Pulmonary Arterial Hypertension

Abstract

Registries of patients with pulmonary arterial hypertension (PAH) have been instrumental in characterizing the presentation and natural history of the disease and provide a basis for prognostication. Since the initial accumulation of data conducted in the 1980s, subsequent registry databases have yielded information about the demographic factors, treatment, and survival of patients and have permitted comparisons between populations in different eras and environments. Inclusion of patients with all subtypes of PAH has also allowed comparisons of these subpopulations. We describe herein the basic methodology by which PAH registries have been conducted, review key insights provided by registries, summarize issues related to interpretation and comparison of the results, and discuss the utility of data to predict survival outcomes. Potential sources of bias, particularly related to the inclusion of incident and/or prevalent patients and missing data, are addressed. A fundamental observation of current registries is that survival in the modern treatment era has improved compared with that observed previously and that outcomes among PAH subpopulations vary substantially. Continuing systematic clinical surveillance of PAH will be important as treatment evolves and as understanding of mechanisms advance. Considerations for future directions of registry studies include enrollment of a broader population of patients with pulmonary hypertension of all clinical types and severity and continued globalization and collaboration of registry databases.

Pulmonary arterial hypertension in Latin America: epidemiological data from local studies

“…Hoeper et al [5], noticed that differences between countries may be explained by population age distribution (older population in Europe and US) and health care systems. However, other factors may play a role such as: referral patterns, PAH awareness, increase patient access to information and widespread use of noninvasive screening tools [6]. As noted by McGoon et al [6], phenotypes may be related to the healthcare environment rather than to different expressions of the disease.…”

“…However, other factors may play a role such as: referral patterns, PAH awareness, increase patient access to information and widespread use of noninvasive screening tools [6]. As noted by McGoon et al [6], phenotypes may be related to the healthcare environment rather than to different expressions of the disease. Similar to the results of international registries, the prevalence of PAH in female patients was higher [2, 6].…”

“…As noted by McGoon et al [6], phenotypes may be related to the healthcare environment rather than to different expressions of the disease. Similar to the results of international registries, the prevalence of PAH in female patients was higher [2, 6]. …”

“…National registries have provided a better understanding of the epidemiology and clinical evolution of the disease [3] as well as valuable information on disease characteristics, demographics and outcomes of patients with PAH [4], allowing the development of risk stratification tools [5]. Two recent reviews [2, 6] have identified 11 PAH registries based in the United States (US), China, France, Scotland, United Kingdom (UK), Spain and a European Union consortium. However, limited information is available on the characteristics of PAH patients outside of North America and Europe.…”

“…Moreover, no Latin American studies were included in these reviews. This is particularly important as researchers have described that there are potential geographical and regional differences which are vital to consider in the design of clinical trials as well as PAH treatment [2, 6, 7]. The aim of this study was to describe the epidemiology of PAH (group 1) in Latin America.…”