https://doi.org/10.4081/monaldi.2026.3794
Non-invasive risk stratification of pulmonary hypertension in idiopathic pulmonary fibrosis: a screening approach from a prospective observational study
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Published: 18 September 2026
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.
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Sonti R, Gersten RA, Barnett S, et al. Multimodal noninvasive prediction of pulmonary hypertension in IPF. Clin Respir J 2019;13:567‑73. DOI: https://doi.org/10.1111/crj.13059
Nathan SD. Pulmonary hypertension in interstitial lung disease. Int J Clin Pract Suppl 2008;62:21‑8. DOI: https://doi.org/10.1111/j.1742-1241.2008.01624.x
Shorr AF, Wainright JL, Cors CS, et al. Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant. Eur Respir J 2007;30:715‑21. DOI: https://doi.org/10.1183/09031936.00107206
Arcasoy SM, Christie JD, Ferrari VA, et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003;167:735‑40. DOI: https://doi.org/10.1164/rccm.200210-1130OC
Zisman DA, Ross DJ, Belperio JA, et al. Prediction of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 2007;101:2153‑9. DOI: https://doi.org/10.1016/j.rmed.2007.05.012
Ketfi A, Selatni F, Djouadi C, Touahri R. Clinical and functional characteristics of interstitial lung disease in algeria: a single-center prospective study. J Respir 2024;4:12-25. DOI: https://doi.org/10.3390/jor4010002
Ketfi A, Tahiat A, Djouadi C, et al. Lung function data of North-African patients with rheumatoid arthritis: acomparative study between anti-citrullinated peptides antibodies positive and negative patients. Tunis Med 2022;100:626‑41.
Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2018;198:e44‑68. DOI: https://doi.org/10.1164/rccm.201807-1255ST
Raymond TE, Khabbaza JE, Yadav R, Tonelli AR. Significance of main pulmonary artery dilation on imaging studies. Ann Am Thorac Soc 2014;11:1623‑32. DOI: https://doi.org/10.1513/AnnalsATS.201406-253PP
Alkukhun L, Wang XF, Ahmed MK, et al. Non-invasive screening for pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 2016;117:65‑72. DOI: https://doi.org/10.1016/j.rmed.2016.06.001
Quanjer PH, Stanojevic S, Cole TJ, et al. Multi-ethnic reference values for spirometry for the 3–95-yr age range: the global lung function 2012 equations. Eur Respir J 2012;40:1324‑43. DOI: https://doi.org/10.1183/09031936.00080312
Ketfi A, Gharnaout M, Bougrida M, Saad HB. The multi‐ethnic global lung initiative 2012 (GLI-2012) norms reflect contemporary adult’s Algerian spirometry. PLoS One 2018;13:e0203023. DOI: https://doi.org/10.1371/journal.pone.0203023
Collard HR, King TE, Bartelson BB, et al. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003;168:538‑42. DOI: https://doi.org/10.1164/rccm.200211-1311OC
Eaton T, Young P, Milne D, Wells AU. Six-minute walk, maximal exercise tests: reproducibility in fibrotic interstitial pneumonia. Am J Respir Crit Care Med 2005;171:1150-7. DOI: https://doi.org/10.1164/rccm.200405-578OC
Leuchte HH, Neurohr C, Baumgartner R, et al. Brain natriuretic peptide and exercise capacity in lung fibrosis and pulmonary hypertension. Am J Respir Crit Care Med 2004;170:360‑5. DOI: https://doi.org/10.1164/rccm.200308-1142OC
Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2023;61:2200879. DOI: https://doi.org/10.1183/13993003.00879-2022
Parikh R, Konstantinidis I, O’Sullivan DM, Farber HW. Pulmonary hypertension in patients with interstitial lung disease: a tool for early detection. Pulm Circ 2022;12:e12141. DOI: https://doi.org/10.1002/pul2.12141
Parikh R, O’Sullivan DM, Farber HW. The PH-ILD detection tool: external validation and use in patients with ILD. Pulm Circ 2023;13:e12273. DOI: https://doi.org/10.1002/pul2.12273
Waxman AB, Elia D, Adir Y, et al. Recent advances in the management of pulmonary hypertension with interstitial lung disease. Eur Respir Rev 2022;31:210220. DOI: https://doi.org/10.1183/16000617.0220-2021
Raghu G, Amatto VC, Behr J, Stowasser S. Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review. Eur Respir J 2015;46:1113‑30. DOI: https://doi.org/10.1183/13993003.02316-2014
Nathan SD, Shlobin OA, Ahmad S, et al. Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Respiration 2008;76:288‑94. DOI: https://doi.org/10.1159/000114246
Piccari L, Kovacs G, Jones S, et al. The European Voice of the Patient living with pulmonary hypertension associated with interstitial lung disease: diagnosis, symptoms, impacts, and treatments. Pulm Circ 2024;14:e12405. DOI: https://doi.org/10.1002/pul2.12405
Tello K, Ghofrani HA, Heinze C, et al. A simple echocardiographic estimate of right ventricular-arterial coupling to assess severity and outcome in pulmonary hypertension on chronic lung disease. Eur Respir J 2019;54:1802435. DOI: https://doi.org/10.1183/13993003.02435-2018
Hoeper MM, Behr J, Held M, et al. Pulmonary hypertension in patients with chronic fibrosing idiopathic interstitial pneumonias. PLoS One 2015;10:e0141911. DOI: https://doi.org/10.1371/journal.pone.0141911
Lettieri CJ, Nathan SD, Barnett SD, et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006;129:746‑52. DOI: https://doi.org/10.1378/chest.129.3.746
Minai OA, Santacruz JF, Alster JM, et al. Impact of pulmonary hemodynamics on 6-min walk test in idiopathic pulmonary fibrosis. Respir Med 2012;106:1613‑21. DOI: https://doi.org/10.1016/j.rmed.2012.07.013
Nathan SD, Chandel A, Wang Y, et al. Derivation and validation of a noninvasive prediction tool to identify pulmonary hypertension in patients with IPF: evolution of the model FORD. J Heart Lung Transplant 2024;43:547‑53. DOI: https://doi.org/10.1016/j.healun.2023.11.005
Furukawa T, Kondoh Y, Taniguchi H, et al. A scoring system to predict the elevation of mean pulmonary arterial pressure in idiopathic pulmonary fibrosis. Eur Respir J 2018;51:1701311. DOI: https://doi.org/10.1183/13993003.01311-2017
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