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

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.

Treatment and management of chronic thromboembolic pulmonary hypertension (CTEPH): A global cross‐sectional scientific survey (CLARITY)

“…The respondent characteristics of the included 353 responders have been described elsewhere. 35 This paper presents the findings on contemporary clinical practices and challenges in the treatment and management of CTEPH patients.…”

“…The survey used for this research was developed with the support of a Scientific Committee of 11 international CTEPH experts from the regions of Europe, North America, Latin America, and Asia‐Pacific, using a modified Delphi Technique, 34 for which the process has previously been described in detail. 35 The online survey consisted of 110 closed‐ and open‐ended questions and was available in 12 languages (English, French, German, Italian, Spanish, Portuguese, Polish, Russian, Turkish, simplified Chinese, Japanese, and Korean). The survey was tailored to each individual respondent's clinical practice and previous responses through the use of conditions and display logic functions.…”

Veno-arterial extra-corporeal membrane oxygenation as rescue therapy in complicated balloon pulmonary angioplasty

“…Adequate treatment, provided in specialised reference centres, is crucial for improving the unfavourable prognosis of this disease. Treatment options typically include pulmonary artery endarterectomy, balloon pulmonary angioplasty (BPA), and pharmacotherapy [1][2][3].…”

Comorbidities, Treatment and Survival Rates of Chronic Thromboembolic Pulmonary Hypertension in a Regional Centre

“…The onset of CTEPH exhibits considerable variability in age, typically manifesting in adulthood, with an average age of onset reported at 67.5 years, with a slightly higher prevalence in women compared to men [20]. Early detection and precise diagnosis of CTEPH pose varied challenges and may take up to an average of 14 months from the onset of symptoms to diagnosis in expert centres [21,22].…”