Epidemiology, disease burden of pulmonary arterial hypertension in China: A systematic literature review

Abstract

Introduction

Although pulmonary arterial hypertension (PAH) may impose a substantial burden in China, the epidemiology of PAH remains unclear. This systematic literature review (SLR) aims to synthesize the literature on PAH epidemiology and disease burden in China.

Methods

A systematic literature review was conducted using original publications since 2013. Comprehensive searches were performed in both English (EMBASE, MEDLINE) and Chinese (CNKI, Wanfang) databases. Articles were included when information on epidemiology, natural history, humanistic or economic burden of PAH was available.

Results

Among 4808 abstracts and 333 full-text articles identified, 156 met inclusion/exclusion criteria: natural history (155), epidemiology (17), health-related quality of life (HRQoL) (32), health resource utilization (HCRU) (8) and cost (3). Majority (85.9 %) relied on single-hospital data. No studies reported population-level incidence or prevalence. Congenital heart disease associated PAH (CHD-PAH) was the most common PAH-subtype. Of the 155 studies, 55.5 % focused on connective tissue disease-associated PAH (CTD-PAH). Excluding studies of special groups, the median proportion of female was 74.3 % (range: 55.0 %–95.0 %), the median age was 36.2 years (range: 32.3–55.6 years), and the median 5-year survival rate was 74.1% (range:15.0%–87.6%). For PAH-targeted therapy, monotherapy (primarily phosphodiesterase-5 inhibitors and endothelin receptor antagonists) was prescribed more often than combination therapies. 36-Item Short Form Survey (SF-36) was the most utilized HRQoL instrument. Among 32 studies detailing HRQoL, 14 demonstrated a significant improvement in HRQoL after intervention. One study reported that 70.0 % of PAH patients were hospitalized at least once a year, and 14.0 % were hospitalized 3–5 times a year. One study highlighted substantial economic burden, citing average annual out-of-pocket costs of $10,388 per patient in 2021.

Conclusion

In China, PAH is predominantly reported among young females, and the most common reported subtype is CHD-PAH. Population-level studies are needed to better understand the epidemiology, treatment patterns, and disease burden of PAH.