Non-invasive risk stratification of pulmonary hypertension in idiopathic pulmonary fibrosis: a screening approach from a prospective observational study.

Abstract

Pulmonary hypertension (PH) is a major complication of idiopathic pulmonary fibrosis (IPF), but its detection remains challenging because right-heart catheterization is invasive and echocardiography has limited accuracy in advanced lung disease. We assessed the feasibility of a multiparametric non-invasive screening approach and its concordance with echocardiographic PH probability in patients with IPF. In this prospective single-center observational study, 27 patients with multidisciplinary-confirmed IPF were enrolled from November 2024 to May 2025. Patients were classified as having low, intermediate, or high PH risk using the PH-ILD Detection Tool, incorporating clinical, functional, imaging, and biomarker variables. All patients underwent transthoracic echocardiography and were classified according to echocardiographic PH probability. No patient underwent right-heart catheterization. The PH-ILD Detection Tool classified 14 patients as low risk, 5 as intermediate risk and 8 as high risk. Echocardiography indicated low PH probability in 14 patients, intermediate probability in 4, and high probability in 9. Thirteen of the 14 patients classified as low risk had a low echocardiographic probability. Higher echocardiographic PH probability was associated with greater dyspnea severity, NT-proBNP >300 ng/L, lower resting PaO₂ and lower nadir SpO₂. The PH-ILD Detection Tool showed promising concordance with echocardiographic PH probability and may help prioritize patients with IPF for cardiovascular assessment. However, the small sample size and absence of right-heart catheterization preclude diagnostic validation, and the findings should be considered exploratory.