Skip to main content
There is currently no cure for PH, but thanks to available treatments, many patients are living longer, healthier lives. 
  • 10 PAH treatments are approved in Canada to slow disease progression and alleviate symptoms.
  • There is a potential cure for CTEPH through surgery. Approved medical treatment may also slow disease progression and alleviate symptoms.
  • Centres specialized in the treatment of PH (adult and pediatric) and CTEPH are located throughout Canada.

AVAILABLE TREATMENT FOR PULMONARY ARTERIAL HYPERTENSION (PAH) 

 

Medical Therapies 

Endothelin receptor antagonists (ERAs) - help prevent blood vessels from narrowing.
 
Name  Administration Health Canada Approval 
Ambrisentan (Volibris®)   Oral  2008
Bosentan (Tracleer®)   Oral  2001
Macitentan (Opsumit®)   Oral  2013
 
Phosphodiesterase inhibitors (PDE 5 inhibitors) - allow the lungs to produce more natural vasodilators.
 
Name Administration  Health Canada Approval 
Sildenafil (Revatio®, Viagra®) Oral 2006
Tadalafil (AdCirca®, Cialis®)  Oral 2010
 
Soluble guanylate cyclase stimulator (sCG stimulator) - increases the sensitivity of sGC to endogenous nitric oxide (NO) and directly stimulates the receptor to mimic the action of NO.
 
Name Administration  Health Canada Approval 
Riociguat (Adempas®) Oral 2014
 
Oral IP receptor agonist – stimulates the IP prostacyclin receptor to cause vasodilation.
 
Name Administration Health Canada Approval
Selexipag (Uptravi®) Oral 2016
 
Prostacyclins - open up the blood vessels and help ease symptoms of PH, including chest pain and shortness of breath.
 
Name Administration Health Canada Approval
Epoprostenol (Flolan®) Intravenous 1997
Room temperature stable  Intravenous 2013
Epoprostenol (Caripul®)
Treprostinil (Remodulin®) Subcutaneous 2002
Intravenous 2005 


AVAILABLE TREATMENT FOR CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION (CTEPH) 

 

Medical Therapy  

Soluble guanylate cyclase stimulator (sCG stimulator) - increases the sensitivity of sGC to endogenous nitric oxide (NO) and directly stimulates the receptor to mimic the action of NO.
 
Name Administration Health Canada Approval
Riociguat (Adempas®) Oral 2013 for the treatment of non-operative or residual post-operative CTEPH (WHO class IV)
 

Surgical Treatment

The standard and potentially curative treatment for patients who have developed CTEPH and are suitable for surgery is a procedure called pulmonary endarterectomy (PEA). PEA is major surgery that clears the blood vessels of the lungs of clots and scar material presented in the disease. To determine whether a patient is a suitable candidate for surgery, they will need to be thoroughly assessed at a centre specialized in the treatment of CTEPH. CTEPH specialists will assess:
 
  • If the blockage is reachable through surgery - if the blockage is too deep within the lungs, it may not be reachable;
  • The patient's overall suitability to undergo surgery. 

If a patient is accepted for surgery at a high-volume centre, it is associated with a low mortality rate, high chance of symptomatic improvement or respolution, and improved long-term survival.
 
For more information on PEA surgery, click here.