Pulmonary hypertension (PH) is a severe, progressive disease characterized by an elevated mean pulmonary artery pressure (mPAP) 25 mmHg. 1,2 Five PH subgroups have been recognized, but majority of studies on survival have focused on group 1 pulmonary arterial hypertension (PAH) determined by right heart catheterization (RHC) with a mPAP 25 mmHg, pulmonary arterial wedge pressure (PAWP) < 15 mmH...

Pulmonary arterial hypertension (PAH) is defined as an intractable disease characterized by a progressive elevation of pulmonary vascular resistance (PVR) and pulmonary arterial pressure (PAP), leading to right heart failure and premature death. The five-year survival rate after diagnosis is approximately 57%. Although extensive research has identified some factors associated with the cause of ...

The effect of oxygen breathing on the stiffness of the large pulmonary artery has not been elucidated. We analyzed the proximal pulmonary arterial impedance with a multisensor catheter in ten patients with pulmonary arterial hypertension (PAH), eight patients with pulmonary venous hypertension, and six control subjects. The stiffness of the vessel was quantified by the characteristic impedance ...

In recent times, the prevalence of pulmonary arterial hypertension (PAH) is more commonly seen among elderly populations. The increased prevalence of hypertension, diabetes, obesity, arterial stiffness, as well as diastolic dysfunction, may cause endothelial dysfunction and affect pulmonary vasculature. Furthermore, older patients have certain differences in clinical characteristics and outcome...

Elevated pulmonary arterial pressure in patients with pulmonary hypertension (PH) is mainly caused by increased pulmonary vascular resistance (PVR), due primarily to sustained pulmonary vasoconstriction and excessive pulmonary vascular remodeling. According to the current classification, PH has been classified into five categories based on etiology (Simonneau et al, 2013). Among them, group 1 o...

BACKGROUND Vasoactive intestinal peptide (VIP), a pulmonary vasodilator and inhibitor of vascular smooth muscle proliferation, has been reported absent in pulmonary arteries from patients with idiopathic pulmonary arterial hypertension (PAH). We have tested the hypothesis that targeted deletion of the VIP gene may lead to PAH with pulmonary vascular remodeling. METHODS AND RESULTS We examined...

Adult-onset Still's disease (AOSD) is a rare inflammatory condition characterized by spiking quotidian fever, rash, chronic arthralgia, leukocytosis, and occasional pulmonary involvement such as pleural effusion and transient pulmonary infiltrates. Pulmonary arterial hypertension (PAH) is a rare pulmonary complication of AOSD, and we are aware of only 5 cases reported in the literature. We repo...

Shroom is a PDZ-domain protein involved in the regulation and maintenance of cytoskeletal architecture by binding to actin. Hypertrophy and altered actin organisation of pulmonary arterial smooth muscle cells (PASMC) is a hallmark of pulmonary arterial hypertension (PAH). The aim of the present study was to localise and characterise Shroom expression in the lung in experimental and idiopathic P...

Although multiple gene and protein expression have been extensively profiled in human pulmonary arterial hypertension (PAH), the mechanism for the development and progression of pulmonary hypertension remains elusive. Analysis of the global metabolomic heterogeneity within the pulmonary vascular system leads to a better understanding of disease progression. Using a combination of high-throughpu...