Pulmonary Hypertension and Pulmonary Function Testing in Idiopathic Pulmonary Fibrosis

doi:10.1378/chest.06-2485

Chest

Volume 131, Issue 3, March 2007, Pages 657-663

https://doi.org/10.1378/chest.06-2485 Get rights and content

Abstract

Background:Pulmonary hypertension (PH) is commonly seen in patients with idiopathic pulmonary fibrosis (IPF). We sought to examine the relationship between pulmonary function tests (PFTs), including the percentage of predicted FVC (FVC%), percentage of predicted total lung capacity, percentage of predicted diffusing capacity of the lung for carbon monoxide (Dlco%), the composite physiologic index (CPI), and PH. The ability of FVC%, Dlco%, and FVC%/Dlco% ratio to predict underlying PH was assessed.

Methods:Retrospective review of IPF patients seen at a tertiary referral center over an 8-year interval in whom both PFT and right-heart catheterization data were available.

Results:The study cohort consisted of 118 patients, of whom 48 patients (40.7%) had PH. There was no correlation between measures of lung volumes or the CPI with underlying PH. There was a modest association between Dlco% and PH, with Dlco% < 30 having a twofold-higher prevalence of PH (56.4%) compared to Dlco% ≥ 30 (28.6%). Cardiac dysfunction might have played a small role, since 16.1% of the patients had an associated elevated pulmonary capillary wedge pressure. There was a trend to a higher prevalence and greater severity of PH in those patients with FVC% > 70 compared to the group with FVC% < 40.

Conclusion:PH is common in patients with IPF. There is a poor correlation between lung function measures and PH, suggesting that factors other than fibrosis may play a role in the etiology. The unexpected high prevalence and severity of PH in patients with well-maintained lung function have implications for the prognosis and management of the disease.

Section snippets

Materials and Methods

The study population consisted of patients with IPF seen at our institution over an 8-year period (from 1997 to 2005) in whom right-heart catheterization (RHC) and pulmonary function studies were available for analysis. Our institution has programs for both interstitial lung disease and lung transplantation. The criteria for performing RHC was age < 65 years in the context of evaluation for lung transplantation. Catheterization in patients > 65 years old was performed at the discretion of the

Results

Over an 8-year period, 347 patients with IPF were evaluated. Of these, 118 patients with RHC and PFT data qualified for the analysis. IPF was diagnosed in all patients as per standard guidelines.¹⁴Of the 118 patients, 86 patients (73%) underwent surgical biopsy or had lung explant tissue consistent with the diagnosis. Patient demographics are shown inTable 1. All but 12 of the patients were ≤ 65 years old. Of the cohort, 48 patients (40.7%) qualified as having PH. The groups with and without PH

Discussion

PH is a common accompaniment of IPF and has a significant impact on outcomes.6, 7A possible explanation for the development of PH is that of progressive fibrosis resulting in destruction of the pulmonary vasculature. If the PH of IPF is due to progressive fibrosis, then one might expect to see a relationship between measures of fibrosis and the prevalence and severity of PH. We sought to test the hypothesis that progressive fibrosis is mostly responsible for the PH of IPF by assessing the

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Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD) and is associated with worse outcomes and increased mortality. Evaluation of PH is recommended in lung transplant candidates, but there are currently no standardized screening approaches. Trials have identified therapies that are effective in this setting, providing another rationale to routinely screen patients with ILD for PH.
What screening strategies for identifying PH in patients with ILD are supported by expert consensus?
The study convened a panel of 16 pulmonologists with expertise in PH and ILD, and used a modified Delphi consensus process with three surveys to identify PH screening strategies. Survey 1 consisted primarily of open-ended questions. Surveys 2 and 3 were developed from responses to survey 1 and contained statements about PH screening that panelists rated from −5 (strongly disagree) to 5 (strongly agree).
Panelists reached consensus on several triggers for suspicion of PH including the following: symptoms, clinical signs, findings on chest CT scan or other imaging, abnormalities in pulse oximetry, elevations in brain natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP), and unexplained worsening in pulmonary function tests or 6-min walk distance. Echocardiography and BNP/NT-proBNP were identified as screening tools for PH. Right heart catheterization was deemed essential for confirming PH.
Many patients with ILD may benefit from early evaluation of PH now that an approved therapy is available. Protocols to evaluate patients with ILD often overlap with evaluations for pulmonary hypertension-interstitial lung disease and can be used to assess the risk of PH. Because standardized approaches are lacking, this consensus statement is intended to aid physicians in the identification of patients with ILD and possible PH, and provide guidance for timely right heart catheterization.
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Les patients atteints de PF-ILD avec HTP sévère ont des densités pulmonaires plus basses que les patients avec HTP modérée ou sans HTP.
The main objective of this work was to investigate a possible link between lung density, small pulmonary vessels, and pulmonary hypertension (PH) in patients with progressive fibrosing interstitial lung disease (PF-ILD).
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Fifty-one patients with usual interstitial pneumonia (UIP) damage on lung explant were included. mPAP was positively correlated with lung mass (r = 0.36, P = 0.03) and lung volume (r = 0.43, P = 0.007). Patients with severe PH had more voxels lower than -856 Hounsfield Units (HU) (+16%, P = 0.02), fewer voxels greater than −700 HU (−20%, P = 0.03), and a higher lung volume (+1.57L, P = 0.007) compared to patients without PH. No correlation was found between vascularization and HTP.
Patients with PF-ILD and severe PH have lower lung density than patients with moderate or without PH.
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: This research was not funded, and none of the authors have any potential or actual conflicts of interest.

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Chest

Original Research: Interstitial Lung DiseasePulmonary Hypertension and Pulmonary Function Testing in Idiopathic Pulmonary Fibrosis

Abstract

Section snippets

Materials and Methods

Results

Discussion

Chest

Chest

Chest

Respir Med

Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis

Am J Respir Crit Care Med

Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends

Am J Respir Crit Care Med

Predicting survival in idiopathic pulmonary fibrosis

Am J Respir Crit Care Med

The clinical course of patients with idiopathic pulmonary fibrosis

Ann Intern Med

Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography

Am J Respir Crit Care Med

Original Research: Interstitial Lung Disease
Pulmonary Hypertension and Pulmonary Function Testing in Idiopathic Pulmonary Fibrosis