Survival of patients with pulmonary arterial hypertension after the advent of specific pulmonary vasodilator therapies

Background: Pulmonary arterial hypertension (PAH) is a rare and progressive disease. Long-term survival remains poor despite of advances in specific vasodilator therapy. Aim: To describe the survival rate in a cohort of PAH patients in two referral centers in Chile. Patients and Methods: One hundred fifteen patients aged 43 +/- 15.6 years (85% females) with PAH qualified for this study. Their median pulmonary artery pressure was 55.4 +/- 14 mmHg and their six minutes walking capacity was 368 +/- 119 m. They were followed for 58 +/- 0.4 months and their actual survival rates were compared with the estimated survival using the equation proposed by the French registry of PAH. Results: One, two and three year survival rates were 97, 94 and 89%, respectively. The observed survival rates were greater than the estimated survival. Conclusions: The improvement in survival rates observed in this cohort of patients is similar to what has been described in literature.