Table 5.

Safety and tolerability in pediatric patients treated with bosentan.

Numbers	F/M	Age, y	Treatment	Safety and side effects	Death	References
19	10/9	3.0–15.0	10 out of 19 received bosentan	No data	No death	Barst et al. (24)
86	49/37	11.0 (0–18.0)	Bosentan concomitant Prostanoid (n = 42) Bosentan (n = 44)	No data	Bosentan + prostanoid: 3 patients died: two hemoptysis and acute respiratory distress syndrome, one worsening right heart failure Bosentan: two died from right heart failure	Rosenzweig et al. (40)
7	6/1	7.4	Bosentan	No data	One patient died of a pulmonary hypertensive crisis	Simpson et al. (43)
7	3/4	9.6 (1.0–16.0)	Sildenafil (n = 5) Bosentan (n = 2)	No patient suffered important side effects	No data	Raposo-Sonnenfeld et al. (35)
146	71/75	2.0–11.0	59 out of 146 patients received bosentan	30.8% had at least one safety signal	About 7.5% patients died in the entire group, but not related to bosentan	Beghetti et al. (48)
36	16/20	10.5 (1.0–16.0)	Bosentan (n = 11) Bosentan plus prostanoid (n = 25)	No data	Six died among 36 patients: one for right heart failure one for sudden death one for worsening pulmonary hypertension one for hemoptysis one for pulmonary hemorrhage one for thromboembolism	Ivy et al. (38)
36	21/15	6.8 (2.0–12.0)	Bosentan	Bosentan-related AEs occurred in 15 (41.7%) patients	Six deaths occurred, but unrelated to bosentan: three from worsen of PAH and cardiac complications, one from respiratory failure following pneumonia	Berger et al. (39)

F, Female; M, Male.