Epidemiology and initial management of pulmonary arterial hypertension: real‐world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE)

Abstract

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.

Pulmonary hypertension in Saudi Arabia: First data from the SAUDIPH registry with a focus on pulmonary arterial hypertension

“…We found a female predominance in the registry, specifically in PAH patients, as documented in the literature 1 and other registries. 22,23 Age at diagnosis was substantially lower than that reported in international registries, [7][8][9]12,16,19,[22][23][24] mainly influenced by lower age in PAH (median = 30.5 years) in Saudi Arabia. 14,15,25 This can be explained by the young age of the population of Saudi Arabia (median age < 30 years) and by the high prevalence of CHD in this study.…”

“…Some previous studies also included prevalent cases but were conducted earlier (closing enrolment in 2008). [8][9][10]17,26 More recent studies show comparable disease severity at diagnosis, 12,13,18,20,22,27 reflecting the impact of better referral networks established over the years. In the last follow-up visit, patients were more likely to be in WHO FC I/II, indicating a reduction in disease severity.…”

Anticoagulation in pulmonary arterial hypertension: a decision analysis

“…1,7 As a result, considerable uncertainty exists on the benefits of offering systemic AC to all IPAH patients without a secondary indication for AC, and recent registry data indicate that only the minority of IPAH patients are placed on systemic AC. 10–14 Additionally, the benefits of systemic AC on PAH quality of life are not well defined, and recommendations for PAH therapy do not currently account for patient values and preferences as part of a shared decision-making model. 15–17…”

Pulmonary hypertension: From an orphan disease to a global epidemic

“…Since the first NIH pulmonary hypertension registry in the USA, more than twenty registries all over the world were planned 47,48 . There were local, hospital-specific, or larger national registries from France 71 , Scotland 72 , United Kingdom 57 , Australia 73 , Spain 74 , China 75 , Germany and other European centres 76,77 , Japan 78 , Korea 79 , Czech Republic 80 , Brazil 81 , Switzerland 82 , Holland 83,84 , Portugal 85 , Greece 6 , Algeria 86 , Saudi Arabia 87,88 , Israel 89 , India 90 and the USA 91–93 .…”