BACKGROUND Idiopathic pulmonary arterial hypertension (IPAH) is a progressive and still incurable disease. Research of IPAH-pathogenesis is complicated by the lack of a direct access to the involved tissue, the human pulmonary vasculature. Various auto-antibodies have been described in the blood of patients with IPAH. The purpose of the present work was therefore to comparatively analyze periph...

Pulmonary vascular remodeling due to overgrowth of pulmonary artery smooth muscle cells (PASMC) is a major cause for the elevated vascular resistance in patients with idiopathic pulmonary arterial hypertension (IPAH). Increased cytosolic Ca ([Ca]cyt), resulting from enhanced capacitative Ca entry (CCE) and upregulated transient receptor potential (TRP) channel expression, is involved in stimula...

Right ventricular (RV) pressure overload has a vastly different clinical course in children with idiopathic pulmonary arterial hypertension (iPAH) than in children with pulmonary stenosis (PS). While RV function is well recognized as a key prognostic factor in iPAH, adverse ventricular-ventricular interactions and LV dysfunction are less well characterized and the pathophysiology is incompletel...

A subgroup of patients with idiopathic pulmonary arterial hypertension (IPAH) has severely reduced diffusing capacity of the lung for carbon monoxide (DLCO) and poor prognosis. Their characteristics are currently unknown. The aim of this study is to contrast clinical characteristics and treatment responses of IPAH-patients with a severely reduced and more preserved DLCO. Retrospectively, 166 IP...

Vascular remodeling in idiopathic pulmonary arterial hypertension (IPAH) involves hyperproliferative and apoptosis-resistant pulmonary artery endothelial cells. In this study, we evaluated the relative contribution of bone marrow-derived proangiogenic precursors and tissue-resident endothelial progenitors to vascular remodeling in IPAH. Levels of circulating CD34+ CD133+ bone marrow-derived pro...

Survival rates for patients with idiopathic pulmonary arterial hypertension (IPAH) have improved with the introduction of PAH-specific therapies. However, the time between patient-reported onset of symptoms and a definitive diagnosis of IPAH is consistently delayed. We conducted a retrospective, multi-center, descriptive investigation in order to (a) understand what factors contribute to persis...

Idiopathic pulmonary arterial hypertension (IPAH) is a fatal disease with a poor prognosis and the molecular pathways underlying the pathogenesis of IPAH are not fully understood. In the present study, the long non-coding RNA (lncRNA) and mRNA expression profiles of lymphocytes obtained from 12 IPAH patients and 12 healthy controls were analyzed using Arraystar Human lncRNA Microarray v2.0, and...

Patient Cause of PH Age (years) Gender mPAP (mm Hg) RAP (mm Hg) CO (L.min) TPR (dyne.sec.cm) 1 IPAH 27 F 55 13 2,9 1733 2 IPAH 56 M (64) (15) (5.6) (923) 3 IPAH 42 M 30 NA 9,2 264 4 EAA 44 M 33 22 6,2 422 5 COPD 59 M 30 15 7,2 330 6 sarcoidosis 44 M 48 11 NA NA 7 IPAH 41 F (60) (13) (3.2) (1481) 8 IPAH 37 F 45 14 5,4 662 9 IPF 64 M 51 8 5,1 805 10 EAA 52 M 43 11 4,5 764 11 PAH-CHD 45 V (71) (14...

BACKGROUND Acute pulmonary vasoreactivity testing has been recommended in the diagnostic work-up of patients with idiopathic pulmonary arterial hypertension (IPAH). Pulmonary arteriolar capacitance (Cp) approximated by stroke volume divided by pulmonary pulse pressure (SV/PP) is considered as an independent predictor of mortality in patients with IPAH. OBJECTIVES We sought to evaluate any dif...

OBJECTIVE To review the current pharmacotherapy for idiopathic pulmonary arterial hypertension (IPAH). METHODS A search of the primary literature was conducted by using MEDLINE, the National Institutes of Health medical research Web site (www.clinicaltrials.gov), and the United States Food and Drug Administration's Center for Drug Evaluation and Research Web site (www.fda.gov/cder). RESULTS...