Background Primary pulmonary hypertension is a progressive disease for which no treatment
has been shown in a prospective, randomized trial to improve survival. Methods We …

Background Endothelin-1 is a potent vasoconstrictor and smooth-muscle mitogen. In a
preliminary study, the orally administered dual endothelin-receptor antagonist bosentan improved …

… Although PAH occurs with greater frequency in individuals who have used intravenous
drugs, no clear etiological link has been established with foreign body emboli or the portal …

Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure ≥25 mm Hg
at rest, measured during right heart catheterization. There is still insufficient evidence to add …

Background: Pulmonary hypertension is a progressive and often fatal complication of the
scleroderma spectrum of disease for which no treatment has been proven effective in a …

Background Endothelin 1, a powerful endogenous vasoconstrictor and mitogen, might be a
cause of pulmonary hypertension. We describe the efficacy and safety of bosentan, a dual …

Background— Factors that determine survival in pulmonary arterial hypertension (PAH) drive
clinical management. A quantitative survival prediction tool has not been established for …

The diagnosis and assessment of pulmonary arterial hypertension is a rapidly evolving area,
with changes occurring in the definition of the disease, screening and diagnostic techniques…

Background The Registry to EValuate Early And Long-term pulmonary arterial hypertension
disease management (REVEAL Registry) was established to provide updated …

Background— Ambrisentan is a propanoic acid–based, A-selective endothelin receptor
antagonist for the once-daily treatment of pulmonary arterial hypertension. Methods and Results…