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Treatment Options

This list illustrates current treatment options for PAH, and one medical treatment approved for CTEPH. The ideal treatment pathway for an individual patient should be decided together with a PH-treating specialist. 

Please see your specialist to discuss the ideal treatment pathway for you.

For more information about the surgical treatment of CTEPH, click here

  • Endothelin Receptor Antagonists (ERAs)

    Endothelin Receptor Antagonists (ERAs) help prevent blood vessels from narrowing.

  • Phosphodiesterase inhibitors (PDE-5 inhibitors)
    Phosphodiesterase 5 inhibitors (PDE-5 inhibitors) allow the lungs to produce more of their own natural vasodilators.
  • Soluble guanylate cyclase stimulators (sGC stimulators)
    Soluble guanylate cyclase stimulators (sGC stimulators) increase the sensitivity of sGC to endogenous nitric oxide (NO). They also directly stimulate the receptor to mimic the action of NO.
  • IP Receptor Agonists
    IP receptor agonists stimulate the IP prostacyclin receptor to cause vasodilation.
     
    IP receptor agonists are vasodilators that also have antiproliferative and antiplatelet properties. Prostacyclin is under-produced in patients with pulmonary hypertension.
  • Prostacyclin Analogue
    Prostacyclin analogue is a vasodilators that also has antiproliferative and antiplatelet properties. Prostacyclin is under-produced in patients with pulmonary hypertension.
    • Oral treprostinil (Orenitram®) – not approved in Canada (approved in USA in 2013)
  • Inhaled Prostacyclins
    Inhaled treatment options, such as prostacyclins, relieve shortness of breath.
    • Iloprost (Ventavis®) – Not approved in Canada but available in the US and Europe
    • Inhaled treprostinil (Tyvaso®) – Not approved in Canada but available in the US
  • Intravenous Prostacyclins
    Intravenous treatment options open up the blood vessels and help ease symptoms of PH, including chest pain and shortness of breath
    • Epoprostenol (Flolan®) – approved in Canada in 1997
    • Intravenous treprostinil (Remodulin®) – approved in Canada in 2005
    • Room temperature stable epoprostenol (Caripul®, Veletri® - in USA and Europe) – approved in Canada in 2013
  • Subcutaneous Prostacyclins
    Subcutaneous treatment options are delivered through a portable infusion pump to open up the blood vessels and ease the symptoms of PH:
    • Subcutaneous treprostinil (Remodulin®) – approved in Canada in 2002
    Although all of the above-mentioned medications help to alleviate the symptoms of PH and slow its progression, they do not provide a cure. As the disease progresses, treating physicians prescribe the available treatments alone or in combination to help alleviate the symptoms and slow progression. Once a combination of treatments is no longer effective, lung transplantation may be considered.

 

What role can patients play in PH research? 

New PH research advances are continuously paving the way towards better, more personalized treatment options. Click here to find out more  about ongoing clinical trials in Canada and how you could help advance the future of PH medicine.

Thanks to advancements in research, many PH treatments are available in Canada. However, they aren’t always available through public funding in every province and territory. This means people in some parts of the country don’t have equal access to the vital treatments they need. Visit our advocacy section to learn more and join us in representing a united PH community.