Real-world, long-term survival of incident patients with pulmonary arterial hypertension

Background: Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. Long-term outcomes data are scarce in Portugal. We aimed to estimate survival of newly diagnosed PAH at a Portuguese referral center in the modern management era. Methods: Between January 2009 and November 2015 all incident PAH cases were consecutively enrolled in a prospective cohort study. Sixty-five patients were followed up for a median of 3.1 [interquartile range 1.7-5.4] years. Kaplan-Meier survival analysis was used to estimate 1-, 3-, and 5-year survival and to compare it with a historical PAH survival estimated from the NIH cohort. Results: Mean age was 48 1 19 years with female preponderance (68%). The most common PAH subgroup was congenital heart disease (PAH-CHD) (n = 31; 48%), followed by connective tissue disease (PAH-CTD) (n = 16; 25%), idiopathic (IPAH) (n = 8; 12%) and hereditary (HPAP) (n =1; 1.5%). BNP values (hazard ratio [HR] 2.07; 95%CI 1.34-3.22; P = 0.001) and male gender [HR 4.34 (1.44-13.09); P = 0.009] were predictors of death. Survival rates at 1-, 3- and 5-years were 95%, 77% and 71%. Survival was not statistically different between PAH etiologies (Log-rank P = 0.7). However, PAH-CHD was associated with a decreased risk of the combined endpoint of all-cause mortality and admission for decompensated heart failure [HR 0.36 (0.15-0.85); P = 0.02]. We found a non-significant numerically higher survival of incident IPAH, HPAH and DPAH patients in comparison with the historical NIH cohort. Conclusions: In this cohort of incident PAH patients, PAH-CHD patients had better overall prognosis. Higher BNP values and male gender were associated with higher mortality. (C) 2017 Sociedade Portuguesa de Pneumologia. Published by Elsevier Espana, S.L.U.