Arvanitaki Alexandra, Boutsikou Maria, Anthi Anastasia, Apostolopoulou Sotiria, Avgeropoulou Aikaterini, Demerouti Eftychia, Farmakis Dimitrios, Feloukidis Christos, Giannakoulas George, Karvounis Haralambos, Karyofyllis Panagiotis, Mitrouska Ioanna, Mouratoglou Sophia, Naka Katerina K, Orfanos Stylianos E, Panagiotidou Evangelia, Pitsiou Georgia, Rammos Spyridon, Stagaki Eleni, Stanopoulos Ioannis, Thomaidi Adina, Triantafyllidi Helen, Tsangaris Iraklis, Tsiapras Dimitrios, Voudris Vassilios, Manginas Athanasios
Cardiology Department, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Cardiology Department, Mediterraneo Hospital, Athens, Greece.
Pulm Circ. 2019 Oct 14;9(3):2045894019877157. doi: 10.1177/2045894019877157. eCollection 2019 Jul-Sep.
Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. Baseline epidemiologic, diagnostic, and initial treatment data of consecutive patients with PAH are presented in this article. In total, 231 patients with PAH were enrolled from January 2015 until April 2018. At baseline, about half of patients with PAH were in World Health Organization functional class II. The majority of patients with PAH (56.7%) were at intermediate 1-year mortality risk, while more than one-third were low-risk patients, according to an abbreviated risk stratification score. Half of patients with PAH were on monotherapy, 38.9% received combination therapy, while prostanoids were used only in 12.1% of patients. In conclusion, baseline data of the Greek PAH population share common characteristics, but also have some differences with other registries, the most prominent being a better functional capacity. This may reflect earlier diagnosis of PAH that in conjunction with the increased proportion of patients with atypical PAH could partially explain the preference for monotherapy and the limited use of prostanoids in Greece. Nevertheless, early, advanced specific therapy is strongly recommended.
肺动脉高压（PAH）是一种预后较差的异质性临床疾病，尽管近期在药物治疗方面取得了重大进展。为了提高对该疾病病理生理学、流行病学和管理的认识，需要建立大型国家登记系统。希腊肺动脉高压登记系统（HOPE）于2015年初启动，登记希腊所有肺动脉高压亚组的患者。本文介绍了PAH连续患者的基线流行病学、诊断和初始治疗数据。从2015年1月至2018年4月，共登记了231例PAH患者。基线时，约一半的PAH患者处于世界卫生组织功能分级II级。根据简化风险分层评分，大多数PAH患者（56.7%）处于中等1年死亡风险，而超过三分之一是低风险患者。一半的PAH患者接受单药治疗，38.9%接受联合治疗，而仅12.1%的患者使用前列环素。总之，希腊PAH人群的基线数据有共同特征，但与其他登记系统也存在一些差异，最突出的是功能能力较好。这可能反映了PAH的早期诊断，再加上非典型PAH患者比例的增加，可能部分解释了希腊对单药治疗的偏好和前列环素的有限使用。尽管如此，强烈建议尽早进行先进的特异性治疗。
