Treatment Options

There is currently no cure for PH, but thanks to available treatments, many patients are living longer, healthier lives. 

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  • 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 Treatments 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
Epoprostenol (Caripul®)		 Intravenous 2013
Treprostinil (Remodulin®)

Subcutaneous

Intravenous

2002

2005


Available Treatments 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. 

Successful surgical outcomes can include an improved life expectancy.

For more information on PEA surgery, click here.

Sometimes It's PH

Additional Resources

Information Sheet for Medical Professionals
Download Resource 

PH Clinical Directory
Access Resource

Clinical Practice Guidelines
Access Resource 

The Impact of PH on Canadians
Access Resource 

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Comprehensive information on pulmonary hypertension for medical professionals.