Pulmonary Hypertension as a Prognostic Indicator at the Initial Evaluation in Idiopathic Pulmonary Fibrosis
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 PaO(2), modified Medical Research Council score, 6-min walk distance, and lowest SpO(2) 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.
Full-textDOI: · Available from: Osamu Nishiyama, May 17, 2015
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ABSTRACT: BackgroundLung-dominant connective tissue disease (LD-CTD) is a new concept for classifying the subset of patients with interstitial pneumonia who have clinical features suggesting an associated CTD, but whose features fall short of a clear diagnosis of CTD under the current rheumatologic classification systems. The impact of mean pulmonary arterial pressure (MPAP) in LD-CTD has not been sufficiently elucidated.ObjectivesTo evaluate the survival impact of MPAP measured during the initial evaluation in patients with LD-CTD.MethodsWe retrospectively analyzed the initial evaluation data of 100 LD-CTD patients undergoing pulmonary function test, 6-min walk test (6MWT), and right heart catheterization (RHC).ResultsThe mean MPAP was 16.2±4.4 mm Hg, and 18 patients had MPAP≥20 mm Hg. A univariate Cox proportional hazard model showed that MPAP and several variables have a statistically significant impact on survival. With stepwise, multivariate Cox proportional analysis, MPAP (HR = 1.293; 95% CI 1.130–1.480; p<0.001) and mean forced vital capacity (FVC) % predicted (HR = 0.958; 95% CI 0.930–0.986; p = 0.004) were shown to be independent determinants of survival.ConclusionsHigher MPAP and lower %FVC at the initial evaluation were significant independent prognostic factors of LD-CTD. MPAP evaluation provides additional information of disease status and will help physicians to predict mortality in LD-CTD.PLoS ONE 09/2014; 9(9):e108339. DOI:10.1371/journal.pone.0108339 · 3.53 Impact Factor
International Journal of Cardiology 01/2015; 178. DOI:10.1016/j.ijcard.2014.10.120 · 6.18 Impact Factor
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ABSTRACT: Pulmonary hypertension (PH) is a comorbidity associated with idiopathic pulmonary fibrosis (IPF). There is limited research regarding the effect on survival after lung transplantation (LTx). To assess the effect of PH on survival in patients with IPF who received LTx, the United Network for Organ Sharing was queried for eligible patients with recorded mean (PAmean) and systolic (PAsystolic) pulmonary artery pressure. The analysis was restricted to the post-lung allocation scoring system starting May 1, 2005, to provide a cohort receiving present-day therapies and management. The last update of the data set was July 6, 2012, so a cutoff date of July 6, 2011, was chosen to allow for the possibility of at least 1 year of follow-up. Thresholds of ≥25 and ≥35 mm Hg were chosen for PAmean and PAsystolic, respectively, as indicators of PH. Of 23,951 LTxs in the UNOS data set, 2,542 met inclusion criteria, 1,234 (49%) with PAmean ≥ 25 mm Hg and 1,680 (66%) with PAsystolic ≥ 35 mm Hg. PAmean and PAsystolic were highly correlated, with an estimated correlation coefficient ρ = 0.93 (p < 0.001). Patients with PH (PAmean ≥ 25 mm Hg or PAsystolic ≥ 35 mm Hg) tended to have higher ischemic times, lung allocation score values, forced vital capacity percentage predicted at LTx, and supplemental oxygen requirement at rest values. In addition, a larger proportion of patients with PH was African American, male, had diabetes, and received bilateral LTx compared with single LTx. Comparing PAmean < 25 vs ≥ 25 mm Hg and PAsystolic < 35 vs ≥ 35 mm Hg, median survival in months was 60.4 (95% confidence interval [CI], 55.2-80.4) vs 61.4 (95% CI, 56.9-66.9; log-rank p = 0.876) and 57.6 (95% CI, 50.9-68.0) vs 64.3 (95% CI, 57.5-71.3; log-rank p = 0. 247), respectively. Hazard ratios for both definitions of PH from univariable and multivariable Cox proportional hazard models were close to 1 and none were statistically significant. On the basis of our models and despite PH being prevalent, there is no strong evidence suggesting that PH significantly alters the risk of death in IPF patients after LTx. Copyright © 2014 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.The Journal of Heart and Lung Transplantation 09/2014; DOI:10.1016/j.healun.2014.09.004 · 5.61 Impact Factor