ABOUT PH

Treatment Options

Since 1997, 10 PH-specific medical treatments have been approved in Canada for the treatment of pulmonary arterial hypertension (PAH). One medication is also approved to treat chronic thromboembolic pulmonary hypertension (CTEPH).

Watch and share our video about the pulmonary hypertension treatment options that exist in Canada and spread PH awareness of your life in purple. Watch all videos here >

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.

Treatment Options

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:

Ambrisentan (Volibris®) – approved in Canada in 2008
Download AMBRISENTAN (VOLIBRIS®) Info Sheet
Bosentan (Tracleer®) – approved in Canada in 2001
Download BOSENTAN (TRACLEER®) Info Sheet
Macitentan (Opsumit®) – approved in Canada in 2013
Download MACITENTAN (OPSUMIT®) Info Sheet
Macitentan + Tadalafil (Opsyvni®) - approved in Canada in 2021

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:

Sildenafil (Revatio®) – approved in Canada in 2006
Download SILDENAFIL (REVATIO®) Info Sheet
Tadalafil (AdCirca®) – approved in Canada in 2010
Download TADALAFIL (ADCIRCA®) Info Sheet
Macitentan + Tadalafil (Opsyvni®) - approved in Canada in 2021

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:

Riociguat (Adempas®) – approved in Canada in 2013 for the treatment of WHO group 4 CTEPH (inoperable or residual PH post-pulmonary endarterectomy [PEA]); also approved in 2014 for WHO group 1 PAH
Download RIOCIGUAT (ADEMPAS®) FOR CTEPH Info Sheet
Download RIOCIGUAT (ADEMPAS®) FOR PAH Info Sheet

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:

Selexipag (Uptravi®) – approved in Canada in 2016
Download SELEXIPAG (UPTRAVI®) Info Sheet

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)

New PH research advances are continuously paving the way toward 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.

Treatment Options

Treatment Options

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