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Lv enlargement in 2 y.o. with multiple CHD
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Lv enlargement in 2 y.o. with multiple CHD

History: daughter age 2 years 11 months. Born with an small ASD, moderate-to-large VSD, and Wolff-Parkinson-White Syndrome. ASD closed on its own. VSD consistently appeared to be growing smaller on echocardiograms from birth through 2 years of age but some aortic regurgitation was showing, classified as mild. Since the AR appeared stable and the VSD seemed to be actively closing, we all agreed to hold of on surgery in hopes it would close on its own. She had a radiofrequency ablation at 2 years 3 months for the WPW due to frequent fainting episodes. During follow up for her WPW ablation, her AR was still showing as a stable/mild on echocardiography, but her cardiologist felt it was getting worse by physical exam (stethoscope) and recommended closure of her VSD.

During her surgery, it was discovered that her VSD had never decreased in size--- rather one of the leaflets of her aortic valve was being pulled into the VSD and blocking blood flow across the VSD making it appear smaller by 2-D echo. In the coming months post-surgery, her aortic root has continued to dilate and her aortic regurgitation has been reclassified as moderate and last week we were also told that her LV is now 37 mm and therefore "slightly enlarged". She has been prescribed enalapril 2.5 mg twice a day since her surgery due to persistent high blood pressure post surgery (130s/40s) and to help her heart function more efficiently (? as explained to me).

I am looking for more understanding of LV "enlargement". What are the normal ranges for this age? What are elevated ranges? How frequently does aortic valve damage and regurgitation heal itself when secondary to a VSD and the VSD is closed? Is there any way to determine the most likely scenarios for moderate AR with a slight LV enlargement over 5 or 10 years?

I'm looking for a general idea of what we can expect/ the possibilities with AR and LV enlargement.
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773637_tn?1327450515
Dear Rjthkids,

Unfortunately, this is a scenario that we sometimes see.  I don’t have all the information about your daughter that I would need to tell you everything you want to know, but I can help a bit.  Most likely, your daughter’s ventricular septal defect (VSD) was a perimembranous, or conoventricular, defect, which means that it was up under the aortic valve.  What we can see with these defects is that the jet of blood going from left to right across the hole also created some suction that pulled down the aortic valve leaflet, damaging the valve and causing the aortic valve regurgitation.  Despite closing the defect, it sounds like the valve leaflet was deformed, again something that we do see.  I would hope that the surgery, besides closing the VSD, addressed the aortic valve to try to repair it.  With the increased aortic root dilation, however, any repair may have been moot, as the valve leaflets are not able to close adequately.  You don’t mention whether the aortic valve is bicuspid, which is a condition that frequently contributes to aortic root dilation as well as aortic valve stenosis, regurgitation, and prolapse.

At this point, in some ways, your daughter has a totally different disease process going on.  The VSD is closed, but the aortic valve is now no longer normal.  The aortic valve does not do a good job of healing itself with the amount of damage that has occurred.  I can’t tell you the exact range of normal left ventricular dimensions for your daughter, because we actually define the ranges based on body surface area.  Based on her age and assuming that she is of normal size, I would estimate that her body surface area would be about 0.6 square meters, which would give an approximate normal left ventricular end diastolic dimension range of about 18 to 34 mm.

However, any left ventricular enlargement in the face of moderate aortic valve insufficiency suggests that there is already a significant volume load on the left ventricle.  The enalapril that was prescribed may help in two ways.  One, it may be able to decrease downstream resistance to blood flow and somewhat reduce the amount of aortic insufficiency, which decreases the left ventricular volume and its work.  Two, it may potentially help the proteins that make up the connective tissue in the aorta itself to stop dilating, although this has not been definitively proven.  Since I don’t know your daughter’s weight, I don’t know if the dose that she is on will be adequate to do this.  That said, this is only going to end up being a temporary measure, as I believe it is likely that at some time in the future, your daughter will likely need to undergo surgery to repair or to replace her valve.  Without seeing all the data, I can’t tell you how long that will be.  Obviously, the goal is to keep a surgeon out of her chest for as long as possible.  But, a chronic significant volume load can damage the heart over the long run, so timing her next intervention prior to that point of irreparable damage will be important.
4 Comments
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Avatar_m_tn
Dr. Boris,

Thank you for your answer. That was indeed very helpful. My daughter just reaches 35 inches tall and is 12.8 kilograms (per last office visit). Her cardiologist did mention that the upper limit of her LV range should be 31 mm. Not having any comparison or knowledge of LV size makes it hard to know just how significant a few mm larger actually is.

So, what I am to understand at this point:
1.) Given the level of aortic valve damage (she does have a tricuspid valve), it is not realistic to expect that it will spontaneously heal itself given time.

2.) Given the current level of regurgitation and LV enlargement and the fact that she is only two, it is realistic to expect that a surgery to repair and/or replace her valve would happen at some point in her childhood.

3.) She now has a distinct, separate "acquired" heart defect instead of a congenital one. Is it safe to presume that unlike a VSD surgery that largely eliminates the problem, management of her aortic valve will require some sort of life long cardiology follow and care, even/when it is repaired or replaced? Is there any good website or informational material that you would recommend parents read to better understand aortic regurgitation and its management?

They did not touch her valve during her VSD closure because they feared doing more damage. I honestly believe both the surgeon and her cardiologist were feeling confident before her surgery that this VSD closure would fix the problem. We were told repeatedly that while she would be at higher risk of needing a valve repair/replacement at some point; it would be much, much later in adulthood if at all. Since her surgery, all the surgeon would say was "There is a good chance she will back here at some point" and all her cardiologist will say is "let's see what happens over the next year." I think they are hesitant to make any predictions or overviews given that this level of damage was an unexpected surprise to all involved. I appreciate the information you have shared, and feel it helps us as her parents to have a better idea of what she is facing.

Thank you for your time,
Rene
P.S. one final question. We do live at moderately high altitude (6800 feet) and regularly are up to 7400 and 7500 feet. Is there any evidence that if we lived at a lower altitude she would/could have a better outcome with managing her heart issues?
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773637_tn?1327450515
Dear Rene,

1.  That is correct

2.  Given the degree of her aortic regurgitation as moderate, I would say that a surgery will need to be done at some point.  I don't have enough information to be able to tell you when, any more than your surgeon or cardiologist do, actually.  Your daughter's heart, with medical assistance, may be able to stay like this for some time.  However, every child is different and we just don't have enough information to say how long this will take for sure.

3.  This is a distinct, acquired process, yes.  In reality, no matter whether this event occurred with her valve, or not, she would have needed some sort of lifelong follow-up.  Contrary to popular belief, surgery for congenital cardiac disease significantly improves the hemodynamics BUT can create the potential for other problems.  These problems can include damage to the sinus node (the natural pacemaker of the heart), risk for arrhythmias, damage to valves during the surgery, and some others.  Overall, we recommend that patients who have just about any kind of surgical intervention for the heart have lifelong follow-up to ensure that they are not having these complications.  It isn't usually close follow-up--if she hadn't had her aortic valve complication, it would have eventually been every 2-5 years, or so, probably.  In fact, we hesitate to use the word, "repair", anymore, only because it gives the wrong impression to both parents and primary care docs.

In regards to reading material for AR and its risks, complications, and management, I am not aware of any non-medical sites that goes into a significant amount of depth without getting too technical.  You may try the American Heart Association's site, though.

Finally, I do not believe that a change in altitude should make a significant difference for this cardiac lesion.  There are certain, complex cardiac defects that do benefit from descending to a lower altitude, but this isn't one of them.  This will likely take a combination of medications and, eventually, surgical intervention.
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Avatar_m_tn
Dr. Boris,
I believe I have a better understanding now. We will simply need to continue her follow-up care, continue medications as recommended by her cardiologist, and wait and see what unfolds in the future regarding her valve. I'm glad I now have a better sense of what the possibilities might be at some point down the road and a better understanding of how her valve issues evolved.

Thank you for your time and expertise.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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