Repost

[Jas07]

9 year old with history of Familial PAH in one parent has reported mild TR between 30-35 mm Hg and 35-37mm Hg respectively in 2 successive Echos over a period of 6 months.
The pediatric cardiologist wrote it down as "Mild PAH" with no changes in heart structure.

What precautions should one take to initiate lowering of pressures and/or prevent progression to PAH?

Thanks.

[Jonathan D. Rich, MD]

There is nothing "proven" to prevent or clearly slow the progression of PAH.  Having said that, this very may be an example of allowing the cart to get ahead of the horse.  First, you need to see if there is even anything to be concerned about.  If PAH is present, modern day therapies have improved somewhat and quality and quantity of life have likewise improved as a result.

But again, really not clear that there is even anything going on at this stage.  Please feel free to keep me posted if you learn new information.

Best,

Dr. Rich

[Jas07]

I understand that the results at best are only indicative.

However, my question essentially is .... is there anything in medicine or nutrition ..... or any kind of lifestyle known to mankind to prevent progression to PAH in adult life?
(for eg.: Keeping L-citrulline levels high might prevent the disease .... so a research study sought to investigate)

[Jonathan D. Rich, MD]

Hello and welcome to the forum.

My apologies for this delayed response.

First, the PA pressures by the echo are all but meaningless.  I simply can not comment on them since they are frequently wrong and not truly representative of actual PA pressures.  I would strongly consider having your child evaluated by a national expert in pediatric PAH.  There is a very good chance that your child is ok but if you have been told that you have a familial form of PAH, I would certainly not trust anybody but a PH expert.  And, if you tell me that the current doc you are seeing "is an expert" I woudl consider getting a 2nd opinion.

Best,

Dr. Rich