New treatments for PAH approved over the last two decades have led to major improvements in the symptoms and prognosis of this serious condition. These include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, guanylate cyclase stimulators and prostacyclin agents, both analogues and agonists. Clinical trials of new agents are ongoing.
Specialist supervision of all patients with pulmonary arterial hypertension remains essential. Treatment is increasingly complex and expensive and requires careful ongoing scrutiny.