Abstract
European Respiratory Society (ERS) guidelines recommend the assessment of patients with interstitial lung disease (ILD) and severe pulmonary hypertension (PH), as defined by a mean pulmonary artery pressure (mPAP) ≥35 mmHg at right heart catheterisation (RHC). We developed and validated a stepwise echocardiographic score to detect severe PH using the tricuspid regurgitant velocity and right atrial pressure (right ventricular systolic pressure (RVSP)) and additional echocardiographic signs.
Consecutive ILD patients with suspected PH underwent RHC between 2005 and 2015. Receiver operating curve analysis tested the ability of components of the score to predict mPAP ≥35 mmHg, and a score devised using a stepwise approach. The score was tested in a contemporaneous validation cohort. The score used “additional PH signs” where RVSP was unavailable, using a bootstrapping technique.
Within the derivation cohort (n=210), a score ≥7 predicted severe PH with 89% sensitivity, 71% specificity, positive predictive value 68% and negative predictive value 90%, with similar performance in the validation cohort (n=61) (area under the curve (AUC) 84.8% versus 83.1%, p=0.8). Although RVSP could be estimated in 92% of studies, reducing this to 60% maintained a fair accuracy (AUC 74.4%).
This simple stepwise echocardiographic PH score can predict severe PH in patients with ILD.
Abstract
A stepwise echocardiographic score to predict severe group 3 pulmonary hypertension http://ow.ly/9cIC30iGSMj
Footnotes
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Conflict of interest: A. Kempny reports receiving grants from Actelion Global, outside the submitted work.
Conflict of interest: K. Dimopoulos reports receiving grants from Actelion, GSK, Pfizer and Bayer, outside the submitted work.
Conflict of interest: J. Jacob reports receiving personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: A. Wells reports receiving personal fees from Intermune, Boehringer Inlgeheim, Gilead, MSD, Roche, Bayer and Chiesi, outside the submitted work.
Conflict of interest: S.J. Wort reports receiving grants from Actelion, GSK, Pfizer and Bayer, outside the submitted work.
Conflict of interest: L.C. Price reports educational grants from Actelion and GSK, during the conduct of the study.
Conflict of interest: Dr. George reports personal fees from Roche, personal fees from Boeringer Ingelheim, outside the submitted work.
Support statement: J. Jacob was supported by a Wellcome Trust Clinical Research Career Development Fellowship (209553/Z/17/Z). Funding information for this article has been deposited with the Crossref Funder Registry.
- Received October 3, 2017.
- Accepted February 16, 2018.
- Copyright ©ERS 2018
This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.