Combination therapy has been recommended for the treatment of pulmonary arterial hypertension (PAH). However, there is scant information on combination therapy after failure of monotherapy, particularly in patients with scleroderma-associated PAH (PAH-SSD). From a group of 82 consecutive patients with PAH who received initial bosentan monotherapy, a total of 13 idiopathic PAH (IPAH) and 12 PAH-...

Increasing numbers of experimental investigations and recently also of clinical trials strongly suggest an integral involvement of the endothelin (ET) system in the pathophysiology of a variety of disease states, mainly of the cardiovascular system.Ambrisentan (LU 208075)approved by the US Food and Drug Administration in 2007, a selective ETA-receptor antagonist, is an orally active diphenyl pr...

conclusions we observed acceptable results regarding both efficacy and safety with 62.5 mg of bosentan, twice daily in this group of patients. further clinical trials investigating pah with lower dosages of bosentan may be warranted. results no adverse drug reaction was observed during the follow-up. clinical worsening occurred in six (14%) patients, at least one year after treatment, two of th...

Pulmonary arterial hypertension (PAH) is a progressive disease with high morbidity and mortality rates. Research has shown that PAH has a prevalence rate of 10-79% in thalassemia major patients. This cross-sectional study was carried out in 2014 to determine the prevalence and risk factors of PAH in all thalassemia major patients of over 18 years of age in Ilam, Iran. A cardiologist measured sy...

Pulmonary arterial hypertension (PAH) is a devastating disease mediated by vasoconstriction and vascular remodeling of the pulmonary vasculature. Current therapies target imbalance vasoconstrictors vasorelaxants in 3 pathways: nitric oxide, prostacyclin, endothelin. While these have extended lifespans for PAH patients, significant morbidity mortality remains. Notably, progress therapy over deca...

BACKGROUND Inhaled treprostinil was recently developed for the treatment of pulmonary arterial hypertension (PAH). We investigated the safety and acute haemodynamic effects of the combination oral sildenafil and inhaled treprostinil in an open label study in patients with precapillary pulmonary hypertension. METHODS AND PATIENTS Inhaled nitric oxide (20ppm; n=50), sildenafil (50mg; n=50) and ...

now-a-days truncus arteriosus has been known as “common arterial trunk” (cat) and is an uncommon congenital cardiac defect presenting in about 1-3% congenital heart disease. environmental and genetic factors effects on incidence of cat and other conotruncal anomalies. the majority patients with cat and 22q11 deletion have other anomalies such as hypoplasia or aplasia of the thymus or parath...

Severe forms of pulmonary arterial hypertension (PAH) are characterized by various degrees of remodeling of the pulmonary arterial vessels, which increases the pulmonary vascular resistance and right ventricular afterload, thus contributing to the development of right ventricle dysfunction and failure. Recent years have seen advances in the understanding of the pathobiology of PAH; however, man...

Pulmonary hypertension (PH) is a haemodynamic and pathophysiological condition defined as increase in mean pulmonary arterial pressure ≥ 25 mmHg at rest as assessed by right heart catheterization (RHC). It can be due to a primary elevation of pressure in the pulmonary arterial system alone (pulmonary arterial hypertension), or secondary to elevations of pressure in the pulmonary venous and capi...

INTRODUCTION Pulmonary arterial hypertension (PAH), defined as a systolic pulmonary artery pressure above 35 mm Hg, is another vascular disease entity recently described in patients receiving hemodialysis. It is a major problem due to its high prevalence and morbidity and mortality. Its pathophysiological mechanism is just known and the strategies for its supported not yet defined. AIMS To de...