conclusions ipah is associated with subclinical hypothyroidism and low patient functional capacity, and is more common in rv failure. results in this research, 84.8% of the participants were women. the mean pap was 51.6 mmhg (31-87) and mean thyroid stimulating hormone (tsh) level was 4.2 miu/ml (0.7 - 10). subclinical hypothyroidism was detected in 26 patients (49.1%). there were significant c...

Idiopathic pulmonary arterial hypertension (IPAH) is a fatal disease characterised by elevated blood pressure in the pulmonary circulation. Initial vasoconstriction, proliferation of pulmonary arterial smooth muscle cells (PASMC) and increased deposition of extracellular matrix (ECM) contribute to pathological remodelling of pulmonary arterioles in IPAH. Glycosaminoglycans (GAGs), components of...

an elevated pulmonary artery pressure (pap) from any cause is associated with increased mortality, especially in cases of primary pulmonary arterial hypertension (pah). one of the many reasons behind a bad prognosis is the inherent difficulty in assessing right ventricular (rv) function with reproducible methods. intensive research involving the left heart has made it relatively easy to assess ...

Idiopathic pulmonary arterial hypertension (IPAH) is a rare, chronically progressive disease associated with high mortality. Despite remarkable achievements in treatment, prognosis remains poor with a survival rate of ,60% 3 years after diagnosis [1]. The clinical course of the disease (short survival and no cure), as well as its pathophysiological aspects, reveals analogies between IPAH and ne...

BACKGROUND Idiopathic pulmonary arterial hypertension (IPAH) is a rare, fatal disease of unknown pathogenesis. Evidence from our recent study suggests that IPAH pathogenesis is related to upregulation of the Wnt/planar cell polarity (Wnt/PCP) pathway. We used microscopic observation and immunohistochemical techniques to identify expression patterns of cascading proteins-namely Wnt-11, dishevell...

conclusions cp could be considered as an index for the prediction of vasoreactivity in patients with ipah. prediction of long-term response to calcium channel blockers in patients with ipah and a positive vasoreactive test by this index should be addressed in further studies. patients and methods fourteen patients with ipah and a vasoreactive adenosine vasoreactivity testing according to the es...

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, ...

Systemic sclerosis-associated pulmonary arterial hypertension (SScPAH) has a worse prognosis and response to pulmonary arterial hypertension (PAH) therapy than idiopathic PAH (IPAH). These differences have not yet been explained. Knowledge concerning histological pulmonary vasculopathy in SScPAH is limited in contrast to IPAH. Therefore, we explored patterns of vasculopathy in SScPAH compared w...