Pulmonary arterial hypertension (PAH) affects 10% to 15% of patients with systemic sclerosis and is a major cause for disease-related morbidity and mortality. Over the past decade, significant progress has been made in the understanding of the pathophysiologic mechanisms underlying PAH. This progress led to the development of several new treatment options and, as a result, dramatically improved...

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...

We report three cases which highlight the complex considerations surrounding genetic counselling for pulmonary arterial hypertension (PAH). The first counselee developed PAH symptoms shortly after his daughter's death from PAH and was diagnosed with a delay of 1 year. An early diagnosis of familial PAH was established in the second counselee. Oral therapy was initiated immediately, and her func...

Although rare in its idiopathic form, pulmonary arterial hypertension (PAH) is not uncommon in association with various associated medical conditions, most notably connective tissue disease (CTD). In particular, it develops in approximately 10% of patients with systemic sclerosis and so these patients are increasingly screened to enable early detection. The response of patients with systemic sc...

Identification of sources of polycyclic aromatic hydrocarbons (PAH) in sediments is an important step in reducing anthropogenic contamination. Identifications based solely on the composition of PAH can be confounded by compositional changes during weathering and biodegradation. Stable isotopic composition of individual PAH offers an additional marker, which can aid in distinguishing sources and...

Pulmonary arterial hypertension (PAH) is a devastating vascular complication of a number of CTDs. In patients with SSc, PAH has a dramatic impact on prognosis and survival and is the single most common cause of disease-related death.Yearly echocardiographic screening for PAH is recommended in patients with SSc. If suspected, confirmation of PAH diagnosis by right heart catheterization is necess...

BACKGROUND In pulmonary arterial hypertension (PAH), the six-minute walk test (6MWT) is believed to be representative of patient's daily life physical activities (DL(PA)). Whether DL(PA) are decreased in PAH and whether the 6MWT is representative of patient's DL(PA) remain unknown. METHODS 15 patients with idiopathic PAH (IPAH) and 10 patients with PAH associated with limited systemic scleros...

Wildland (forest) firefighters are exposed to a wide range of carcinogenic polycyclic aromatic hydrocarbons (PAH) in forest fire smoke. PAH undergo metabolic activation and can subsequently bind to DNA. In this study, we investigated the association between occupational and dietary PAH exposures and the formation of WBC PAH-DNA adducts in a population of wildland firefighters. An enzyme-linked ...

BACKGROUND Pulmonary arterial hypertension (PAH) is a common complication for individuals with limited systemic sclerosis (lSSc). The identification and characterization of biomarkers for lSSc-PAH should lead to less invasive screening, a better understanding of pathogenesis, and improved treatment. METHODS AND FINDINGS Forty-nine PBMC samples were obtained from 21 lSSc subjects without PAH (...

RATIONALE The impact of modern treatments of pulmonary arterial hypertension (PAH) on pulmonary vascular pathology remains unknown. OBJECTIVES To assess the spectrum of pulmonary vascular remodeling in the modern era of PAH medication. METHODS Assessment of pulmonary vascular remodeling and inflammation in 62 PAH and 28 control explanted lungs systematically sampled. MEASUREMENTS AND MAIN...