Characteristics and survival data from Latvian pulmonary hypertension registry: comparison of prospective pulmonary hypertension registries in Europe

Abstract

Patient registries are a valuable tool in the research of rare conditions such as pulmonary hypertension (PH). We report comprehensive hemodynamic and survival data of 174 patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), included in the prospective Latvian PH registry over a period of > 9 years. In total, 130 adult PAH patients (75%) and 44 adult CTEPH patients (25%) were enrolled. The median follow-up period was 33 months for PAH and 18 months for CTEPH, P = 0.001. Latvian CTEPH patients had significantly higher plasma levels of B-type natriuretic peptide, higher pulmonary vascular resistance, and lower cardiac index than Latvian PAH patients. Calculated incidence of PAH and CTEPH in Latvia in 2016 was 13.7 and 5.1 cases per million inhabitants, calculated prevalence was 45.7 and 15.7 cases per million inhabitants, respectively. Survival rates at one, three, and five years for PAH patients was 88.0%, 73.3%, and 58.1%, and 83.8%, 59.0%, and 44.2% for CTEPH patients, respectively. We compared our study results with data from European adult PH registries. Latvian PAH patients had the fourth lowest and CTEPH patients the lowest one-year survival rate among European adult PH registries. As most PH registries in Europe are small, yet with equivalent patient inclusion criteria, it would be desirable to combine these registries to produce more reliable and high-quality study results.

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

“…The 6-MWD distance was slightly better compared with other registries, while hemodynamics (lower mPAP and PVR, higher SVO 2 %) were slightly more favorable in our population. 8,10,11,13–15,18,19 Similar to other registries, the majority of the PAH population was at intermediate 1-year mortality risk, according to the modified PAH risk assessment score. 13,16,17 This observation implies that PAH patients in our cohort may have underestimated their symptoms and their FC, quite common in the PAH-CHD cohort, 25 which could have led the physician to opt for monotherapy.…”

“…Despite major advances in pharmacotherapy of PAH in the last 15 years, 6 we still lack thorough knowledge about the pathophysiology and the epidemiology of PAH. Although large national and international registries 7–15 have so far provided useful information on the epidemiology, the diagnosis, and management of patients with PAH, regional differences certainly exist regarding patients' characteristics, diagnostic workup, and treatment availability. Moreover, since the registries include a mix of PAH patients with advanced age and various comorbidities, there is a scientific need for more epidemiological data from different countries, with different healthcare organization and financial background, in order to obtain a more comprehensive view of PAH worldwide.…”

Characterization of Patients with Pulmonary Arterial Hypertension: Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

“…Thirdly, we are the first to show the multicenter, prospectively collected data on PAH epidemiology in a Central-Eastern European country with a significantly different political and economic background than Western World countries. Previous reports from this geographical region presented retrospectively collected [40] or single center data [41].…”

Pulmonary Endarterectomy in Latvia: A National Experience

“…The calculated incidence of CTEPH in Latvia in 2016 was 5.1 cases per million inhabitants, whereas the prevalence was 15.7 cases per million inhabitants, and it is one of the highest in Europe [12]. The awareness of this disease has markedly increased due to successful informative campaigning for Latvian healthcare professionals.…”