The important take away from this is that each WHO Group has its own very specific treatment plan to follow which is why it is essential to have an accurate WHO group classification when being diagnosed.

Last reply from my pulmonologist here was “very mild PH”. Requires no treatment !! Then why do I feel so terrible?? Hope Rochester clinic has some answers. 😢

@A myPHteam Member makes an excellent point. Ask specifically what WHO group you fall under and what the treatment plan is. Something else we all think about is life expectancy. I asked my pulmonologist about this on our first meeting after my left and right heart caths. She replied very optimistically which put me at ease.

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A myPHtea...

Group 1, or PAH, has 4 distinct "pathways" for treatment. Groups 2 and 3 are treated by treating the underlying cause of the PH...specifically the heart disease for Group 2 or the lung disease for Group 3. The medications in the 1st, 2nd and 3rd pathways for treating PAH are very high risk for Group 2 and can cause serious cardiac consequences. I can't speak to Groups 4 and 5 as I don't know enough about them. The 4th pathway is currently in clinical trial for approval for use with Group 2.

When I was misclassified, I was taking medications in the 1st and 2nd pathways (Adempas and Opsumit) and as soon as I was reclassified, I was immediately taken off those meds.

Here's a copy and paste:
Four main pathways are targeted in the treatment of Pulmonary Arterial Hypertension (PAH): nitric oxide, endothelin, prostacyclin, and activin signaling pathways. These pathways are involved in the complex mechanisms that lead to PAH, and medications are designed to modulate these pathways to improve blood flow and reduce the strain on the heart.
Here's a breakdown of each pathway and how they are targeted:

1. Nitric Oxide Pathway:
. Nitric oxide (NO) is a signaling molecule that causes vasodilation (widening of blood vessels) and inhibits smooth muscle cell proliferation. In PAH, the nitric oxide pathway is often impaired. Treatments like phosphodiesterase-5 (PDE5) inhibitors (e.g., sildenafil, tadalafil) and soluble guanylate cyclase (sGC) stimulators (e.g., riociguat) increase the levels of NO or enhance its effects, leading to vasodilation and reduced pulmonary artery pressure, according to the National Institutes of Health (NIH), and the European Respiratory Society (ERS).

2. Endothelin Pathway:
.Endothelin-1 (ET-1) is a potent vasoconstrictor (a substance that narrows blood vessels) and a growth factor. In PAH, ET-1 levels are often elevated, contributing to vasoconstriction and vascular remodeling. Endothelin receptor antagonists (ERAs) (e.g., ambrisentan, bosentan, macitentan) block the action of ET-1, reducing vasoconstriction and promoting vasodilation.

3. Prostacyclin Pathway:
.Prostacyclin is a natural substance that causes vasodilation and inhibits platelet aggregation. In PAH, the production of prostacyclin is often reduced. Prostacyclin analogues (e.g., epoprostenol, treprostinil, iloprost) mimic the effects of prostacyclin, promoting vasodilation and reducing pulmonary artery pressure, according to the PAH Initiative.

4. Activin Signaling Pathway:
.Recently, the activin signaling pathway has been identified as a target for PAH treatment. Activin, a member of the transforming growth factor-beta superfamily, can contribute to vascular remodeling in PAH. Sotatercept, a drug that inhibits activin signaling, was recently approved for PAH treatment, helping to rebalance vascular homeostasis and reduce the progression of the disease

What meds would conflict with group 2 ?? Confused