Screening Strategies for Pulmonary Hypertension in Patients With Interstitial Lung Disease

Abstract

BackgroundPulmonary 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.Research QuestionWhat screening strategies for identifying PH in patients with ILD are supported by expert consensus?Study Design and MethodsThe 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).ResultsPanelists 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.InterpretationMany 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. 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. Take-home PointsStudy Question: What screening strategies for identifying pulmonary hypertension (PH) in patients with interstitial lung disease (ILD) are supported by expert consensus?Results: Panelists reached consensus on several triggers for suspicion of PH based on certain signs, symptoms, and other findings in usual tests performed in the care of patients’ with ILD (eg, pulmonary function tests, CT scans).Interpretation: Many patients with ILD may benefit from early evaluation of PH now that an approved therapy is available. This consensus statement may help physicians to identify PH in patients with ILD. Study Question: What screening strategies for identifying pulmonary hypertension (PH) in patients with interstitial lung disease (ILD) are supported by expert consensus? Results: Panelists reached consensus on several triggers for suspicion of PH based on certain signs, symptoms, and other findings in usual tests performed in the care of patients’ with ILD (eg, pulmonary function tests, CT scans). Interpretation: Many patients with ILD may benefit from early evaluation of PH now that an approved therapy is available. This consensus statement may help physicians to identify PH in patients with ILD. Pulmonary vascular involvement by numerous etiologies can result in pulmonary hypertension (PH), which is defined by a mean pulmonary artery pressure of > 20 mm Hg.1Simonneau G. Montani D. Celermajer D.S. et al.Haemodynamic definitions and updated clinical classification of pulmonary hypertension.Eur Respir J. 2019; 531801913Crossref Scopus (2281) Google Scholar The interstitial lung diseases (ILDs) are a broad, heterogenous group of conditions with > 200 etiologies, which are always accompanied by variable amounts of inflammation and/or fibrosis. PH is a common complication of ILD and is classified as group 3 in the World Symposium on Pulmonary Hypertension classification.1Simonneau G. Montani D. Celermajer D.S. et al.Haemodynamic definitions and updated clinical classification of pulmonary hypertension.Eur Respir J. 2019; 531801913Crossref Scopus (2281) Google Scholar PH associated with ILD is associated with worse outcomes, including an approximately threefold increased risk of mortality, a heightened propensity for acute exacerbations, impaired quality of life, decreased exercise capability, and increased need for supplemental oxygen.2DuBrock H.M. Nathan S.D. 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Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis.Chest. 2007; 131: 657-663Abstract Full Text Full Text PDF PubMed Scopus (220) Google Scholar, 18Shorr A.F. Wainright J.L. Cors C.S. Lettieri C.J. Nathan S.D. Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung Respir J. 2007; PubMed Scopus Google Scholar, M. Kawut S.M. and of in idiopathic pulmonary fibrosis.Chest. 2013; Full Text Full Text PDF PubMed Scopus Google Scholar, J.M. conditions in idiopathic pulmonary and Med PubMed Scopus Google Scholar, S.D. Shlobin O.A. Barnett S.D. et pressure by as a of pulmonary hypertension in idiopathic pulmonary fibrosis.Respir Med. Full Text Full Text PDF PubMed Scopus Google Scholar is an screening for PH, has in the right pressure in the of ILD and is used for the risk of PH for S.D. Shlobin O.A. Barnett S.D. et pressure by as a of pulmonary hypertension in idiopathic pulmonary fibrosis.Respir Med. Full Text Full Text PDF PubMed Scopus Google M. et al.Pulmonary hypertension in interstitial lung disease: of to PubMed Scopus Google Scholar of PH right heart catheterization to In to confirming the diagnosis, and PH providing other prognostic factors (eg, right G. Montani D. Celermajer D.S. et al.Haemodynamic definitions and updated clinical classification of pulmonary hypertension.Eur Respir J. 2019; 531801913Crossref Scopus (2281) Google Scholar evaluation of PH in patients with ILD was performed primarily as of the for lung and to evaluate the D. et consensus for the of lung transplant from the Pulmonary of the for and 2015; Full Text Full Text PDF PubMed Scopus Google Scholar In the of an effective therapy for pulmonary hypertension-interstitial lung disease the and of screening and have to the in the of lung transplant J. Nathan S.D. 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