BACKGROUND Pulmonary hypertension occurs in chronic hypoxic lung diseases, significantly worsening morbidity and mortality. The important role of altered bone morphogenetic protein (BMP) signaling in pulmonary hypertension was first suspected after the identification of heterozygous BMP receptor mutations as the underlying defect in the rare heritable form of pulmonary arterial hypertension. Su...

BACKGROUND The plexiform lesion is the hallmark of severe pulmonary arterial hypertension. However, its genesis and hemodynamic effects are largely unknown because of the limited availability of lung tissue samples from patients with pulmonary arterial hypertension and the lack of appropriate animal models. This study investigated whether rats with severe progressive pulmonary hypertension deve...

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Pulmonary arterial hypertension (PAH) is characterized by vascular obstruction and the variable presence of vasoconstriction, leading to increased pulmonary vascular resistance and right-sided heart failure. PAH can present in an idiopathic form, usually called primary pulmonary hypertension (PPH), and PAH is also associated with the scleroderma spectrum of diseases, HIV infection, portal hyper...

Pulmonary arterial hypertension (PAH) is a term used to classify a variety of conditions that have in common an injury to the pulmonary vasculature that produces elevations in pulmonary arterial pressure. There have been considerable advances in our understanding of the pathogenesis and treatment of PAH over the past decade. The article reviews the classification of diseases associated with PAH...

ABBREVIATIONS : AHI 5 apnea-hypopnea index ; BNP 5 B-type natriuretic peptide ; CRP 5 C-reactive protein ; CSA 5 central sleep apnea ; CSR 5 Cheyne-Stokes respiration ; CTEPH 5 chronic thromboembolic pulmonary hypertension ; IPAH 5 idiopathic pulmonary arterial hypertension ; LV 5 left ventricular ; mPAP 5 mean pulmonary artery pressure ; NPV 5 negative predictive value ; OHS 5 obesity hypovent...

Pulmonary hypertension is a complex and multidisciplinary disorder. The classification of pulmonary hypertension includes 5 groups. Pulmonary arterial hypertension is a rare disorder that can be idiopathic or heritable in nature, or associated with other conditions, such as scleroderma or congenital heart disease. The recent decades have realized advances in the treatment of this once devastati...

Pulmonary arterial hypertension (PAH) is a rare condition characterised by increased pulmonary vascular resistance leading to right heart failure and death. The clinical classification distinguishes idiopathic PAH, familial PAH, and PAH associated with other conditions (connective tissue disease, congenital heart disease, portal hypertension, human immunodeficiency virus infection, or appetite ...

Pulmonary arterial hypertension is characterized by pulmonary vascular remodeling, cellular proliferation, and poor long-term outcomes. Dysfunctional bone morphogenetic protein pathway signaling associated with both hereditary idiopathic subtypes. Sotatercept, a novel fusion protein, binds activins growth differentiation factors in the attempt to restore balance between growth-promoting growth-...