Pulmonary Arterial Hypertension in Latin America: Characteristics, Treatment, and Survival From the LATAM-PAH Meta-Analysis and Systematic Review

Marcos Vinicius Fernandes Garcia, Katherine Julian, Evans R. Fernández Pérez, Rogerio Souza, Tim Lahm

https://doi.org/10.1002/pul2.70403 

Abstract

Pulmonary arterial hypertension (PAH) in Latin America and the Caribbean (LAC) remains poorly characterized, with limited regional data on clinical presentation, hemodynamics, treatment patterns, and outcomes. We aimed to characterize demographic features, clinical presentation, hemodynamic severity, treatment patterns, and survival outcomes of right heart catheterization-confirmed PAH cohorts across LAC. We performed a systematic review and meta-analysis of retrospective and prospective cohort studies of adults with right heart catheterization-confirmed PAH in LAC, searching six databases in English, Spanish, and Portuguese without date restrictions. Study-level aggregate data were pooled using random-effects models. Sixteen studies from eight countries (1999–2025) including 2,553 patients were analyzed. Mean age was 43.6 years and 81.5% were female. Etiologies included idiopathic PAH (40.3%), congenital heart disease-associated PAH (25.6%), connective tissue disease-associated PAH (20.6%), and schistosomiasis-associated PAH (15.3% in Brazilian cohorts). At diagnosis, 51% were in functional class III/IV, mean 6-min walk distance was 406 m, mean pulmonary arterial pressure was 55.8 mmHg, and cardiac index was 2.63 L/min/m2. Treatment consisted primarily of monotherapy (56%) or dual therapy (33%). Pooled survival was 93.9% at 1 year, 87.5% at 2 years, and 83.0% at 3 years. Predominantly reflecting South American PAH care, our findings establish the first regional benchmark for PAH in LAC. Patients are younger and predominantly female, with a high burden of CHD-PAH and schistosomiasis-associated PAH. Despite delayed diagnosis and limited combination therapy access, survival is comparable to high-income registries, highlighting priorities such as earlier referral, risk assessment, expanded therapy access, and prospective registry development.

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