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Respiration. 2013;85(6):456-63. doi: 10.1159/000345221. Epub 2012 Dec 19.

Pulmonary hypertension as a prognostic indicator at the initial evaluation in idiopathic pulmonary fibrosis.

Author information

1
Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Abstract

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.

PMID:
23257350
DOI:
10.1159/000345221
[Indexed for MEDLINE]
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