Idiopathic pulmonary arterial hypertension (IPAH) is a devastating pulmonary vascular disease in which autoimmune and inflammatory phenomena are implicated. B cells and autoantibodies have been associated with IPAH and identified as potential therapeutic targets. However, the specific populations of B cells involved and their roles in disease pathogenesis are not clearly defined. We aimed to as...

OBJECTIVE We explored the potential role of the endogenous NO synthase inhibitor asymmetrical dimethylarginine (ADMA) in patients with idiopathic pulmonary arterial hypertension (IPAH). Method and Results- We correlated plasma ADMA levels and cardiovascular indices from right heart catheterization in 57 patients with IPAH. Predictors of survival in patients with IPAH were studied. Furthermore, ...

Pulmonary arterial hypertension (PAH) is fatal if untreated. Intravenous epoprostenol improves exercise capacity and haemodynamics in PAH, and increases survival in idiopathic PAH (IPAH). To evaluate the effects of subcutaneous (SC) treprostinil, a longer-acting prostacyclin analogue, followed by the addition of other PAH therapies if needed, 860 PAH patients treated with SC treprostinil for up...

It has been proposed that targeted treatments should be combined for patients with idiopathic pulmonary arterial hypertension (IPAH) responding insufficiently to monotherapy. This study followed the clinical course of nine patients with severe IPAH, in whom the endothelin receptor antagonist bosentan caused transient clinical improvement, eventually followed by a decline in exercise tolerance, ...

Despite the development of specific therapies for pulmonary arterial hypertension (PAH) some patients fail to respond to such treatment. One of the potential reasons for the unresponsiveness to targeted therapies may be the presence of fibrous occlusion of small pulmonary veins that accompanies pre-capillary arteriopathy. This type of pathologic change is called pulmonary veno-occlusive disease...

BACKGROUND Although pulmonary endarterectomy (PEA) is potentially curative in chronic thromboembolic pulmonary hypertension (CTEPH), some patients have distally distributed disease that is not amenable to surgery. The aetiology and characteristics of this patient group are currently not well understood. OBJECTIVES This study compares the baseline demographic features and outcomes in subjects ...

he earliest clinical descriptions of primary pulmonary hypertension, now known as idiopathic pulmonary arterial hypertension (IPAH), were accompanied by the hypothesis that pulmonary vasoconstriction was one of its key pathophysiological elements [1] and that vasodilators could, therefore, be therapeutically useful. In support of this concept, DRESDALE et al. [2] first reported attenuation of p...

OBJECTIVE To investigate the probable pathogenesis, clinical features, diagnosis, and therapy of patients with pulmonary hypertension (PH) in Takayasu arteritis (TA). METHODS A total of 48 patients with TA who had PH, 20 patients with TA who had pulmonary arterial involvement (PA) without PH, and 30 patients with idiopathic pulmonary arterial hypertension (IPAH) were enrolled in the study fro...

Background Stimulators of the nitric oxide (NO) receptor soluble guanylate cyclase (sGC) are a new class of substances which enhance the biosynthesis of cyclic guanosin monophosphate (cGMP) independently from the presence of nitric oxide (NO). In pulmonary smooth muscle cells, increased intracellular cGMP concentrations lead to vasodilatation, making sGC-stimulators potentially useful for the t...

Despite systematic screening and improved treatment strategies, the prognosis remains worse in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) compared to idiopathic/hereditary (IPAH). We aimed investigate differences clinical characteristics, outcome, performance of ESC/ERS risk stratification tool these patient groups. This retrospective analysis i...