Skoro-Sajer Nika, Sheares Karen, Forfia Paul, Heresi Gustavo A, Jevnikar Mitja, Kopeć Grzegorz, Moiseeva Olga, Terra-Filho Mario, Whitford Helen, Zhai Zhenguo, Beaudet Amélie, Gressin Virginie, Meijer Catherina, Tan Yan Zhi, Abe Kohtaro
Division of Cardiology, Department of Internal Medicine II Medical University of Vienna Vienna Austria.
Royal Papworth Hospital Cambridge UK.
Pulm Circ. 2024 Jun 30;14(2):e12406. doi: 10.1002/pul2.12406. eCollection 2024 Apr.
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.
在过去十年中,慢性血栓栓塞性肺动脉高压(CTEPH)的治疗进展改变了该疾病的格局,但全球对临床实践的了解仍然有限。CTEPH全球横断面科学调查(CLARITY)旨在收集有关CTEPH当前诊断、治疗和管理的信息,并确定未满足的医疗需求。本文重点关注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学组织分发给医院的医学专家。参与CTEPH治疗的212名受访者中,大多数来自每年进行多达50例肺动脉内膜剥脱术(PEA)和/或球囊肺动脉成形术(BPA)的中心。在报告的被认为有资格接受PEA/BPA治疗的患者比例以及接受这些手术的患者比例方面存在差异,包括多模式治疗和后续随访实践。在术前和大多数无法手术的患者中,不同比例的患者报告了使用肺动脉高压特异性治疗。报告的维生素K拮抗剂和直接口服抗凝剂的使用情况相似(86%对82%),但驱动因素不同。本研究表明CTEPH的治疗方法存在异质性,这可能归因于特定中心的经验和特定地区的护理障碍,突出了开展新的临床和队列研究、制定全面临床指南以及持续教育的必要性。