FOR PHYSICIANS
Treatment Options
There is currently no cure for pulmonary hypertension (PH), but thanks to available treatments, many patients are living longer, healthier lives.
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11 PAH treatments are approved in Canada to slow disease progression and alleviate symptoms.
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There is a potential cure for CTEPH through surgery. Approved medical treatment may also slow disease progression and alleviate symptoms.
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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®)
Bosentan (Tracleer®)
Macitentan (Opsumit®)
Macitentan & Tadalafil (Opsyvni)
Oral
Oral
Oral
Oral
2008
2001
2013
2021
Phosphodiesterase inhibitors (PDE 5 inhibitors) - allow the lungs to produce more natural vasodilators.
Name
Administration
Health Canada Approval
Sildenafil (Revatio®, Viagra®)
Tadalafil (AdCirca®, Cialis®)
Macitentan & Tadalafil (Opsyvni)
Oral
Oral
Oral
2006
2010
2021
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®)
Room temperature stable
Epoprostenol (Caripul®)
Treprostinil (Remodulin®)
Intravenous
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Intravenous
Subcutaneous Intravenous
1997
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2013
2002
2005
Activin Signalling Inhibitors - restores balance between the growth-promoting activin differentiation factor pathway and the growth-inhibiting BMP pathway
Name
Administration
Health Canada Approval
Sotatercept (Winrevair™)
Subcutaneous
2024
AVAILABLE TREATMENT FOR CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION (CTEPH)
Medical Therapies
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:
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If the blockage is reachable through surgery - if the blockage is too deep within the lungs, it may not be reachable;
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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, a high chance of symptomatic improvement or resolution, and improved long-term survival.
In some cases, PH remains after PTE surgery. Those patients also might be treated with BPA or medication.