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Abstract：
Advances in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) over the past decade changed the disease landscape, yet global insight on clinical practices remains limited. The CTEPH global cross-sectional scientific survey (CLARITY) aimed to gather information on the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the treatment and management of CTEPH patients. The survey was circulated to hospital-based medical specialists through Scientific Societies and other medical organizations from September 2021 to May 2022. The majority of the 212 respondents involved in the treatment of CTEPH were from centers performing up to 50 pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA) procedures per year. Variation was observed in the reported proportion of patients deemed eligible for PEA/BPA, as well as those that underwent the procedures, including multimodal treatment and subsequent follow-up practices. Prescription of pulmonary arterial hypertension-specific therapy was reported for a variable proportion of patients in the preoperative setting and in most nonoperable patients. Reported use of vitamin K antagonists and direct oral anticoagulants was similar (86% vs. 82%) but driven by different factors. This study presents heterogeneity in treatment approaches for CTEPH, which may be attributed to center-specific experience and region-specific barriers to care, highlighting the need for new clinical and cohort studies, comprehensive clinical guidelines, and continued education.
摘要：
在过去的十年中，慢性血栓栓塞性肺动脉高压（CTEPH）的治疗进展改变了疾病的格局，然而，全球对临床实践的洞察力仍然有限。CTEPH全球横断面科学调查（CLARITY）旨在收集有关当前诊断的信息，治疗,和CTEPH的管理，并确定未满足的医疗需求。本文重点介绍CTEPH患者的治疗和管理。该调查于2021年9月至2022年5月通过科学协会和其他医疗组织分发给医院医学专家。参与CTEPH治疗的212名受访者中，大多数来自每年执行多达50例肺动脉内膜切除术（PEA）和/或球囊肺血管成形术（BPA）手术的中心。在被认为符合PEA/BPA的患者的报告比例中观察到差异，以及那些接受手术的人，包括多模式治疗和随后的后续实践。据报道，在术前设置和大多数不可手术的患者中，不同比例的患者使用了肺动脉高压特异性治疗的处方。维生素K拮抗剂和直接口服抗凝剂的报告使用情况相似(86%vs.82%)，但受不同因素驱动。这项研究提出了CTEPH治疗方法的异质性，这可能归因于特定中心的经验和特定地区的护理障碍，强调需要新的临床和队列研究，综合临床指南，和继续教育。
