Alterations in extracellular matrix (ECM) have been implicated in the pathophysiology of pulmonary hypertension. Here, we have undertaken a compartment-specific study to elucidate the expression profile of collagens and their processing enzymes in donor and idiopathic pulmonary arterial hypertension (IPAH) pulmonary arteries. Predominant intimal, but also medial and perivascular, remodeling and...

The behavior of Shigella flexneri ipaH mutants was studied in human monocyte-derived macrophages (HMDM), in 1-day-old human monocytes, and in J774 mouse macrophage cell line. In HMDM, strain pWR700, an ipaH(7.8) deletion mutant of S. flexneri 2a strain 2457T, behaved like the wild-type strain 2457T. This strain caused rapid host cell death by oncosis, and few bacterial CFU were recovered after ...

AIMS To determine the diagnostic utility of circulating angiopoietin-1 (Ang-1) and its antagonist angiopoietin-2 (Ang-2) as potential biomarkers of disease severity or response to treatment in idiopathic pulmonary arterial hypertension (IPAH). Imbalances in angiogenic factors including vascular endothelial cell growth factor (VEGF) and the angiopoetin-Tie2 receptor system have been implicated i...

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...

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...

We present a case of a 36-year-old woman who developed a severe form of Idiopathic Pulmonary Arterial Hypertension (IPAH) during pregnancy and after emergency delivery. The management of IPAH during or after pregnancy is complex. Due to the severity of her IPAH, an upfront triple combination therapy, including i.v. epoprostenol, was started. The rapid institution of this treatment regimen allow...

Objective. Systemic sclerosis-associated pulmonary arterial hypertension (SScPAH) has a disturbed function of the right ventricle (RV) when compared to idiopathic PAH (IPAH). Systemic sclerosis may also affect the heart. We hypothesize that RV differences may occur at the level of interstitial inflammation and-fibrosis and compared inflammatory cell infiltrate and fibrosis between the RV of SSc...

BACKGROUND Systemic sclerosis–associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis–related pulmonary hypertension with interstitial lung disease imposes a greater pulmo...

Oxidative stress may play a role in the pathogenesis of idiopathic pulmonary arterial hypertension (IPAH) [1–4]. A major contributor to oxidative stress is the endothelium-bound enzyme xanthine oxidase (XO), which is involved in the generation of superoxide anions and peroxynitrite [5]. These reactive oxygen species have been implicated in endothelial dysfunction and vascular damage [5]. In viv...

BACKGROUND A majority of patients with idiopathic pulmonary arterial hypertension (IPAH) display persistent exercise intolerance despite new specific therapies. Whether patients with IPAH exhibit peripheral muscle dysfunction that may contribute to this limitation remains unknown. The hypothesis that the muscles of patients with IPAH are weaker and display morphological changes compared with th...