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.