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The management of CTEPH has evolved due to advances in diagnosis, treatment, and the adoption of best practice guidelines. However, there is limited information regarding contemporary real-world clinical care. The aim of this survey was to collect clinical practice data from hospital-based medical specialists and to identify unmet needs in CTEPH. CLARITY was developed by an independent committee of international CTEPH experts using the Delphi method. Questions were designed to gather quantitative and qualitative insights on the awareness, diagnosis, and treatment of CTEPH. Twenty-one international, regional, and national scientific societies and other medical organizations have supported the web-survey distribution. Responses were collected from 09.10.2021 to 05.01.2022. 416 questionnaires were collected, 353 of which were suitable to be analyzed. Respondents came from Europe (44%), Asia-Pacific (32%), Latin America (11%), US / Canada (11%), and the Middle East / Africa (2%). Most respondents specialized in pulmonology (44%) or cardiology (41%) and had over 15 years of experience (56%). Around 30% were not working in a pulmonary hypertension or CTEPH expert center and among them, only 30% were affiliated to such a center. The most commonly reported barriers to CTEPH recognition and diagnosis, operability assessment, and treatment by pulmonary endarterectomy (PEA) / balloon pulmonary angioplasty (BPA) were lack of disease awareness and structured pulmonary embolism follow-up, delayed referral, lack of standardized operability criteria and multidisciplinary team/reference center, limited access to PEA / BPA, and insufficient expertise in performing PEA / BPA, respectively (Table). The survey provided global insights on the contemporary management of CTEPH and an understanding of key barriers to optimal care. It may serve as a basis to call for action to improve CTEPH detection and management. [ABSTRACT FROM AUTHOR]

To evaluate the effect of a multidisciplinary team in the management of patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary thromboendarterectomy (PTE) (Figure A). From 1997 to 2022, 306 patients underwent PTE for CTEPH. In 2011, a multidisciplinary team was assembled to systematically evaluate, select, and manage patients for PTE based on hemodynamic profile, anatomic scar accessibility, and comorbidities. The cohort was divided into an early era of 62 cases (20%) prior to 2011 and 244 cases (80%) thereafter (recent era). Baseline demographics and hemodynamic profiles were similar between eras, with mean age 53±14 years. More early-era patients were in New York Heart Association class III/IV (64% vs. 40%); however, pulmonary hypertension and pulmonary vascular resistance (PVR) were similar. Recent-era patients had shorter circulatory arrest times (36 vs. 42 min) and more tricuspid valve repairs (22% vs. 3%). Recent-era patients had lower hospital mortality (3% vs. 12%), fewer hospital morbidity, including less prolonged ventilation (32% vs. 59%) and less need for dialysis (1.6% vs. 21%), and shorter length of stay (16 vs. 21 days). Difference in mortality was sustained long-term (12% vs. 30% at 6 years, Figure B). Recent-era patients had lower residual mean pulmonary artery pressures (26 vs. 33 mmHg) and lower residual PVR (2.7 vs. 4.2 Wood units). Establishing a multidisciplinary CTEPH team allows more accurate identification of patients most likely to benefit from PTE surgery, which leads to more complete resolution of pulmonary hypertension and improved survival. A team-based approach for patient selection and perioperative management of these complex patients should be considered standard of care. [ABSTRACT FROM AUTHOR]