The condition primarily affects persons between the ages of 20 and 40, and it affects women more frequently than it does men. Between 313 and 767 Canadian adults are thought to have Pulmonary Arterial Hypertension. Based on symptoms and function level, the World Health Organization has created a classification system for PAH. Patients may fall into Functional Classes (FC) I through IV, with higher numbers indicating more severe conditions.
Oxygen, diuretics to reduce fluid retention, anticoagulants to prevent blood clots, and other treatments focused at alleviating symptoms are all examples of supportive care. The majority of patients will need advanced therapy, or Pulmonary Arterial Hypertension Drug designed to address the disease itself. Canada has authorised eight of these medications. They fall into four categories:
prostanoids (injectables: epoprostenol, treprostinil) (injectables: epoprostenol, treprostinil)
anti-endothelin receptor drugs (oral: bosentan, ambrisentan, macitentan)
inhibitors of phosphodiesterase type 5 (PDE-5) (oral: sildenafil [both oral and injectable], tadalafil)
stimulator of soluble guanylate cyclase (sGC) (oral: riociguat)
Patients who cannot get their disease under control with monotherapy (one medicine) are typically given the option of combination therapy, which involves two drugs.
