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

In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in
Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification …

Primary pulmonary hypertension is a condition characterized by sustained elevations of
pulmonary-artery pressure without a demonstrable cause. The diagnostic criteria used in the …

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

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

Background Uncontrolled studies suggested that aerosolized iloprost, a stable analogue of
prostacyclin, causes selective pulmonary vasodilatation and improves hemodynamics and …

… , David B Badesch, and Lewis J Rubin contributed in patient recruitment and assessments,
and … the study, assisted by Lewis J Rubin, monitored clinical and laboratory assessments, …

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 …