Pulmonary arterial hypertension (PAH) is a rare disease often resulting in right-sided heart failure and premature death [1]. PAH is idiopathic (IPAH), heritable, or related to conditions such as connective tissue diseases (CTD) [2]. IPAH prognosis remains poor despite improved patient survival with current treatment options. Therefore, elucidating the pathophysiology of IPAH is important for t...

BACKGROUND/AIMS Idiopathic pulmonary arterial hypertension (IPAH) is an incurable disease with high mortality. Although most studies recommend anticoagulation treatment for IPAH, the benefits are uncertain, particularly in Korea, where it has not been studied. The purpose of this study was to evaluate survival outcomes of Korean patients with IPAH treated with warfarin. METHODS We performed a...

Idiopathic pulmonary arterial hypertension (IPAH) is a rare and progressive disease. Several processes are believed to lead to the fatal progressive pulmonary arterial narrowing seen in IPAH including vasoconstriction, cellular proliferation inflammation, vascular remodeling, abnormalities in the lung matrix, and in situ thrombosis. Nitric oxide (NO) produced by NO synthases (NOS) is a potent v...

Knowledge of molecular mechanisms underlying pulmonary arterial hypertension (PAH) continues to increase with the emerging theme that PAH is a heterogeneous disease involving multiple molecular abnormalities. Mutations in several genes have been identified in subsets of patients with PAH, and multiple signaling systems that influence vascular tone, function, and remodeling have been associated ...

To the Editor: We read with great interest the article in CHEST (June 2015) by Hautefort et al, 1 who demonstrated activity of the T helper 17 pathway in patients with idiopathic pulmonary arterial hypertension (IPAH). Th e authors further substantiated a potential role of an autoimmune pathogenesis in IPAH, a disease defi ned by the absence of known causes or associated conditions. “A fl avor ...

Idiopathic pulmonary arterial hypertension (IPAH) is a progressive, nearly fatal condition that until recently has had very few treatment options. Median survival time for untreated IPAH was 2.8 years without effective drug intervention. IPAH is characterized by deregulated proliferation of pulmonary arterial endothelial and intimal smooth muscle cells resulting in progressive pulmonary vascula...

RATIONALE An increase in cytosolic free Ca(2+) concentration ([Ca(2+)](cyt)) in pulmonary arterial smooth muscle cells (PASMC) is a major trigger for pulmonary vasoconstriction and an important stimulus for PASMC proliferation and pulmonary vascular remodeling. The dihydropyridine Ca(2+) channel blockers, such as nifedipine, have been used for treatment of idiopathic pulmonary arterial hyperten...

I n this issue of the European Respiratory Journal, MATHAI et al. [1] (from Johns Hopkins University, Baltimore, MD, USA) report on their experience with the use of sildenafil, a phosphodiesterase-5 inhibitor, in patients with pulmonary arterial hypertension (PAH) who failed monotherapy with bosentan, an endothelin receptor antagonist. Within a 4-yr period, 82 patients with either idiopathic PA...

The pore-forming alpha-subunit, Kv1.5, forms functional voltage-gated K(+) (Kv) channels in human pulmonary artery smooth muscle cells (PASMC) and plays an important role in regulating membrane potential, vascular tone, and PASMC proliferation and apoptosis. Inhibited Kv channel expression and function have been implicated in PASMC from patients with idiopathic pulmonary arterial hypertension (...

Idiopathic pulmonary arterial hypertension (IPAH) in human patients is associated with mutations in type 2 receptor for the bone morphogenic protein pathway (BMPR2). Mice expressing an inducible dominant negative form of BMPR2 in smooth muscle develop elevated right ventricular pressures when the transgene is activated. We hypothesized that transcriptional changes in these mice may allow insigh...