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Treatment Options

Many medications can treat PH, helping to alleviate the symptoms of PH, improving patients' wellbeing, activity, and quality of life. Medical treatment can also slow the progression of PH and improve RV failure, and often improve survival. However, medical treatments do not provide a cure.

The list below describes current treatment options for PAH, and one medical treatment approved for CTEPH. 

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

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

  • Endothelin Receptor Antagonists (ERAs)

    Patients with PH make excess endothelin, one of the most powerful chemicals which constricts blood vessels, and causes cells to multiply, narrowing pulmonary arteries and increasing PAP. Endothelin Receptor Antagonists (ERAs) block the action of this excess endothelin, and can open up pulmonary arteries and reduce PAP, as well as improve RV function. ERAs also have anti-proliferative (make cells stop multiplying) and anti-fibrotic (decrease scar tissue formation) properties.

    Endothelin Receptor Antagonists (ERAs) include:

  • Phosphodiesterase inhibitors (PDE-5 inhibitors)
    Patients with PH make less nitric oxide (NO), which is a normal pulmonary vasodilator. Phosphodiesterase inhibitors (PDE-5 inhibitors) are medications which improve the action of patients' own NO. PDE-5 inhibitors are pulmonary vasodilators that can open up pulmonary arteries and reduce PAP, as well as improve RV function.

    PDE-5 inhibitors include:
  • Soluble guanylate cyclase stimulators (sGC stimulators)
    Patients with PH make less nitric oxide (NO), which is a normal pulmonary vasodilator. Soluble guanylate cyclase stimulators (sGC stimulators) are medications which improve the action of patients' own NO. sGC stimulators are pulmonary vasodilators that can open up pulmonary arteries and reduce PAP, as well as improve RV function.

    sGC stimulators include:
  • IP Receptor Agonists
    Patients with PH make less prostacyclin, which is a normal pulmonary vasodilator. Chemicals like prostacyclin (IP Receptor Agonists) are also pulmonary vasodilators that can open up pulmonary arteries and reduce PAP, as well as improve RV function. IP Receptor Agonists also have anti-proliferative (make cells stop multiplying) and anti-platelet (decrease blood clot formation) properties.
     
    IP receptor agonists include:
  • Prostacyclin Analogues
    Patients with PH make less prostacyclin, which is a normal pulmonary vasodilator. Chemicals like prostacyclin (analogues) are also pulmonary vasodilators that can open up pulmonary arteries and reduce PAP, as well as improve RV function. Prostacyclin analogues also have anti-proliferative (make cells stop multiplying) and anti-platelet (decrease blood clot formation) properties.

    Intravenous Prostacyclin Analogues are delivered through a catheter (tubing) inserted into a vein in the neck, by a portable infusion pump. Intravenous treatment options include:
    • Epoprostenol (Flolan®) – approved in Canada in 1997
    • Treprostinil (Remodulin®) – approved in Canada in 2005
    • Room temperature stable epoprostenol (Caripul®, Veletri® - in USA and Europe) – approved in Canada in 2013

    Subcutaneous Prostacyclin Analogues are delivered through a nedle inserted most often under the skin of the abdomen (belly) by a portable infusion pump. Subcutaneous treatment options include:
    • Treprostinil (Remodulin®) – approved in Canada in 2002

    Inhaled Prostacyclin Analogues are delivered by a device (nebulizer or inhaler) which allows a patient to breathe in a medication. Inhaled treatment options include: 
    • Iloprost – Not officially approved in Canada for inhalation, but available in the US and Europe (Ventavis®)
    • Epoprostenol (Flolan®, Caripul®) – Not officially approved in Canada for inhalation
    • Treprostinil (Tyvaso®) – Not officially approved in Canada for inhalation, but available in the US
     
    Oral Prostacyclin Analogues treatment options include: 
    • Treprostinil (Orenitram®) – not approved in Canada (approved in USA in 2013)

 

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.