Tests that you are already performing could also help diagnose PH in patients with ILD

Consider the following recommendations, which include those provided by the 2018 World Symposium on Pulmonary Hypertension1-6*:


Suspect PH-ILD when patient
presents with:

Signs and symptoms out of
proportion to lung

  • Low DLCO (<30%-40%)
  • Poor 6MWT Results
    • Low 6MWD
    • Excessive desaturation or dyspnea
    • Impaired HR recovery

Signs of right ventricular strain or failure2,4,5

  • Elevated BNP or NT-proBNP
  • PA:A ratio >0.9 (CT)


Support your suspicion of PH-ILD with:


  • Elevated RVSP (>45-50 mm Hg)
  • Signs of right ventricular dysfunction


Confirm PH-ILD with:

Right heart catheterization2

Hemodynamic definition6

  • mPAP ≥25 mm Hg
  • PAWP ≤15 mm Hg
  • PVR >3 WU

PH is generally associated with an elevated BNP or NT-proBNP, a lower DLCO, and diminished exercise capacity.2 

Start regular screening of patients with ILD to identify mild to moderate PH-ILD before it can impact prognosis.

View the efficacy data for TYVASO® to see how it can help your patients with PH-ILD.

*United Therapeutics does not provide medical advice.

The 2018 World Symposium on Pulmonary Hypertension proposed a cutoff for mPAP of >20 mm Hg.6


6MWD=6-minute walk distance; BNP=B-type natriuretic peptide; CT=computed tomography; DLCO=diffusing capacity of the lung for carbon monoxide; ECG=electrocardiogram; HR=heart rate; ILD=interstitial lung disease; mPAP=mean pulmonary arterial pressure; NT-proBNP=N-terminal pro−B-type natriuretic peptide; PA:A ratio=pulmonary artery to aorta ratio; PAWP=pulmonary arterial wedge pressure; PH=pulmonary hypertension; PH-ILD=pulmonary hypertension associated with interstitial lung disease; PVR=pulmonary vascular resistance; RVH=right ventricular hypertrophy; RVSP=right ventricular systolic pressure; WU=Wood units.