Table 4.

Patients' survival of treatment with bosentan.

Numbers	F/M	Age, y	Treatment	Survival	Conclusion	References
86	49/37	11.0 (0–18.0)	Bosentan concomitant Prostanoid (n = 42) Bosentan (n = 44)	The entire group: survival at 1- and 2-year was 100 and 88%, respectively Bosentan: 1- and 2-year survival was 98 and 94% Concomitant prostanoid: 1- and 2- year survival was 98 and 89%, respectively	Bosentan prolongs the life	Rosenzweig et al. (40)
7	6/1	7.4	Bosentan	The 3- and 5-year survival was 100 and 75%, respectively	Survival improved	Simpson et al. (43)
64	26/38	4.3 (1.5–8.9)	Bosentan (n = 23) Prostanoids (n = 15) Sildenafil (n = 9) Combined therapy (n = 11) Calcium channel antagonists (n = 6)	The entire group: 1-, 3-, and 5-year survival was 89, 84, and 75% for the entire group, respectively	The entire group improved, but with no data in bosentan, and there had no difference among bosentan, prostanoids, and sildenafil	Moledina et al. (36)
36	16/20	10.5 (1.0–16.0)	Bosentan (n = 11) Bosentan plus prostanoid (n = 25)	The entire group: survival at 1-, 2-, 3-, and 4-year was 98, 88, 82, and 82%, respectively	Most children improved in survival	Ivy et al. (38)
42	26/16	9.7	Bosentan	The survival values was 95, 95, 95, and 55% in 1, 2, 3, and 5 years, respectively	Effective in the long-term management	Hislop et al. (16)
122	73/49	15.0	53 out of 122 patients taking endothelin receptor antagonist: eight out of 53 received sitaxsentan, 45 out of 53 received bosentan	The survival of 122 patients in 6 months, 1- and 2-year was 99, 95, and 90%, respectively	Survival has been improved by targeted therapy	Barst et al. (30)
36	21/15	6.8 (2.0–12.0)	Bosentan	Estimated long-term survival at 2- and 4-year was 91.2 and 84.0%, respectively	There was an improvement in survival	Berger et al. (4)

F, Female; M, Male.