Abstract Background Pulmonary hypertension (PH) is a common comorbidity of interstitial lung disease (ILD), associated with impaired quality of life and increased mortality. Despite its substantial clinical burden, diagnosis is often delayed. Recent data for inhaled treprostinil in PH-ILD suggest that timely detection of PH may improve outcomes. Methods This was a retrospective, multicenter study aiming to identify clinical features that predict PH in patients with ILD and to propose a scoring system that optimizes the selection of patients for right heart catheterization (RHC). In the derivation cohort (ILD cohort - University of South Florida), we identified predictors of PH in ILD patients and created a scoring system using the rounded value of odds ratio for each significant parameter. Association with outcomes was investigated both in the derivation and validation cohort (Idiopathic Pulmonary Fibrosis cohort - Cleveland Clinic). Results We included 165 patients with ILD that underwent RHC. Parameters that significantly predicted precapillary PH in the derivation cohort were: age42 years, heart rate recovery at 1 minute ≤15 bpm in 6-minute walk test (6MWT), 6MWT≤270 m, B-type natriuretic peptide (BNP)136 pg/ml, pulmonary artery (PA) to ascending aorta ratio1 in high-resolution computed tomography (HRCT), PA diameter29 mm in HRCT, right ventricular systolic pressure42 mmHg. A risk scoring system using these parameters was developed (range:0-28) and receiver operating characteristic (ROC) identified an optimal cut-off threshold (14) with 80% sensitivity, 68% specificity, with AUC: 0.78 for the detection of PH. A simplified scoring system without BNP (range: 0-24) maintained the same AUC. The simplified scoring system exhibited positive correlation with mean pulmonary artery pressure both in the derivation (r = 0.471, p 0.0001) and validation cohort (r = 0.33, p = 0.02). Conclusions This study demonstrates that incorporation of demographic, functional, imaging, and echocardiographic characteristics can enhance the early identification of precapillary PH in patients with ILD, paving the way to structured screening strategies. This abstract is funded by: None
Karampitsakos et al. (Fri,) studied this question.