... without demonstrable cause, always leading to right ventricular failure and premature death [ 1 - 3 ]. Since the publication of epidemiologic and survival data for patients with primary pulmonary hypertension in the NIH registry before pulmonary arterial hypertension (PAH)-specific therapies [ 4 , 5...

... Abstract HIV is an independent risk factor for development of pulmonary arterial hypertension (PAH). PAH can complicate the course of HIV infection regardless of the route of HIV transmission, the stage of HIV infection, and the degree of immunosuppression. The clinical presentation...

... Abstract Germline mutations of BMPR2 gene ( bone morphogenetic protein receptor type 2 ), or more rarely of ACVRL1 ( activin A receptor type II-like 1 ), ENG ( endoglin ), and Smad8 genes have been identified in patients displaying pulmonary arterial hypertension (PAH). BMPR2 , ACVRL1...

... Abstract Pulmonary veno-occlusive disease (PVOD) is a rare disorder that can be misdiagnosed as idiopathic pulmonary arterial hypertension (PAH) and accounts for 5–10% of cases initially considered as idiopathic PAH. PVOD and idiopathic PAH share a similar clinical presentation, genetic...

... Abstract Connective tissue diseases (CTD) are commonly complicated by pulmonary arterial hypertension (PAH) which is often the leading cause of death in this population. Despite recent advances in therapy for PAH in general, the response to treatment in patients with CTD, particularly patients...

... Abstract Pulmonary arterial hypertension (PAH) is a progressive disease leading to remodeling of small pulmonary arteries. The exact pathogenesis of PAH is uncertain; however, several risk factors are likely to contribute. These include inflammatory ‘hits’, such as a viral infection...

... Abstract Pulmonary arterial hypertension (PAH) commonly arises in patients with congenital heart diseases (CHD), and is most often related with congenital shunt lesions. These patients initially exhibit a left-to-right (systemic-to-pulmonary) shunt, but as the disease progresses, vascular...

.... Little is known about the possible effects on humans of internalizing these chemicals. Analysis has shown that the production of the main component, carbon black, can lead to the formation of pigments with polycyclic aromatic hydrocarbon (PAH) contents that range from very high to almost completely...

... Abstract In a remarkably short interval of time, pulmonary artery hypertension (PAH) has evolved from a disease of unknown pathogenesis devoid of effective therapy to a condition whose cellular and molecular underpinnings are unfolding, and for which three treatment classes have been developed...

...; precapillary lesions are typically located in muscular arteries <500 µm diameter and arterioles in patients with pulmonary arterial hypertension (PAH), while septal veins and preseptal venules are involved in patients with postcapillary pulmonary veno-occlusive disease (PVOD)/pulmonary capillary...

... arterial pressure (mPAP) ≧25 mm Hg at rest as assessed by right heart catheterization. No definition for PH on exercise was considered. PH was defined as precapillary when pulmonary capillary wedge pressure was ≤15 mm Hg associated with a normal or reduced cardiac output. In Group 1 (PAH), the term...

... Abstract Although medical therapies are now available for patients with pulmonary arterial hypertension (PAH), lung transplantation remains an important therapeutic option, especially for those patients not responding sufficiently to optimized medical therapy. Bilateral lung transplantation...

... Abstract The most significant manifestations of pulmonary arterial hypertension (PAH) are dyspnea, exercise intolerance, and poor quality of life. Mechanisms of exercise intolerance in PAH are clearly multifactorial and involve cardiac, respiratory, and peripheral muscle abnormalities. Exercise...

... (PAH) is a rare disease characterized by a sustained increase in pulmonary vascular resistance and pulmonary arterial pressure resulting in progressive right ventricular dysfunctional and premature death [ 1 ]. In less than 20 years, PAH has been transformed from a disorder characterized by a uniformly...

... to generate a negatively charged surface, determined by electrophoresis as the ζ-potential [ 10 ]. If an aqueous solution of a positively charged polyelectrolyte, such as poly(allylamine hydrochloride) (PAH) (fig. 1 b), is applied to the pigment, the PAH will be adsorbed onto the surface due to electrostatic...

... Abstract Pulmonary arterial hypertension (PAH) is a severe and rapidly progressive condition, ultimately leading to right heart failure and death. Although medical therapy has improved over the past 15 years, PAH remains incurable and many patients will be in need of lung transplantation...