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Pulmonary Hypertension as a Prognostic Indicator at the Initial Evaluation in Idiopathic Pulmonary Fibrosis

Kimura M.a · Taniguchi H.b · Kondoh Y.b · Kimura T.b · Kataoka K.b · Nishiyama O.c · Aso H.a · Sakamoto K.a · Hasegawa Y.a
aDepartment of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, bDepartment of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, and cDepartment of Respiratory Medicine and Allergology, Kinki University School of Medicine, Osakasayama, Japan Respiration 2013;85:456-463 (DOI:10.1159/000345221)


Background: The impact of pulmonary hypertension (PH) on survival has been demonstrated in severe cases with idiopathic pulmonary fibrosis (IPF) who were referred for transplantation. However, whether PH is a predictor of survival remains unclear in milder cases. Objectives: To evaluate the survival impact of pulmonary artery pressure measured during the initial evaluation in patients with IPF. Methods: We retrospectively analyzed the initial evaluation data of 101 consecutive IPF patients undergoing right heart catheterization. Patients evaluated with supplemental oxygen were excluded. Predictors of 5-year survival were analyzed using the Cox proportional model. Results: The mean forced vital capacity (FVC) % predicted, diffusing capacity of the lung for carbon monoxide (DLCO) % predicted, and mean pulmonary artery pressure (MPAP) were 70.2 ± 20.1%, 47.9 ± 19.5%, and 19.2 ± 6.5 mm Hg, respectively. A univariate Cox proportional hazard model showed that the body mass index, %FVC, %DLCO, baseline PaO2, modified Medical Research Council score, 6-min walk distance, and lowest SpO2 of the 6-min walk test were significantly predictive of survival. The MPAP and pulmonary vascular resistance of right heart catheterization were also significant. With stepwise, multivariate Cox proportional analysis, MPAP (HR = 1.064; 95% CI 1.015-1.116, p = 0.010) and %FVC (HR = 0.965, 95% CI 0.949-0.982, p < 0.001) were independent determinants of survival. Analysis of the receiver operating curve revealed MPAP >20 mm Hg to be optimal for predicting the prognosis. Conclusions: Higher MPAP and lower %FVC at the initial evaluation were significant independent prognostic factors of IPF. The current results suggested the importance of the initial evaluation of PH for patients with IPF.


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