The effects of fasudil on the degree of muscularization. The proportions of partially and fully muscularized pulmonary arteries significantly increased in SM22-tet-BMPR2R899X mice (26.5 and 4.3%, respectively) compared with those in the controls (7.8 and 0.5%, respectively). Fasudil decreased the proportions of partially and fully muscularized pulmonary arteries (19.5 and 1.8%, respectively). N, nonmuscular; P, partially muscular; M, fully muscular. Brown, von Willebrand factor; green, α-smooth muscle actin. *P < 0.0001 and **P < 0.01.