Screening Strategies for Pulmonary Hypertension in Patients With Interstitial Lung Disease: A Multidisciplinary Delphi Study

Franck F Rahaghi; Nicholas A Kolaitis; Ayodeji Adegunsoye; Joao A de Andrade; Kevin R Flaherty; Lisa H Lancaster; Joyce S Lee; Deborah J Levine; Ioana R Preston; Zeenat Safdar; Rajan Saggar; Sandeep Sahay; Mary Beth Scholand; Oksana A Shlobin; David A Zisman; Steven D Nathan

doi:10.1016/j.chest.2022.02.012

Screening Strategies for Pulmonary Hypertension in Patients With Interstitial Lung Disease: A Multidisciplinary Delphi Study

Chest. 2022 Jul;162(1):145-155. doi: 10.1016/j.chest.2022.02.012. Epub 2022 Feb 15.

Authors

Franck F Rahaghi¹, Nicholas A Kolaitis², Ayodeji Adegunsoye³, Joao A de Andrade⁴, Kevin R Flaherty⁵, Lisa H Lancaster⁶, Joyce S Lee⁷, Deborah J Levine⁸, Ioana R Preston⁹, Zeenat Safdar¹⁰, Rajan Saggar¹¹, Sandeep Sahay¹², Mary Beth Scholand¹³, Oksana A Shlobin¹⁴, David A Zisman¹⁵, Steven D Nathan¹⁶

Affiliations

¹ Advanced Lung Disease Clinic, Cleveland Clinic Florida, Weston, FL.
² UCSF Pulmonary and Critical Care, San Francisco, CA.
³ Section of Pulmonary & Critical Care, The University of Chicago School of Medicine, Chicago, IL.
⁴ Vanderbilt Lung Institute, Vanderbilt University Medical Center, Nashville, TN.
⁵ Michigan Medicine Interstitial Lung Disease Program, University of Michigan, Ann Arbor, MI.
⁶ Vanderbilt University Medical Center, Nashville, TN.
⁷ Pulmonary Sciences & Critical Care, University of Colorado School of Medicine, Aurora, CO.
⁸ Pulmonary Hypertension Center, UT Health San Antonio, San Antonio, TX.
⁹ Pulmonary Hypertension Center, Tufts Medical Center, Boston, MA.
¹⁰ Houston Methodist, Houston, TX.
¹¹ Pulmonary and Critical Care Medicine, David Geffen School of Medicine, UCLA, Los Angeles, CA.
¹² Houston Methodist Lung Center, Houston, TX.
¹³ Pulmonary Medicine, University of Utah Health, Farmington, UT.
¹⁴ Inova Fairfax Heart & Lung Transplant Program, Inova Medical Group, Falls Church, VA.
¹⁵ Sansum Clinic Santa Barbara, Santa Barbara, CA.
¹⁶ Advanced Lung Disease Program, Lung Transplant Program, Inova Fairfax Hospital, Falls Church, VA. Electronic address: steven.nathan@inova.org.

Abstract

Background: 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.

Research question: What screening strategies for identifying PH in patients with ILD are supported by expert consensus?

Study design and methods: 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).

Results: 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.

Interpretation: 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.

Keywords: echocardiography; idiopathic pulmonary fibrosis; interstitial lung disease; pulmonary hypertension; right heart catheterization; screening.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Delphi Technique
Echocardiography
Humans
Hypertension, Pulmonary* / diagnosis
Hypertension, Pulmonary* / etiology
Lung Diseases, Interstitial* / complications
Lung Diseases, Interstitial* / diagnosis
Respiratory Function Tests / adverse effects

Grants and funding

K23 HL146942/HL/NHLBI NIH HHS/United States