ArticleOpen heart2025
Long-term outcome of patients with Eisenmenger syndrome receiving pulmonary arterial hypertension-targeted therapy.
Article in Open heart, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
1 citing paper in PubMed.
- A second-hit conceptual framework for pulmonary arterial hypertension in adult congenital heart disease: genetics, hemodynamics, and treat-and-repair.Frontiers in cardiovascular medicine · 2026Review
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Authors and funding
9 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundEisenmenger syndrome (ES) is a severe and fatal complication of uncorrected congenital heart disease characterised by the development of pulmonary arterial hypertension (PAH) due to chronic left-to-right shunting. Despite the increasing use of PAH-targeted therapies, evidence of their long-term effects on the survival of patients with ES remains limited.
methodsWe reviewed 101 consecutive adult patients with ES (69% female; mean age, 34±18 years) who were referred to our centre between December 1972 and December 2023. We investigated the clinical, haemodynamic and treatment data from medical records. The primary outcome measure was transplantation-free survival. The effect of PAH-targeted therapy on transplantation-free survival was analysed using a time-dependent Cox proportional hazards model.
resultsAmong the 101 patients, 57 (56%) received PAH-targeted therapy (PAH therapy group), while 44 (44%) did not (treatment-naïve group). In the PAH therapy group, 75% received combination therapy (25%, monotherapy; 56%, dual-combination therapy; and 19%, triple-combination therapy). The median follow-up time since diagnosis of ES was 8.8 (IQR: 4.1-22.3) years. The median follow-up transplantation-free survival rates were significantly higher in the PAH therapy group than in the treatment-naïve group (100%, 100% and 89% vs 95%, 66% and 53% at 1, 5 and 10 years, respectively; p<0.001). In the multivariate analysis adjusting for age, sex, WHO functional class (III/IV vs I/II) and PAH-targeted therapy, PAH-targeted therapy was independently associated with improved transplantation-free survival (adjusted HR=0.275, 95% CI 0.132 to 0.572).
conclusionsThe long-term survival of patients with ES who received PAH-targeted therapy was better than that of those who did not. These results support the benefits of PAH-targeted therapies in this population while underscoring the need for large-scale studies to better define optimal treatment strategies.
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