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Screening & Diagnosis for Pulmonary Hypertension in ILD Patients

PH-ILD Screening

Early PH evaluation may improve ILD patient outcomes, as an approved therapy is available.

A modified Delphi study was conducted to determine screening strategies for pulmonary hypertension in patients with interstitial lung disease using expert consensus.

Conclusion

Routine ILD clinical evaluations can trigger suspicion of PH, allowing for evaluation of additional tests including echocardiography and PH confirmation with a RHC

Pulmonary Hypertension Diagnostic Criteria for ILD Patients

1 Triggers for PH Screening in Routine ILD Evaluations

Panelists reached consensus on several triggers raising the suspicion for PH including:

  • Symptoms
  • Clinical signs
  • Chest CT scan findings or other imaging
  • Pulse oximetry abnormalities
  • 6MWT worsening unexplained in pulmonary function tests

2 Initial Set of PH Screening

Panelist consensus: When PH is suspected, echocardiogram and BNP or NT-proBNP are useful as subsequent screening tests

  • Echocardiogram
  • BNP or NT-proBNP elevation brain natriuretic peptide or N-terminal pro-brain natriuretic peptide

3 Confirm a PH Diagnosis

Right heart catheterization

Clinical Process and Criteria for PH Diagnosis

Risk factors/symptoms

  • History of pulmonary embolism or heart failure
  • Dizziness, palpitations, syncope

Signs

  • Altered heart sounds (loud P2 or S2)
  • Signs of right heart failure
  • Jugular venous distention
  • Ankle swelling/peripheral edema
  • Hepatomegaly/ascites

Diagnostic criteria used in concert to risk stratify for pulmonary hypertension

PFTs

  • DLCO decline > 15%
  • DLCO < 40% predicted
  • Worsening FVC/ DLCO
  • FVC%/ DLCO% > 1.6

Oxygen saturation & 6MWT

  • Any supplemental oxygen needs
  • Desaturation disproportionate to ILD severity
  • Worsening desaturation
  • Lower distance on 6MWT

CT scan

  • RV enlargement
  • PA enlargement
  • PA/aorta ratio > 1.0

BNP/NT-proBNP

  • Elevated BNP (> 200 pg/mL)
  • Elevated NT-proBNP (> 395 pg/mL)

Individual laboratories may have different thresholds

Testing that is routinely obtained in patients with ILD
Not routinely obtained, but considered if there is suspicion for underlying heart failure or PH in patients with ILD
Clinical suspicion of PH should be followed up with an echo to determine if a RHC is needed. High PH probability of PH with echo or intermediate PH probability by echo with high clinical suspicion should lead to a RHC. Consider a RHC for non-low risk scenarios.

Delphi Panel of Experts

  • Illustration of Doctor Franck Rahaghi, MD. - Cleveland Clinic Florida - Weston, FL
    Franck Rahaghi, MD Cleveland Clinic Florida Weston, FL
  • Illustration of Doctor Nicholas Kolaitis, MD. - UCSF Health - San Francisco, CA
    Nicholas Kolaitis, MD UCSF Health San Francisco, CA
  • Illustration of Doctor Ayodeji Adegunsoye, MD. - The University of Chicago School of Medicine - Chicago, IL
    Ayodeji Adegunsoye, MD The University of Chicago School of Medicine Chicago, IL
  • Illustration of Doctor Joao de Andrade, MD. - Vanderbilt University Medical Center - Nashville, TN
    Joao de Andrade, MD Vanderbilt University Medical Center Nashville, TN
  • Illustration of Doctor Kevin Flaherty, MD. - University of Michigan Health System - Ann Arbor, MI
    Kevin Flaherty, MD University of Michigan Health System Ann Arbor, MI
  • Illustration of Doctor Lisa Lancaster, MD. - Vanderbilt University Medical Center - Nashville, TN
    Lisa Lancaster, MD Vanderbilt University Medical Center Nashville, TN
  • Illustration of Doctor Joyce Lee, MD. - University of Colorado School of Med - Aurora, CO
    Joyce Lee, MD University of Colorado School of Medicine Aurora, CO
  • Illustration of Doctor Deborah Levine, MD. - Stanford University - Stanford, CA
    Deborah Levine, MD Stanford University Stanford, CA
  • Illustration of Doctor Ioana Preston, MD. - Tufts Medical Center - Boston, MA
    Ioana Preston, MD Tufts Medical Center Boston, MA
  • Illustration of Doctor Zeenat Safdar, MD. - Houston Methodist - Houston, TX
    Zeenat Safdar, MD Houston Methodist Houston, TX
  • Illustration of Doctor Rajan Saggar, MD. - UCLA School of Medicine - Los Angeles, CA
    Rajan Saggar, MD UCLA School of Medicine Los Angeles, CA
  • Illustration of Doctor Sandeep Sahay, MD. - Houston Methodist - Houston, TX
    Sandeep Sahay, MD Houston Methodist Houston, TX
  • Illustration of Doctor Mary Beth Scholand, MD. - University of Utah Health - Salt Lake City, UT
    Mary Beth Scholand, MD University of Utah Health Salt Lake City, UT
  • Illustration of Doctor Oksana Shlobin, MD. - Inova Fairfax Hospital - Falls Church, VA
    Oksana Shlobin, MD Inova Fairfax Hospital Falls Church, VA
  • Illustration of Doctor David Zisman, MD. - Sansum Clinic - Santa Barbara, CA
    David Zisman, MD Sansum Clinic Santa Barbara, CA
  • Illustration of Doctor Steven Nathan, MD. - Inova Fairfax Hospital - Falls Church, VA
    Steven Nathan, MD Inova Fairfax Hospital Falls Church, VA
The expert panel included 16 pulmonologists practicing in the U.S. who had a median of 15 to 19 years of experience treating ILD and had treated a median of 1,000 to 1,999 patients each.

Practice settings by the numbers*

  • ILD Centers
    12
  • PH Centers
    8
  • General Pulmonology
    1
  • PH  &  ILD Centers
    2

* Some panelists practice in multiple settings

Method & panel selection

The modified Delphi method is a systematic approach of obtaining consensus opinions from a panel of independent experts. A PH-ILD working group convened by United Therapeutics constituted most of the Delphi panel. The remaining panelists were nominated by working group members, reviewed, and invited based on the number of nominations and diversity to ensure the Delphi panel was composed of experts from varied backgrounds and practices.

Modified Delphi Methodology

3 Surveys

Developed and moderated by 3 expert panelists

Consensus

Likert scale mean score ≥ 2.5 with standard deviation not crossing 0

5-POINT LIKERT SCALE

Consensus scoring was determined using the Likert scale, which ranges from strongly disagree (-5) to strongly agree (5). Zero is a neutral score.

Survey Contents

Survey 1

Open questions on PH in ILD screening

  • Who would benefit from early diagnosis and treatment?
  • Test and imaging results
  • Physical signs and symptoms
  • Role of comorbidities and overall approach to screening
Survey 2

Statements regarding screening for PH in ILD

  • Panelists rated agreement with statements on the above Likert scale
  • Panelists could expand on responses in open-response questions
Survey 3

Review of the results of Survey 2

  • Panelists could reevaluate their answers with the group’s response
  • Intended to build consensus