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±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.