introduction: pulmonary arterial hypertension (pah) is a late progressive sclerodermarelated complication, which can lead to right heart failure and cor pulmonale. given that cardiac catheterization is a diagnostic method of choice for pah, and considering the high risks of this method, the purpose of this study was to evaluate the relationship between serum pro‐brain natriuretic peptide (pro‐b...

Pulmonary hypertension can be classified into 4 categories: pulmonary arterial hypertension (PAH), pulmonary venous hypertension, pulmonary hypertension associated with hypoxemia, and pulmonary hypertension due to chronic thrombotic or embolic disease. PAH is a progressive and often fatal condition that predominantly affects women. Approximately 10% of patients diagnosed with PAH without a demo...

Pulmonary arterial hypertension (PAH) is a severe and fatal disease with a prevalence of 15 cases in a million[1] and characterized by increased pulmonary vascular resistance and right heart failure. PAH can be idiopathic (IPAH), familial (FPAH), or associated with other disorders. In 1984 the National Institute of Health (NIH) compiled the first large registry of PAH patients (all IPAH, FPAH, ...

There is limited data regarding the feasibility of transitioning from intravenous prostacyclins to selexipag in pulmonary arterial hypertension (PAH) patients. We present a case series successful transitions majority carefully selected five stable PAH patients using standardized protocol outpatient setting.

This statement of clinical guidelines, although at times subjective in its ratings of evidence, is valuable as an overview of the field of pulmonary hypertension and comprehensive in its review of the extant literature. Sections include diagnosis and management of pulmonary arterial hypertension (PAH); methodology and grading for pulmonary hypertension evidence review and guideline development;...

Prevalence of pulmonary arterial hypertension (PAH) was studied by echocardiography and Doppler in 43 splenectomized patients with various disorders 1-20 years after splenectomy. Pulmonary arterial hypertension was detected only in thalassemia major, intermedia, hereditary sphereocytosis and myelofibrosis groups comprising a total of 21 patients. Six patients out of 21 was found to have PAH wit...

Connective Tissue Disease (CTD) is an autoimmune disease involving connective tissues, including sjogren syndrome (pSS), rheumatoid arthritis (RA), systemic sclerosis (SSc), mixed tissue (MCTD), lupus erythematosus (SLE), etc. [1,2]. Pulmonary Arterial Hypertension (PAH) refers to a pathological condition in which the pulmonary artery pressure rises above certain threshold, causing right heart ...

Alterations of the nitric oxide receptor, soluble guanylate cyclase (sGC) may contribute to the pathophysiology of pulmonary arterial hypertension (PAH). In the present study, the expression of sGC in explanted lung tissue of PAH patients was studied and the effects of the sGC stimulator BAY 63-2521 on enzyme activity, and haemodynamics and vascular remodelling were investigated in two independ...

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension that shares some clinical and haemodynamic features with idiopathic arterial (PAH). However, suspicion PVOD crucial, considering PAH-specific treatment may precipitate life-threatening oedema lung transplant should be considered from diagnosis [1, 2]. Cardiopulmonary exercise tests provide additional data for the n...

onnective tissue disease (CTD)-associated pulmonary arterial hypertension (CPAH) is the second most prevalent type of pulmonary arterial hypertension (PAH) after idiopathic PAH (IPAH). CPAH is estimated to account for 30% of all adult cases of PAH. Approximately 15% of mixed CTD (MCTD), 10% of systemic sclerosis (SSc), and several percent of systemic lupus erythematosus (SLE) patients develop P...