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. 2024 Feb 21;14(1):e12330.
doi: 10.1002/pul2.12330. eCollection 2024 Jan.

Recognition, diagnosis, and operability assessment of chronic thromboembolic pulmonary hypertension (CTEPH): A global cross-sectional scientific survey (CLARITY)

Grzegorz Kopeć  1   2 , Paul Forfia  3 , Kohtaro Abe  4 , Amélie Beaudet  5 , Virginie Gressin  6 , Mitja Jevnikar  7 , Catherina Meijer  8 , Yan Zhi Tan  8 , Olga Moiseeva  9 , Karen Sheares  10 , Nika Skoro-Sajer  11 , Mario Terra-Filho  12 , Helen Whitford  13 , Zhenguo Zhai  14 , Gustavo A Heresi  15
Affiliations

Recognition, diagnosis, and operability assessment of chronic thromboembolic pulmonary hypertension (CTEPH): A global cross-sectional scientific survey (CLARITY)

Grzegorz Kopeć et al. Pulm Circ. .

Abstract

Early recognition and diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is crucial for improving prognosis and reducing the disease burden. Established clinical practice guidelines describe interventions for the diagnosis and evaluation of CTEPH, yet limited insight remains into clinical practice variation and barriers to care. The CTEPH global cross-sectional scientific survey (CLARITY) was developed to gather insights into the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the recognition and diagnosis of CTEPH and the referral and evaluation of these patients. The survey was offered to hospital-based medical specialists through Scientific Societies and other medical organizations, from September 2021 to May 2022. Response data from 353 physicians showed that self-reported awareness of CTEPH increased over the past 10 years among 96% of respondents. Clinical practices in acute pulmonary embolism (PE) follow-up and CTEPH diagnosis differed among respondents. While 50% of respondents working in a nonexpert center reported to refer patients to an expert pulmonary hypertension/CTEPH center when CTEPH is suspected, 51% of these physicians did not report referral of patients with a confirmed diagnosis for further evaluation. Up to 50% of respondents involved in the evaluation of referred patients have concluded a different operability status than that indicated by the referring center. This study indicates that early diagnosis and timely treatment of CTEPH is challenged by suboptimal acute PE follow-up and patient referral practices. Nonadherence to guideline recommendations may be impacted by various barriers to care, which were shown to vary by geographical region.

Keywords: chronic thromboembolic pulmonary hypertension; clinical practice; diagnosis; pulmonary embolism; pulmonary hypertension.

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Conflict of interest statement

Grzegorz Kopeć reports fees for lectures and/or consultations from Acceleron, Actelion, AOP Health, Bayer, Ferrer, Janssen, and MSD; Kohtaro Abe reports having received a research grant from Actelion Pharmaceuticals Japan; Amélie Beaudet and Virginie Gressin are employees of Actelion Pharmaceuticals Ltd., a Janssen Pharmaceutical Company of Johnson & Johnson; Karen Sheares reports educational support and honoraria for consultations from Actelion, Janssen; Nika Skoro‐Sajer reports fees for lectures and/or consultations from Actelion, AOP Orphan Pharmaceuticals, Bayer, Cordis, GlaxoSmithKline, Pfizer, Medtronic, and United Therapeutics; Mario Terra‐Filho reports consultation fees from Bayer and Janssen; Gustavo Heresi reports fees for non‐branded, non‐promotional lectures from Bayer and fees for consultations from Bayer and Janssen.

Figures

Figure 1
Figure 1
Survey development process.
Figure 2
Figure 2
Sankey chart for order of diagnostic procedures. Sankey chart illustrating the sequence of diagnostic tools and tests performed by respondents involved in the diagnosis of chronic thromboembolic pulmonary hypertension (n = 299). The order of the first three diagnostic procedures, in sequential order, is shown here. The thickness of the lines indicates higher level of use of a particular diagnostic pathway. CT, computed tomography; CTPA, computed tomography pulmonary angiogram; MRI, magnetic resonance imaging; RHC, right heart catheterization; V/Q, ventilation–perfusion.
See this image and copyright information in PMC

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