Revista Portuguesa de Pneumologia Revista Portuguesa de Pneumologia
Rev Port Pneumol 2017;23:124-31 - Vol. 23 Num.3 DOI: 10.1016/j.rppnen.2017.01.006
Original article
Real-world, long-term survival of incident patients with pulmonary arterial hypertension
P. Marques-Alvesa, R. Baptistaa,b, A. Marinho da Silvaa, M. Pêgoa, G. Castroa,,
a Pulmonary Vascular Unit, Department of Cardiology A, Centro Hospitalar e Universitário de Coimbra, Praceta Mota Pinto, Coimbra, Portugal
b CNC.IBILI Research Consortium, University of Coimbra, Coimbra, Portugal
Received 08 September 2016, Accepted 16 January 2017

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.


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.


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.


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.

Pulmonary arterial hypertension, Survival, Predictors of mortality, Predictors of heart failure
Rev Port Pneumol 2017;23:124-31 - Vol. 23 Num.3 DOI: 10.1016/j.rppnen.2017.01.006