19 months old child with muscular VSD 5mm

[crosalind]

My son is 19 months and has been diagnosed with a ASD and VSD since birth. The ASD was diagnosed at 2mm and VSD at 12.7mm at birth. The ASD has since closed naturally but VSD is currently at 5mm.

His paed cardiologist at his last check up on 6 April is of the opinion that the VSD will not close on its own and have suggested elective open heart surgery.

This was his medical report on 6 April:

Echo
Balanced ventricles with good function, LVEDd 3.55cm, LVEDs 2.02cm, EF 75.1%
No ASD
No MR, Mild TR
Small high muscular VSD measured 5mm with left to right shunt, PG 75mmHg
Very mild deformed RCC but no significnat prolapse or AR
No PS or Trivial PR
No CoA or PDA

Conclusion
Small high muscular VSD with mild eformed RCC

Plan
See 3 months
SBE prophylaxis

- Does a surgery needs to be done now or can he still wait?
- Can you advice whether theMy son is 19 months and has been diagnosed with a ASD and VSD since birth. The ASD was diagnosed at 2mm and VSD at 12.7mm at birth.
The ASD has since closed naturally but VSD is currently at 5mm.

His paed cardiologist at his last check up on 6 April is of the opinion that the VSD will not close on its own and have suggested elective open heart surgery.

This was his medical report on 6 April:

Echo
Balanced ventricles with good function, LVEDd 3.55cm, LVEDs 2.02cm, EF 75.1%
No ASD
No MR, Mild TR
Small high muscular VSD measured 5mm with left to right shunt, PG 75mmHg
Very mild deformed RCC but no significnat prolapse or AR
No PS or Trivial PR
No CoA or PDA
Conclusion
Small high muscular VSD with mild eformed RCC

Plan
See 3 months
SBE prophylaxis

- Does he have to do the surgery now or can he still afford to wait till he's older?
- Can you advice whether cardiac catheterizationcan be performed to close the VSD in this case for a young child?
- Are there any other methods of closure besides an open heart surgery?

Thank you very much.

[Marie M Gleason, M.D.]

If what you are saying is correct, then it sounds like your child was born with a large ventricular septal defect (12 mm); so the fact that it has gotten smaller is quite fortunate.  Also quite fortunate is the fact that the current VSD is restrictive (despite being moderate sized at 5 mm) with a 75 mmHg gradient between the 2 ventricles.  This would suggest that the lung artery pressure is not significantly elevated.  You have not stated whether your child is taking any medications or whether their growth has been affected by this VSD.  The decision to close a ventricular septal defect depends upon the child's clinical status, the concern about high pressure in the lungs and how much heart enlargement there is.  Also if there is outflow tract narrowing or deformity of the aortic valve with valve leakage (your child has the latter but no current valve leakage).  Without knowing your child's height and weight, I cannot say if a 3.55 cm left ventricle dimension is dilated, but it may be large for the body size.  If the heart chamber is enlarged, at some point a cardiac catheterization might be indicated to assess the lung artery pressure and resistance, and to quantify the shunt (how much blood flows across the VSD into the lungs).  That can help determine if surgery is indicated.  If it is truly a muscular defect, then there is still the possibility that it could further decrease in size.   Additionally, depending upon location, some muscular VSDs can be closed with a device in the cath lab, instead of surgically, but the criteria for doing so are individualized for each patient. I would suggest that you discuss these particular issues with your cardiologist and determine why they are recommending surgery NOW versus any other time in the past?  Also, before moving forward with an intervention, you could consider getting a second opinion elsewhere.

[Marie M Gleason, M.D.]

A VSD device has to be able to anchor to heart muscle on all sides, so if the VSD is close to the aortic valve, the device will interfere with the function of that valve.

In our institution, we also do not recommend a surgical intervention for a VSD with aortic cusp prolapse until we start to see a valve leak. This reuqires frequent serial follow up.  We do not wait for the leak to be severe, we just need to see that the VSD is causing enough valve deformity to warrant a surgical intervention.  

Although open heart surgery for congenital heart disease is frequently done and quite successful in the majority of cases, there is no doubt that it is a serious procedure, and even in the best of hands, adverse outcomes can occur.  As such, the indication to perform an open heart surgery must be CLEAR and there should be evidence that if things were left untreated that it would be bad for the child.  The benefit or surgery must outweigh the risk.

so yes, I would say a third opinion is in order here.

As far as open heart surgery cost in the USA, that varies from institution to institution.

[crosalind]

Hi Dr Gleason,

Again thank you for your reply. He was on furosemide oral solution 10 mg/ml and potassium chloride - for the first 6 months of life. Thereafter, the doc said we could take him off the medication which we did.

We have sought for a 2nd opinion and the other doc is not keen on surgery as there is no indication that the valve is leaking. Hence, he is taking a conservative - the wait and see approach.

But his current doc opines that we should not wait till there is a valve leakage and should proceed with elective surgery to close the hole. He states that once the valve leaks, it will bring on more complication, i.e. valve replacement.

Hence, we have two different opinions and don't know what is best? Should we seek for a third?

Can I pls ask you another question - what is the reason that the VSD cannot be closed via device if it is near the aortic valve? Risk of damanging the valve?

How much does it cost in the US to perform a open heart surgery to close the hole?

Thank you very much for your time, you have been much help.

[Marie M Gleason, M.D.]

If the VSD is close to the aortic valve, it cannot be closed with a device.  If a VSD is muscular, it is surrounded on all sides by muscle, and with the growth of the child, the VSD can sometimes get smaller (but some are stubborn and do not do so).  The naming of the position of a VSD is based on the interpretation of the echocardiographer.  It is surprising that your child is not on medication if the VSD really started out as 12 mm.   Clearly, if it is 5 mm in size now it HAS gotten smaller. Your child is 19 months old and has lots of grwoing to do.  But if the growth curve shows that the child is decreasing in growth velocity, then that would be an indication to close the hole.  It sounds like you may need another cardiologist to see this data and your child and render a complete opinion.

[crosalind]

Hi Dr Gleason,

Thank you so much for your comment. To answer some of your questions:

1) He is NOT on any medication at the moment.
2) His weight = 10.4kg
3) His doctor said that his weight is at the 10th percentile - slightly below the average for a child of this age.
4) His VSD is located near the aortic valve
5) His doctor is recommending surgery as he says the area around the VSD is very clear and there are no new tissue growing. Hence, the likelyhood of the VSD closing naturally is very slim.

Could you help to clarify when you said that if this is truly a muscular defect, there is a possibility that it will reduce in size?

Also is a closure via catheterization possible for a VSD located near the aortic valve?

Thank you so much.