TABLE 1

Findings suggestive for interstitial lung disease associated pulmonary hypertension

ClinicalBimalleolar oedema
Jugular venous distension
Signs of RV dysfunction
Pulmonary function testsDisproportionately severe decrease in diffusing capacity of the lung, while lung volumes are normal or only modestly reduced
6-min walk testLower than expected 6-min walk distance
Marked exertional desaturation
Laboratory testingElevated (NT-pro)BNP
Research: heart-type fatty acid binding protein, growth differentiation factor-15
Chest imagingIncreased pulmonary artery to ascending aorta ratio (>0.9)
Main pulmonary artery diameter >29 mm
RV enlargement
EchocardiographyPeak tricuspid regurgitation velocity ≥2.8 m·s−1
RV/LV basal diameter ratio >1.0
Flattening of the interventricular septum
RV outflow Doppler acceleration time <105 ms and/or midsystolic notching
Pulmonary artery diameter >25 mm
Inferior vena cava diameter >21 mm with decreased inspiratory collapse
Right atrial area >18 cm2

RV: right ventricle; NT-proBNP: N-terminal pro-brain natriuretic peptide; LV: left ventricle.