▪ Objective: To characterize mortality in persons diagnosed with primary pulmonary hypertension
and to investigate factors associated with survival. ▪ Design: Registry with prospective …

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 …

Pulmonary hypertension (PH) is a complex, multidisciplinary disorder. Recent advances
have led to increased recognition and new therapies. While some data exist to form …

Background Sildenafil inhibits phosphodiesterase type 5, an enzyme that metabolizes cyclic
guanosine monophosphate, thereby enhancing the cyclic guanosine monophosphate–…

Level of Evidence A Data derived from multiple randomised clinical trials or meta-analyses
Level of Evidence B Data derived from a single randomised clinical trial or large non-…

Pulmonary arterial hypertension is a life-threatening disease for which continuous intravenous
prostacyclin has proven to be effective. However, this treatment requires a permanent …

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

Pulmonary arterial hypertension (PAH) is diagnosed by various investigations that are
essential for making the diagnosis, and by additional tests to clarify the category of pulmonary …

Familial primary pulmonary hypertension is a rare autosomal dominant disorder that has
reduced penetrance and that has been mapped to a 3-cM region on chromosome 2q33 (locus …