Table 2.

PH enrichment criteria

Category 1: PFT and HRCT	Category 2: signs and symptoms	Category 3: echocardiography
DLCO < 40% DLCO decline ≥ 15% on two most recent assessments DLCO decline > 10% and FVC decline < 5% on two most recent assessments PA enlargementa PA to Aorta ratio > 1 RV to LV ratio > 1 Ventricular septal flattening Enlarged peripheral PAsb	Symptoms disproportionate to ILD severity or progression, in the opinion of the investigator Desaturation during 6MWT disproportionate to ILD severity, in the opinion of the investigator Worsening desaturation BNP > 200 pg/ml or NT-proBNP > 395 pg/ml ≥ 1 of the following on physical exam: syncope, JVD, loud S2 heart sound, peripheral edema, ascites, or hepatomegaly	TAPSE < 2 cm RSVP > 35 mmHg Any dilation, enlargement, or other abnormality of the RV

6MWT six-minute walk test, BNP B-type natriuretic peptide, DLCO diffusing capacity of the lungs for carbon monoxide, FVC forced vital capacity, HRCT high-resolution computed tomography, ILD interstitial lung disease, JVD jugular vein distention, LV left ventricle, NT-proBNP N-terminal pro B-type natriuretic peptide, PA pulmonary artery, PFT pulmonary function test, RV right ventricle, RVSP right ventricular systolic pressure, TAPSE tricuspid annular plane systolic excursion

^aDetermination of PA enlargement is based on a composite evaluation of central pulmonary arteries integrating trunk diameter > 30–32 mm, PA/Aorta ratio > 1, and subjective enlargement of the main and interlobular arteries

^bPeripheral PA enlargement is based on non-tapering vessels, vessels extending to pleural surface, and acute peripheral arterial dilation after visually excluding any areas with fibrosis