3.2 Associations of PAH-specific vasodilators with long-term survival
A total of 1208 PAH patients were included in the survival analyses. Figure 2 shows Kaplan-Meier curves of PAH-specific vasodilators divided into respective drug classes. The PAH patients treated with epoprostenol had significantly longer survival than those treated with treprostinil and non-user patients (p < 0.001). The median survival times (95% confidence interval [CI]) of the PAH patients treated with epoprostenol and treprostinil and the non-user patients were 8.41 years (8.41–not available [NA]), 7.73 years (4.75­–NA), and 5.00 years (3.95–6.26), respectively. In contrast, there was no difference in survival between the PAH patients treated with iloprost, beraprost, and selexipag and non-user patients (p = 0.461). The median survival times (95% CI) of the PAH patients treated with iloprost, beraprost, and selexipag and the non-user patients were not reached (1.85–NA), 5.11 years (4.33–7.73), 5.55 years (3.48–6.83), and 9.43 years (6.05–NA), respectively. The PAH patients treated with drugs targeting the NO pathway showed significant differences in survival (p = 0.020). The median survival times (95% CI) of the PAH patients treated with sildenafil, tadalafil, and riociguat and non-user patients were 4.47 years (3.82–6.05), 6.26 years (5.25–9.43), 6.83 years (6.83–NA), and 10.93 years (6.21–NA), respectively. The PAH patients treated with endothelin receptor antagonists (ERAs) showed significantly longer survival than non-user patients (p< 0.001). The median survival times (95% CI) of the PAH patients treated with bosentan, ambrisentan, and macitentan and the non-user patients were 6.05 years (5.00–NA), 4.40 years (3.43–NA), 6.83 years (6.26–NA), and 2.44 years (1.53–NA), respectively.