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VSD with RCC prolapse for 3 year old - surgery?
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VSD with RCC prolapse for 3 year old - surgery?

My son was born prematurely (31 weeks) and is now 2Y11M. He has had a VSD since birth, with approximate size 4-5mm. Regular echocardiography was done every six months. He was on diuretics for the first 15 months, and then was discontinued. He has been completely asymptomatic apart from being underweight (current weight 24lbs).

At age 10m, we were told there was mild RCC prolapse of the aortic valve. Now (at age 2Y11M), we were told the RCC is deformed and enlarged, and prolapsed into the VSD - with mild to moderate prolapse. Initially it was thought the VSD was perimembranous, but now they think it may be outlet muscular type VSD. No aortic regurgitation has been detected. There has been differing opinions in how to proceed - one view is to wait and watch as there is no AR, the other is to have surgery soon.

My questions are as follows:

1. On what basis do we decide if surgery is needed or not? What are the pros and cons of waiting?
2. Is surgery an eventuality, or is there possibility that he won't need surgery at all?
3. Will surgery involve only patchup of VSD, or valve repair as well?
4. As the RCC is deformed and enlarged, is this permanant damage which will have later repurcussions in life?
5. Is this a one off surgery which will fix things permanatly or will there be interventions required later on in life as well?
6. Does his poor weight gain have anything to do with his condition?

While we do have good healthcare where we live in Hong Kong, we do not have a dedicated children's heart hospital. I would like to have my son's case evaluated at a place which has the best expertise in this area, travelling to the US is not a problem.

I have copies of all his echocardiogram CDs and reports, and I would really appreciate if you could review them and I could have your expert advice on his condition and how I should proceed.

Thank you.
773655_tn?1340656399
Thank you for your posting. When the right coronary cusp of the aortic valve prolapses into a VSD (it can either be a perimembranous or outlet type for this to happen), it is a concern, because the distortion of the aortic valve can lead to the development of valve leakage (because the leaflets do not close properly).  In general, if this is being watched closely, as you are doing every 6 months, it is highly unlikely that severe aortic valve leakage would develop quickly.  In fact, at our institution, we generally wait until we start to see some trivial to mild aortic valve leakage on color doppler by echo before we would refer for surgical intervention, unless the size of the VSD alone warrants surgical intervention.  I cannot say if your child's current weight is related to the VSD or not, because I don't have any details about whether the heart chambers are enlarged, and I assume that there is no elevation of lung artery pressure, or he would have been referred for surgery already. In general, this operation would be the same as a routine VSD closure, and if the valve is not leaking alot they essentially push the right coronary cusp back into position without the need for valve surgery.  Obviously more advanced cases of valve leakage might need a valve procedure as well.  We generally follow these patients long term to see if the valve changes over time, either with or without surgery.  Although the conventional wisdom is that most patients will need a surgery, that is not always the case; I can think of 2 patients of mine who have cusp prolapse, a small VSD and no aortic valve leakage, so surgery has not been recommended for them.   If you would like us to review your child's records, you can contact our CHOP Cardiology Nurse navigators at either ***@**** or via phone 267-426-9600.  Thank you.
5 Comments
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Avatar_f_tn
Thank you for your posting, I appreciate the help and insight you have provided.

During our last appointment, the pediatric cardiologist said his reason for recommending surgery (if not now, then in a few months) is the dilation of the aorta. He measured the Ao at sinuses to be 2.05cm, which he said had a Z-score of +3.07, and anything over 2 standard deviations is already an indication for surgery. These are the measurements he found:

RVIDd = 0.54cm
LVIDd = 3.37cm (+1.5)
LVIDs = 2.2cm
LVPWd = 0.4cm
Ao at sinuses = 2.05cm (Z +3.07)
La/Ao = 0.95
FS = 34.7%
VSD max PG = 65.0 mmHg
No AR

For your reference he is 10.8kg, height = 90cm and age is 2Y10M

My question is this an indication for surgery? the ped cardio has said the prolapse is significant, with the RCC deformed and enlarged significantly, whereas a month before another cardiologist said there was only a slight protrusion of the RCC and mild prolapse not needing any surgery and just regular observation. Why the discrepancy?

Also is the deformity something that surgery can fix, i.e. as you mentioned just to push the RCC back in place?

Are we doing any harm by waiting and just having careful observation every 6 months, rather than opting for surgery?

How and when do we decide if we need surgery, as there are different opinions?

I really do appreciate the help, I hope you can help me answer my concerns.

Thank you, I will contact the nurse navigators and have sent the echo CD + reports sent to your hospital for your kind review.
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773655_tn?1340656399
The dilation of the aorta is not enough of an indication to proceed with surgery if there is no aortic valve leakage.  Careful follow up should allow you to pick up even minor changes in aortic valve function.  Unfortunately it sounds like you need the tie-breaker 3rd opinion.
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Avatar_f_tn
Thank you for your response. I've contacted the nurse navigator and in process of submitting forms and reports to CHOP.

One question is eating away at me though. By not performing surgery now are we not allowing for further damage to the aortic valve? Wouldn't it be better to have surgery done so that further damage does not happen?
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773655_tn?1340656399
Usually the progression to aortic valve leakage is very slow in this situation.  Once a trivial amount of valve leakage develops then surgery could be undertaken.  Aortic valve leakage of a trivial degree usually does not progress.  However, if you feel very uncomfortable about this aspect of the condition, then it might push you to agree to surgical repair at this point in time.
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