Background: The influence of pulmonary hypertension (PH) on survival of patients with advanced idiopathic pulmonary fibrosis (IPF) is not verified in China.
Methods: The characteristics of 199 patients with IPF receiving lung transplantation evaluation from January 2015 to August 2016 were queried. The waiting time was tracked from wait list entry date until death or censoring, the censoring issues including receiving lung transplantation, removing from the waiting list without transplant, and still waiting for donor until the last follow-up day (2016-09-30). The patients were divided to 2 groups to determine the effect of PH on survival prior to transplant on the basis of different definitions of PH. Mild PH was defined as mean pulmonary artery pressure (mPAP) ≥25 mmHg and severe ≥ 35 mmHg by right heart catheterization measurements. The study was approved by ethics committee of our hospital and written informed consent was signed by patient.
Results: Of 199 patients, there were 149 males and 50 males, with mean age of 58.70 years old, 96 patients received lung transplantation, and the remaining 103 not, 115 with mPAP ≥25 mmHg and 76 with mPAP ≥35 mmHg.A total of 58 cases (29.15%) died on the waiting list, 31 were removed from the list prior to transplant, and 14 still on the waiting list. Kaplan–Meier survival function showed survival of patients with mild PH or severe PH was significant inferior to that of without PH(P < 0.001). Using Cox proportional hazards models, univariate analysis revealed significant differences in survival for mild PH (HR = 1.872,95% CI 1.249-3.482, p = 0.001) and severe PH (HR = 3.267,95% CI 2.135-5.267, p < 0.001). Multivariate Cox models identified significant risk for death for mild PH (HR = 1.624,95% CI 1.276-3.035, p < 0.001) and severe PH HR = 2.932,95% CI 1.849-3.649, p < 0.001).
Conclusions: PH is associated with significant increased risk of death among IPF patients waiting for lung transplantation, and early referral for lung transplantation is warranted for IPF with PH.