Non-invasive early exclusion of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: The InShape II study

Gudula J. A. M. Boon, Yvonne M. Ende-Verhaar, Roisin Bavalia, Lahassan H. el Bouazzaoui, Marion Delcroix, Olga Dzikowska-Diduch, Menno V. Huisman, Katarzyna Kurnicka, Albert T. A. Mairuhu, Saskia Middeldorp, Piotr Pruszczyk, Dieuwertje Ruigrok, Peter Verhamme, Hubert W. Vliegen, Anton Vonk Noordegraaf, Joris W. J. Vriend, Frederikus A. Klok*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Background The current diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism (PE) is unacceptably long, causing loss of quality-adjusted life years and excess mortality. Validated screening strategies for early CTEPH diagnosis are lacking. Echocardiographic screening among all PE survivors is associated with overdiagnosis and cost-ineffectiveness. We aimed to validate a simple screening strategy for excluding CTEPH early after acute PE, limiting the number of performed echocardiograms. Methods In this prospective, international, multicentre management study, consecutive patients were managed according to a screening algorithm starting 3 months after acute PE to determine whether echocardiographic evaluation of pulmonary hypertension (PH) was indicated. If the 'TEPH prediction score' indicated high pretest probability or matching symptoms were present, the 'TEPH rule-out criteria' were applied, consisting of ECG reading and N-terminalpro-brain natriuretic peptide. Only if these results could not rule out possible PH, the patients were referred for echocardiography. Results 424 patients were included. Based on the algorithm, CTEPH was considered absent in 343 (81%) patients, leaving 81 patients (19%) referred for echocardiography. During 2-year follow-up, one patient in whom echocardiography was deemed unnecessary by the algorithm was diagnosed with CTEPH, reflecting an algorithm failure rate of 0.29% (95% CI 0% to 1.6%). Overall CTEPH incidence was 3.1% (13/424), of whom 10 patients were diagnosed within 4 months after the PE presentation. Conclusions The InShape II algorithm accurately excluded CTEPH, without the need for echocardiography in the overall majority of patients. CTEPH was identified early after acute PE, resulting in a substantially shorter diagnostic delay than in current practice.

Original languageEnglish
Pages (from-to)1002-1009
Number of pages8
Issue number10
Early online date2021
Publication statusPublished - 1 Oct 2021

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