Abstract
Idiopathic pulmonary fibrosis (IPF) is a leading indication for lung transplantation. Pulmonary hypertension (PH), a common comorbidity in IPF, has gained renewed attention following the updated ESC/ERS guidelines, which redefine diagnostic thresholds for PH. This study evaluates the impact of the revised PH criteria on transplant waitlist outcomes among IPF patients. Specifically, we assessed the prevalence of PH under the new guidelines and its association with waitlist survival. We conducted a retrospective analysis using the OPTN/SRTR database, including 14,156 IPF candidates listed for lung transplantation. Survival analyses were performed using Kaplan-Meier and multivariate models to examine the influence of revised mPAP and PVR thresholds on waitlist mortality. The prevalence of PH, defined by the revised criteria, was significantly higher compared to the prior definition. Kaplan-Meier analysis demonstrated worse waitlist survival for patients with PH under both diagnostic thresholds. However, multivariate analysis revealed that mPAP and PVR thresholds were not independently predictive of mortality. Instead, clinical parameters, including 6MWD, functional status, BMI, FVC, PaCO2, and double lung transplant preference, were significant predictors of waitlist mortality. In conclusion, while the revised PH diagnostic criteria increase PH prevalence in IPF patients, their independent prognostic utility for waitlist survival is limited. This national transplant database study underscores the importance of comprehensive clinical evaluation and timely referral for transplantation in managing IPF with PH.