Predictors of Mortality in Patients with Interstitial Lung Disease-Associated Pulmonary Hypertension

Abstract

Background: Pulmonary hypertension (PH) is a well-established complication in interstitial lung disease (ILD) patients. The aim of this study is to investigate the physiological and hemodynamic parameters that predict mortality in patients with ILD-PH. Methods: Consecutive ILD patients who underwent right heart catheterization (n = 340) were included. The information analyzed included demographics and physiological and hemodynamic parameters. Cox regression models were used to identify independent predictors of survival. Results: In total, 96 patients had PH and an additional 56 patients had severe PH. The overall survival of idiopathic pulmonary fibrosis (IPF) patients with PH was significantly worse than the survival of patients with other types of ILD with PH (p < 0.0001 by log-rank analysis). Patients with a reduced diffusing capacity of the lung for carbon monoxide (DLco) (<35% predicted), six-minute walk test final oxygen saturation by pulse oximetry (SpO2) <88% and pulmonary vascular resistance ≥4.5 Wood units in the ILD-PH cohort had significantly worse survival. IPF diagnosis, forced vital capacity, DLco, systolic pulmonary artery pressure and cardiac index were identified as independent predictors of survival among the ILD-PH cohort. Conclusions: Patients with ILD-PH have poor prognosis. Physiological and hemodynamic parameters were important factors independently associated with outcome.

Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement

“…The recognition of PH in the course of interstitial lung disease (ILD) is burdened with a poor prognosis [17]. Negative prognostic factors in PH-ILD were identified as TLCO < 35%, 6MWT desaturation < 88%, and PVR > 4.5 WU [17].…”

“…The recognition of PH in the course of interstitial lung disease (ILD) is burdened with a poor prognosis [17]. Negative prognostic factors in PH-ILD were identified as TLCO < 35%, 6MWT desaturation < 88%, and PVR > 4.5 WU [17]. The worst outcome is observed in IPF patients, but PH in the course of CTD with UIP-like lung involvement carries a comparably poor prognosis [10,17].…”

Acute exacerbation in interstitial lung disease

“…As such, our findings are consistent with other racial cohorts, and importantly, we have identified additional variables highlighting the importance of parameters obtained from the 6MWT and RHC as prognostic markers in ILD patients. [ 43 ]…”

PH-ILD: Identification, Evaluation, and Monitoring: A Diagnostic View From Both Sides

“…23 A study of 340 ILD patients undergoing RHC showed 96 (28%) of patients with PH, of which 56 were considered to be severe. 24 In a study of 135 patients with IPF being evaluated for lung transplantation, 39 patients (29%) had PH-ILD. 25 Evaluation of 488 IPF patients with mild or moderate restrictive disease showed that 14% of subjects met the criteria for PH-ILD.…”

“…33 Findings similar to those in IPF have been reproduced in more general ILD population with reduced 6MWD and survival noted in patients with PH-ILD. 24,34 The severity of PH in the context of ILD is believed to generally be biased toward mild to moderate elevations in pulmonary arterial pressures. 3 In addition to fundamental pathophysiologic differences, other explanations for this include classification bias (patients with severe PH are classified as group 1) and survivorship bias (patients with advanced PH and ILD do not survive or are transplanted).…”