Pulmonary Arterial Hypertension

The treatment options for patients with PAH can be broadly classified into three main groups – general measures, supportive measures and PAH-specific therapies.

• General Measures
  General measures include the avoidance of strenuous activities, the avoidance of pregnancy through careful family planning and the use of appropriate contraception, and timely vaccination for influenza and pneumococcus.
• Supportive Measures
  Supportive measures include the use of oxygen for patients who require long-term oxygen therapy, the use of diuretics to help relieve organ congestion and the use of oral anticoagulation to help to prevent thromboembolism to the lungs.
• PAH-specific therapy
  PAH-specific therapy mainly targets three main pathways. The first pathway targets the Nitric Oxide Pathway including the synthesis of nitric oxide, as well as nitric oxide, soluble guanylate cyclase and cyclic GMP signaling pathway. The drugs in this category include medication such as sildenafil, tadalafil and roiciguat. The second group of medication targets Endothelin Receptors (eg. Bosentan) and the final pathway targets the Prostacyclin Pathway which includes drugs such as inhaled iloprost, iv prostacyclin etc.