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Authors/Task Force Members: Nazzareno Galie (Chairperson) (Italy) ; Marius M. Hoeper (Germany); Marc Humbert (France); Adam Torbicki (Poland); Jean-Luc Vachiery (France); Joan Albert Barbera (Spain); Maurice Beghetti (Switzerland); Paul Corris (UK); Sean Gaine (Ireland); J. Simon Gibbs (UK); Miguel Angel Gomez-Sanchez (Spain); Guillaume Jondeau (France);(More)
Authors/Task Force Members: Stavros Konstantinides* (Chairperson) (Germany/ Greece), Adam Torbicki* (Co-chairperson) (Poland), Giancarlo Agnelli (Italy), Nicolas Danchin (France), David Fitzmaurice (UK), Nazzareno Galiè (Italy), J. Simon R. Gibbs (UK), Menno Huisman (The Netherlands), Marc Humbert† (France), Nils Kucher (Switzerland), Irene Lang (Austria),(More)
Authors/Task Force Members: Vera Regitz-Zagrosek (Chairperson) (Germany)*, Carina Blomstrom Lundqvist (Sweden), Claudio Borghi (Italy), Renata Cifkova (Czech Republic), Rafael Ferreira (Portugal), Jean-Michel Foidart† (Belgium), J. Simon R. Gibbs (UK), Christa Gohlke-Baerwolf (Germany), Bulent Gorenek (Turkey), Bernard Iung (France), Mike Kirby (UK), Angela(More)
RATIONALE Phosphodiesterase type 5 (PDE5) inhibition has been proposed for the treatment for pulmonary arterial hypertension (PAH). OBJECTIVE This study compared adding sildenafil, a PDE5 inhibitor, to conventional treatment with the current practice of adding bosentan, an endothelin receptor antagonist. METHODS Twenty-six patients with PAH, idiopathic(More)
Uncontrolled and controlled clinical trials with different compounds and procedures are reviewed to define the risk-benefit profiles for therapeutic options in pulmonary arterial hypertension (PAH). A grading system for the level of evidence of treatments based on the controlled clinical trials performed with each compound is used to propose an(More)
BACKGROUND Opioids are commonly used to treat dyspnoea in palliative medicine but there has been no formal evaluation of the evidence for their effectiveness in the treatment of dyspnoea. A systematic review was therefore carried out to examine this. METHODS The criteria for inclusion required that studies were double blind, randomised, placebo controlled(More)
BACKGROUND Primary pulmonary hypertension (PPH), resulting from occlusion of small pulmonary arteries, is a devastating condition. Mutations of the bone morphogenetic protein receptor type II gene (BMPR2), a component of the transforming growth factor beta (TGF-beta) family which plays a key role in cell growth, have recently been identified as causing(More)
RATIONALE Pulmonary arterial hypertension in association with connective tissue disease (CTD-PAH) has historically had a poor prognosis, with a 1-year survival rate among patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) of 45%. However, more therapies have become available. OBJECTIVES To investigate the survival and(More)
Pulmonary hypertension (PH), a common complication of left heart diseases (LHD), negatively impacts symptoms, exercise capacity, and outcome. Although the true prevalence of PH-LHD is unknown, a subset of patients might present significant PH that cannot be explained by a passive increase in left-sided filling pressures. The term "out-of-proportion" PH has(More)