Elevated pulmonary vascular resistance is associated with increased risk of death in IPF

Pulmonary hypertension (PH) is a common finding in idiopathic pulmonary fibrosis (IPF) and is associated with poor outcomes. Our objective was to evaluate the survival associations of PH in IPF. Methods: We performed a retrospective review of adult IPF patients in the Scientific Registry of Transplant Recipients between Jan 2005 and Dec 2014. Patients were classified as: PH absent (mean pulmonary artery pressure (mPAP) Borderline PH, preserved pulmonary vascular resistance (PVR) (mPAP 21-24 mmHg, PVR Borderline PH, high PVR (PVR ≥3 WU) PH, preserved PVR (mPAP 25-34 mmHg, PVR PH, high PVR (PVR ≥3 WU) Severe PH (mPAP ≥35 mmHg) Groups were evaluated by log-rank test and Cox regression modeling. Patients were censored at transplant. Results: Of 5890 IPF patients, 2832 (48%) had PH, 921 (16%) had severe PH, and 1119 died. Median survival was 1.7y (95% CI 1.5-2.6y) for group 1, and 1.0y (95% CI 0.8-1.5y) for group 6 (p Conclusions: Increased PVR appears to predict poorer survival regardless of mPAP in patients with IPF. These findings suggest that detailed hemodynamic assessment should be part of risk stratification in patients with IPF, even in the absence of clinically apparent PH.