3 year old pulmonary hypertension

[Damien3981]

My 3 year old son was born with a 10mm VSD that went undiagnosed for his first 8 months of life. We were told it was a pretty straight forward procedure and had the VSD closed immediately. We were sent home from hospital with Lasix and kept up our visits with our cardiologist and everything looked great. Our visits were then stretched out from 4 times a year to twice a year. About 5 months ago we could feel our sons heart really pounding away through his layers of clothes. Parental instincts kicked in and before we knew it our son was diagnosed with idiopathic PH via a cath. He is currently on bosentan.

My question is would it be possible that my son's pulmonary arteries have never had the chance to develop naturally over those first 8 months? Is it also possible that his body may need just a little more time to develop so that his pulmonary arteries function normally and as he grows his lung function will improve? We just don't know if the closure of the VSD has caused these high pressures as his body was functioning so differently for those first few months.

Thank you for your time

[Jeffrey R Boris, M.D.]

Dear Damien,

Because there are multiple potential etiologies of PH with different treatments and prognoses, it is unfortunately difficult to see with accuracy about your son's specific PH prognosis.

[Damien3981]

Thank you for your reply.

I understand it's hard to comment without knowing my sons history or current condition, but generally speaking if the PH is picked up in the early stages, is it possible for the damage to pulmonary arteries due to the high resistance of flow to be "stopped in their tracks" with correct medication or does it continue to get worse over time regardless?

[Jeffrey R Boris, M.D.]

Dear Damien,

There are lots of potential reasons for pulmonary hypertension, unfortunately.  Most children do just fine with a VSD early on, and then go into what is called congestive heart failure, due to too much blood recirculating across the defect into the lungs.  However, it seems that your son's pulmonary arteries had what we call high resistance to flow, and maintained that high resistance, so that the VSD wasn't picked up like it ordinarily would have been.  Thus, it is possible that there was an intrinsic abnormality in the pulmonary arteries to begin with.  Without seeing him, I cannot say exactly what he needs, or what happened.  I would discuss him further with your cardiologist.  However, I recommend that he be cared for at a center that manages many patients with pulmonary hypertension, so that they can take advantage of their experience.