplanted neo-aortic valve (old pulmonary valve) with significant leakage, 2) development of narrowing of the homograft placed between the right ventricle and the pulmonary artery and 3) back flow through the right ventricular to pulmonary homograft that can cause enlargement of the right sided heart structures.  Even under the best of circumstances, a Ross will likely “last” only about 15 years or so and the aortic valve will need replacing again in his lifetime.  However, a mechanical valve in an 8 year old may be small and require replacement later to an adult sized valve.  Some surgeons actually prefer using a homograft valve in the aortic position, knowing that it will need to be replaced in 10-15 years, but that way, you avoid anti-coagulation in a young child and do not mess with the pulmonary valve. The exercise stress test is a way of tracking subtle effects of valve leakage and blockage on your son’s heart, and if abnormal, will factor into the timing of the valve surgery.  However, the severity of the abnormal echocardiographic dimensions will likely be the final determinant in the timing of the surgical intervention.
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Dr. Gleason,
Thank you for your response. I had considered a homograft valve. Because of my son's age, weight, and height in conjunction with the size of the aortic annulus there is no question that he will need a resurgery in his teens should we go with a homograft or mechanical valve. I was lead to believe that a homograft in a child of his age may only last a few years- that we would, in fact, be lucky if the homograft valve made it four years. Wiould a homograft last him to his teens? I realize that even after a homograft or mechanical valve that the Ross could still potentially be performed- but would it be beneficial at that time? I can tell that the Ross has fallen out of favor for many institutions, but is it worth a shot if the Ross is the only procedure that would offer a child of my son's age any lasting results? I realize that with any procedure, there are risks- and that with any procedure there are "optimal" outcomes and then there are less favorable outcomes. I also understand that any surgery that my son has will only be palliative.

Have you heard of the double Ross? A procedure where the pulmonary valve is moved to the aortic position and the aortic valve is "repaired" and placed in the pulmonary position? A few years back a surgeon recommended that I consider this procedure- but I have been unable to find any statistics regarding its success- or resurgery.

It is hard for me to convey the results of this ekg and echo exams in a forum so I realize that the information is very limited. The measurement that is spoken of the most is how symptomatic he has become. The biggest question that seems to be asked is how he does with excercise. Since he rarely excercises anymore (since summer) that is a difficult question to answer. In an attempt to increase my son's activity level I bought a Wii sports just last week. He was having a great time- until his chest began to hurt. He tried to play through it but I told him he should take a moment to rest. He was fine after that until he played again.
Thank you again for discussing this with me.
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773655_tn?1340656399
Dear delvalle6:  the "life expectancy" between a Ross and a homograft valve in a growing child is likely much the same: anticipated 10-15 years, but could be much shorter, depending upon the child and rate of calcification that occurs.  I do not have any information for you re: a "double Ross".  However, not needing anti-coagulation, with either of these surgeries, from a life style perspective, is very desirable in this age group.  As you say, the doctors will be very concerned if there is a change in exercise performance or the development of chest discomfort (and both have occurred in your child), as this influences the timing of surgery. It sounds like you need to reconvene with your cardiologist very soon to address this important issue, as an aortic valve replacement sounds like it will be needed in the near future.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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Marie M Gleason, M.D.Blank
The Children’s Hospital of Philadelphia
Philadelphia, PA
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