Pulmonary fibrosis without emphysema (HR 4.42, 95% CI 1.41–13.87, p = 0.01), PVR > 5 WU (HR 2.17, 95% CI 1.53–3.57, p = 0.03), 6MWD < 150 m (HR 2.50, 95% CI 1.41–8.45, p = 0.04), BNP > 200 pg/dL (HR 2.77, 95% CI 1.18–6.51, p = 0.02), and age > 65 years (HR 3.30, 95% CI 1.36–8.02, p = 0.01) were identified to be significant and independent predictors of one-year mortality. Here, NPPB is linked to pulmonary fibrosis.