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 ...

The pathogenesis of idiopathic pulmonary arterial hypertension (PAH) remains poorly understood. The present authors recently reported that mice with vasoactive intestinal peptide (VIP) gene disruption show a spontaneous phenotype of PAH, with pulmonary vascular remodelling and lung inflammation. To explore the underlying molecular mechanisms in this model, it was examined whether absence of the...

Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common type of pulmonary arterial hypertension (PAH) and a frequent complication of congenital heart disease (CHD). PAH-CHD represents a heterogeneous patient population and it is important to distinguish between the underlying cardiac defects considering the prognostic and therapeutic implications. Improved...

Pul monary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure ≥25 mmHg by right heart catheterization and a pulmonary artery wedge pressure ≤15 mmHg, indicating an absence of left ventricular dysfunction. In many patients, PAH is a progressive disease associated with morbidity and often fatal. 1 The traditional concept of primary versus secondary pulmonary hypertension h...

Anti-endothelial cell antibodies (AECAs) have been identified in patients with systemic sclerosis (SSc) with and without pulmonary arterial hypertension (PAH) and in patients with idiopathic pulmonary arterial hypertension (iPAH). However, their target antigens remain poorly identified. Sera from 24 patients with SSc without PAH, 20 patients with SSc with PAH, 30 with iPAH and 12 healthy contro...

Human immunodeficiency virus (HIV) attacks the immune system and can cause acquired syndrome. Thankfully, antiretroviral therapy is highly effective, people infected with HIV live long lives proper adherence to therapy. One of important noninfectious complications development pulmonary arterial hypertension (PAH). This review will cover epidemiology, pathogenesis, clinical characteristics HIV-a...

The Ministry of Health, Labour and Welfare (Japan) has approved research into primary pulmonary hypertension (PPH) and pulmonary hypertension due to chronic thromboembolic and/or embolic disease (CTE-PH) to examine their epidemiology, pathophysiology, and develop new therapeutic strategies. The Respiratory Failure Research Group, with grant support from the Ministry of Health, Labour and Welfar...

OBJECTIVES The objective of this study was to evaluate the natural history of untreated schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) patients as compared to idiopathic pulmonary arterial hypertension (IPAH) with respect to hemodynamics recorded at presentation and 36 months survival. BACKGROUND Schistosomiasis (Sch) is one of the most prevalent chronic infectious disea...

Pulmonary arterial hypertension (PAH) is an intractable disease of the small pulmonary artery that involves multiple inflammatory factors. We hypothesized that a redox-sensitive transcription factor, nuclear factor B (NFB), which regulates important inflammatory cytokines, plays a pivotal role in PAH. We investigated the activity of NFB in explanted lungs from patients with PAH and in a rat mod...