PULMONARY hypertension affected most patients with progressive pulmonary fibrosis, while a CT measurement emerged as a screening clue.
In a retrospective study of 139 patients with interstitial lung disease who underwent echocardiography at a single hospital, pulmonary hypertension was identified in 76 patients (54.7%). Among the 61 patients with progressive pulmonary fibrosis (PPF), 37 (60.7%) had pulmonary hypertension. That proportion was similar to the 30 of 47 patients (63.8%) with idiopathic pulmonary fibrosis (IPF).
The investigators classified pulmonary hypertension using either a mean pulmonary artery pressure above 20 mm Hg on right heart catheterization or a high probability of pulmonary hypertension on echocardiography. PPF was classified using the 2022 guideline definition. Patients had undergone echocardiography between 2017 and 2021.
Within the PPF group, lower percent predicted forced expiratory volume in one second and diffusing capacity for carbon monoxide, higher brain natriuretic peptide, electrocardiographic signs of right heart strain, and a higher pulmonary artery-to-aorta diameter ratio on chest CT were associated with pulmonary hypertension in the initial analyses.
When brain natriuretic peptide, diffusing capacity, and the CT ratio were assessed together, only the pulmonary artery-to-aorta ratio remained statistically significant. A ratio cutoff of 0.97 yielded 75.7% sensitivity and 87.5% specificity in this cohort. Because chest CT is routinely used to assess interstitial lung disease, the measurement could help identify patients who warrant closer evaluation for pulmonary hypertension. The cutoff needs validation before it can guide screening on its own.
Pulmonary hypertension was not significantly associated with shorter survival among patients with PPF over the three-year observation period (hazard ratio, 1.32; 95% CI, 0.51–3.42). This finding does not establish that pulmonary hypertension has no prognostic effect: the estimate was imprecise, and the PPF group included several underlying diseases.
The study’s retrospective, single-center design also limits how broadly the prevalence estimate applies. Enrollment required echocardiography, potentially selecting patients already more likely to have pulmonary hypertension, and many classifications relied on echocardiographic probability rather than catheterization. Larger studies with catheterization-confirmed diagnoses are needed to test both the CT cutoff and its clinical value.
Reference
Takeda K et al. Prevalence and predictors of pulmonary hypertension in patients with progressive pulmonary fibrosis: A retrospective study. Respir Investig. 2026. doi:10.1016/j.resinv.2026.101512.
Featured Image: Sirikarn Rinruesee on Adobe Stock.
Featured Image: Sirikarn Rinruesee on Adobe Stock.
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