Severe pulmonary hypertension has a poor prognosis and is complicated
to treat. It is caused by states of hypoxia, thromboembolism, chronic
inflammation, pulmonary venous congestion and/or hypercirculation. In
the course of some days up to many years, remodelling of the pulmonary
vasculature map occur. Morphologic characteristics of the remodelling
process have extensively been described, while the underlying cellula
r and molecular mechanisms remain largely unknown. Increasing knowledg
e of the features of pulmonary vasoconstriction and vascular remodelli
ng, will, however, improve the options for future therapy.