ABOUT PH

Treatment Options

Since 1997, 11 pulmonary hypertension-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).

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

The tabs below describe current treatment options for pulmonary arterial hypertension (Group 1 PH), and one medical treatment approved for CTEPH (Group 4 PH). Find out more about treatments for CTEPH.

Other types of pulmonary hypertension (WHO Groups 2, 3, and 5) are primarily treated by treating the underlying condition or cause.

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

Treatment Options

Patients with pulmonary arterial hypertension make excess endothelin, one of the most powerful chemicals which constricts blood vessels. This causes cells to multiply, narrowing pulmonary arteries and increasing pulmonary arterial pressure. Endothelin receptor antagonists (ERAs) block the action of this excess endothelin opening up pulmonary arteries and reducing pulmonary arterial pressure, as well as improving right ventricular function. ERAs also make cells stop multiplying (they are anti-proliferative) and decrease scar tissue formation (they are anti-fibrotic).

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 pulmonary arterial hypertension 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 nitric oxide. PDE-5 inhibitors are pulmonary vasodilators that can open up pulmonary arteries and reduce pulmonary arterial pressure, as well as improve right-side heart 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 pulmonary arterial hypertension 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 pulmonary arterial pressure, as well as improve right-side heart function.

sGC stimulators include:

Riociguat (Adempas®) – approved in Canada in 2013 for the treatment of CTEPH (WHO Group 4 PH) which is inoperable, or residual pulmonary hypertension after pulmonary endarterectomy [PEA] surgery. It was also approved in 2014 for WHO group 1 pulmonary arterial hypertension

Download riociguat (Adempas®) for CTEPH Info Sheet

Download riociguat (Adempas®) for pulmonary arterial hypertension Info Sheet

Patients with pulmonary arterial hypertension 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 pulmonary arterial pressure, as well as improve right-side heart function. IP receptor agonists also make cells stop multiplying (they are anti-proliferative) and decrease blood clot formation (they are anti-platelet).

IP receptor agonists include:

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

Patients with pulmonary arterial hypertension 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 pulmonary arterial pressure, as well as improve right-side heart function. Prostacyclin analogues also make cells stop multiplying (they are anti-proliferative) and decrease blood clot formation (they are anti-platelet).

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 needle 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 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)

The pathway associated with BMPR2 signalling is important in controlling cell growth in pulmonary arteries. In patients with pulmonary arterial hypertension, this signal seems to be under-active, and a competing pathway known as the activin pathway becomes predominant and stimulates abnormal cell growth. The activin signalling inhibitor binds the activins and inhibits abnormal cell proliferation in the pulmonary arteries.

Activin signalling inhibitors include:

Sotatercept (Winrevair™) – approved in Canada in 2024

New research advances are continuously paving the way toward better, more personalized treatment options for pulmonary hypertension.

Find out more about ongoing clinical trials in Canada and how you could help advance the future of pulmonary hypertension medicine.
Find out more about patient-oriented research.

Thanks to advancements in research, many treatments for pulmonary arterial hypertension 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 pulmonary hypertension community.

Treatment Options

Treatment Options

What role can patients play in PH research?