ArticleERJ open research2018
A stepwise composite echocardiographic score predicts severe pulmonary hypertension in patients with interstitial lung disease.
Article in ERJ open research, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 21 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
21 citing papers in PubMed.
- Navigating the Complexity of PH-ILD: From Molecular Mechanisms to Integrated Clinical Evaluation.International journal of molecular sciences · 2026Review
- Pulmonary hypertension associated with interstitial lung disease: a state-of-the-art review.The European respiratory journal · 2026Review
- Screening for Pulmonary Hypertension in Interstitial Lung Disease: Preliminary Results from the PHINDER Study.Advances in therapy · 2026Observational
- Deep-learning networks accurately detect pulmonary hypertension in patients with idiopathic pulmonary fibrosis.ERJ open research · 2026Article
- Review
- Comorbidities' Effect on IPF: Pathogenesis and Management.Biomedicines · 2025Review
- Review of the Diagnosis and Management of Pulmonary Hypertension Associated with Interstitial Lung Disease (ILD-PH).Journal of clinical medicine · 2025Review
- Article
- Screening and diagnosis of pulmonary hypertension associated with chronic lung disease (PH-CLD): A consensus statement from the pulmonary vascular research institute's innovative drug development initiative-group 3 pulmonary hypertension.Pulmonary circulation · 2024Review
- Pulmonary hypertension associated with lung diseases.The European respiratory journal · 2024Review
- Noninvasive diagnostic modalities and prediction models for detecting pulmonary hypertension associated with interstitial lung disease: a narrative review.European respiratory review : an official journal of the European Respiratory Society · 2024Review
- Towards a Better Prognosis in Patients with Systemic Sclerosis-Related Pulmonary Arterial Hypertension: Recent Developments and Perspectives.Journal of clinical medicine · 2024Review
- Sarcoidosis-associated pulmonary hypertension due to pulmonary arteries stenosis - a case report.BMC pulmonary medicine · 2024Article
- Phenotypes of Sarcoidosis-Associated Pulmonary Hypertension-A Challenging Mystery.Diagnostics (Basel, Switzerland) · 2023Review
- Pulmonary Hypertension in the Course of Interstitial Lung Diseases-A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy.Diagnostics (Basel, Switzerland) · 2023Review
- Review
- Non-invasive detection of severe PH in lung disease using magnetic resonance imaging.Frontiers in cardiovascular medicine · 2023Article
- Pulmonary hypertension in interstitial lung disease: an area of unmet clinical need.ERJ open research · 2022Review
- Article
- Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis.BMC pulmonary medicine · 2019Article
Corrections and comments
- Erratum issuedErratum:2018
Authors and funding
18 authors.
Funding
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%
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.