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...

RATIONALE Pulmonary hypertension (PH) is characterized by increased arterial load requiring more right ventricular (RV) hydraulic power to sustain adequate forward blood flow. Power can be separated into a mean and oscillatory part. The former is associated with mean and the latter with pulsatile blood flow and pressure. Because mean power provides for net blood flow, the ratio of oscillatory t...

Hyaluronan (HA), a large glycosaminoglycan found in the ECM, has major roles in lung and vascular biology and disease. However, its role in idiopathic pulmonary arterial hypertension (IPAH) is unknown. We hypothesized that HA metabolism is abnormal in IPAH. We measured the plasma levels of HA in IPAH and healthy individuals. We also evaluated HA synthesis and the expression of HA synthases and ...

The aim of this study was to confirm the utility of aerosolised iloprost for identifying long-term responders to calcium channel blockers (CCBs) in patients with idiopathic pulmonary arterial hypertension (IPAH). While undergoing right heart catheterisation, 74 patients with IPAH sequentially received incremental infusions of adenosine and aerosolised iloprost. The effects of the two vasodilato...

BACKGROUND Direct evidence for Rho-kinase activation in patients with pulmonary hypertension (PH) is still lacking. METHODS AND RESULTS Rho-kinase activity in circulating neutrophils was examined by determining the ratio of phosphorylated/total forms of myosin-binding subunit, a substrate of Rho-kinase, in 40 consecutive PH patients and 40 healthy controls. Next, Rho-kinase expression and act...

BACKGROUND Long-term anticoagulation is recommended in idiopathic pulmonary arterial hypertension (IPAH). In contrast, limited data support anticoagulation in pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-PAH). We assessed the effect of warfarin anticoagulation on survival in IPAH and SSc-PAH patients enrolled in Registry to Evaluate Early and Long-term PAH Disea...

Vasoconstriction and vascular medial hypertrophy, resulting from increased intracellular [Ca2+] in pulmonary artery smooth muscle cells (PASMC), contribute to elevated vascular resistance in patients with idiopathic pulmonary arterial hypertension (IPAH). Caveolae, microdomains within the plasma membrane, contain the protein caveolin, which binds certain signaling molecules. We tested the hypot...

INTRODUCTION Non-tuberculous mycobacterial lung diseases (NTMLD) occur rarely and are diagnosed mainly in patients belonging to risk groups. Pulmonary hypertension (PH) has not been recognised as a risk factor for NTMLD yet. The aim of the study was to analyse the clinical course and predisposing factors of NTMLD recognised in our centre between 2002 and 2012 in patients with chronic thromboemb...

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...

Said, Sami I. Mediators and modulators of pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol 291: L547–L558, 2006. First published May 12, 2006; doi:10.1152/ajplung.00546.2005.—Pulmonary hypertension (PH), defined as a mean pulmonary arterial (PA) pressure of 25 mmHg at rest or 30 mmHg during exercise, is characterized by a progressive and sustained increase in pulmonary vascul...