A critical analysis of survival in idiopathic pulmonary arterial hypertension

Idiopathic pulmonary arterial hypertension (PAH) is a rare disease characterized by a progressive increase in pulmonary vascular resistance leading to right heart failure and death. Recent survival data in PAH cohorts have been biased by the inclusion of prevalent patients (survivors) who have a better prognosis than incident cases. Nevertheless, a critical analysis of recent multicenter incident cohorts has shown survival improvements in the current management era, thanks to better management. However idiopathic PAH remains a severe disease with a poor prognosis. Current recommendations emphasize the necessity of an aggressive goal-oriented management of symptomatic PAH. Modern survival figures also highlight the relevance of lung transplantation in eligible PAH patients refractory to current best standard of care.