A stepwise composite echocardiographic score was validated and then used to predict severe pulmonary hypertension (PH) in patients with interstitial lung disease (ILD), according to a study published in by ERJ Open Research.

Echocardiography measurements, right heart catheterization, and pulmonary function testing from consecutive patients with ILD (n=210) at a single site were used to compile an echocardiographic score according to a validated stepwise method. This score was then used to predict severe PH in a validation cohort (n=61).

The strongest predictors of severe PH were selected for the composite stepwise echocardiographic score. These included right ventricular systolic pressure (RVSP), early pulmonary regurgitation gradient, right atrial area size, tricuspid regurgitation velocity, left ventricle eccentricity index, and right ventricle fractional area change.

Continue Reading

The stepwise echocardiographic score was positive in 54% of the derivation cohort. Of the patients who scored positive, 88% achieved this on the first step as a result of having RVSP greater than 64 mm Hg. Right atrial area assessment resulted in an additional 4.8% of patients reaching the threshold. The echocardiographic score correctly assigned 78% of individuals to the correct PH status, with a 5% false negative rate and a 17% false positive rate.

The study authors wrote, “We have shown that a contingent stepwise echocardiographic score, using both RVSP as well as other PH echocardiographic signs of RV dysfunction, can predict severe PH within a large cohort of [patients with] ILD, with a high sensitivity and specificity.”

They added, “The use of this noninvasive tool should guide onward assessment at PH and ILD centers, and help stratify patients with severe PH-ILD into much needed clinical trials.”

Related Articles


Bax S, Bredy C, Kempny A, et al. A stepwise composite echocardiographic score predicts severe pulmonary hypertension in patients with interstitial lung disease. ERJ Open Res. 2018;4(2).

This article originally appeared on The Cardiology Advisor