Open AccessEpidemiology and initial management of pulmonary arterial hypertension: real‐world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE)
Author(s) -
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
Publication year - 2019
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1177/2045894019877157
Subject(s) - medicine , epidemiology , pulmonary hypertension , chronic thromboembolic pulmonary hypertension , disease , intensive care medicine , pediatrics
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.