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 Macitentan & Tadalafil (Opsyvni) Oral 2021 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 Macitentan & Tadalafil (Opsyvni) Oral 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®) 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. In some cases, PH remains after PTE surgery. Those patients also might be treated with BPA or medication. For more information on PEA surgery, click here. For more information about BPA, click here.
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 Macitentan & Tadalafil (Opsyvni) Oral 2021 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 Macitentan & Tadalafil (Opsyvni) Oral 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®) 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. In some cases, PH remains after PTE surgery. Those patients also might be treated with BPA or medication. For more information on PEA surgery, click here. For more information about BPA, click here.