نتایج جستجو برای: idiopathic pulmonary arterial hypertension ipah

تعداد نتایج: 480229  

2011
Maria J Overbeek Anco Boonstra Alexandre E Voskuyl Madelon C Vonk Anton Vonk-Noordegraaf Maria PA van Berkel Wolter J Mooi Ben AC Dijkmans Laurens S Hondema Egbert F Smit Katrien Grünberg

INTRODUCTION Systemic sclerosis (SSc) complicated by pulmonary arterial hypertension (PAH) carries a poor prognosis, despite pulmonary vascular dilating therapy. Platelet-derived growth factor receptor-β (PDGFR-β) and epidermal growth factor receptor (EGFR) are potential therapeutic targets for PAH because of their proliferative effects on vessel remodelling. To explore their role in SScPAH, we...

2016
Mieke M. P. Driessen Wei Hui Bart H. Bijnens Andreea Dragulescu Luc Mertens Folkert J. Meijboom Mark K. Friedberg

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...

Journal: :European Heart Journal 2022

Abstract Background One of the most challenging differential diagnoses in pulmonary hypertension clinical practice, is discrimination between idiopathic arterial (IPAH) and due to heart failure with preserved ejection fraction (PH-HFpEF). Aim We elaborate a score (considering patient history, demographics, echocardiographic characteristics) that can predict, noninvasively, PH-HFpEF vs IPAH diag...

Journal: :Journal of the American College of Cardiology 2016
Christian F Opitz Marius M Hoeper J Simon R Gibbs Harald Kaemmerer Joanna Pepke-Zaba J Gerry Coghlan Laura Scelsi Michele D'Alto Karen M Olsson Silvia Ulrich Werner Scholtz Uwe Schulz Ekkehard Grünig Carmine D Vizza Gerd Staehler Leonhard Bruch Doerte Huscher David Pittrow Stephan Rosenkranz

BACKGROUND Pulmonary hypertension (PH) is hemodynamically classified as pre-capillary (as seen in idiopathic pulmonary arterial hypertension [IPAH]) or post-capillary (as seen in heart failure with preserved ejection fraction [HFpEF]). Overlaps between these conditions exist. Some patients present with risk factors for left heart disease but pre-capillary PH, whereas patients with HFpEF may hav...

2008
Sujana Katta Shivani Vadapalli B. K. S. Sastry Pratibha Nallari

AIM The aim of the present study was to identify the possible genotypic association of 3'UTR Hind III polymorphism of Plasminogen activator Inhibitor-1 (PAI-1) gene with idiopathic pulmonary arterial hypertension (IPAH). BACKGROUND IPAH is a disorder with abnormally raised mean pulmonary arterial pressure and increase in the resistance to blood flow in pulmonary artery. One of the pathologica...

2015
Marius M. Hoeper Juergen Behr Matthias Held Ekkehard Grunig C. Dario Vizza Anton Vonk-Noordegraaf Tobias J. Lange Martin Claussen Christian Grohé Hans Klose Karen M. Olsson Thomas Zelniker Claus Neurohr Oliver Distler Hubert Wirtz Christian Opitz Doerte Huscher David Pittrow J. Simon R. Gibbs Masataka Kuwana

BACKGROUND Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies. METHODS We ...

Journal: :Circulation research 2006
Krystyna Teichert-Kuliszewska Michael J B Kutryk Michael A Kuliszewski Golnaz Karoubi David W Courtman Liana Zucco John Granton Duncan J Stewart

Mutations in the bone morphogenetic protein (BMP) receptor-2 (BMPR2) have been found in patients with idiopathic pulmonary arterial hypertension (IPAH); however, the mechanistic link between loss of BMPR2 signaling and the development of pulmonary arterial hypertension is unclear. We hypothesized that, contrary to smooth muscle cells, this pathway promotes survival in pulmonary artery endotheli...

2012
Wei Fang Lan Zhao Chang-ming Xiong Xin-hai Ni Zuo-xiang He Jian-guo He Martin R. Wilkins

This study measured glucose uptake in the right ventricle (RV) of patients with pulmonary hypertension and investigated the relationship to hemodynamics and survival. Myocardial 18F-fluorodeoxy-glucose (FDG) uptake was measured using single-photon positron emission tomography (SPECT) in 24 patients with idiopathic pulmonary arterial hypertension (IPAH) and 43 patients with congenital heart dise...

Journal: :Respiratory Research 2006
Alasdair J Edgar Matilde R Chacón Anne E Bishop Magdi H Yacoub Julia M Polak

BACKGROUND To elucidate further the pathogenesis of sporadic, idiopathic pulmonary arterial hypertension (IPAH) and identify potential therapeutic avenues, differential gene expression in IPAH was examined by suppression subtractive hybridisation (SSH). METHODS Peripheral lung samples were obtained immediately after removal from patients undergoing lung transplant for IPAH without familial di...

2015
Diana Zabini Slaven Crnkovic Hui Xu Maria Tscherner Bahil Ghanim Walter Klepetko Andrea Olschewski Grazyna Kwapiszewska Leigh M Marsh

Extracellular high-mobility group box-1 (HMGB1) acts as a signalling molecule during inflammation, cell differentiation and angiogenesis. Increased abundance of HMGB1 is associated with several pathological disorders such as cancer, asthma and chronic obstructive pulmonary disease (COPD). In this study, we investigated the relevance of HMGB1 in the pathological remodelling present in patients w...

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