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Results 201 - 250 of 1655
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Koumakis Eugénie - - 2010
We present the unusual cases of 2 systemic sclerosis patients with a history of liver transplant, who developed pulmonary hypertension in the course of their diseases. Sildenafil was the preferred pulmonary arterial hypertension drug because of its safety within this context. Clinical and functional responses were good, with a follow-up ...
Kulik Thomas J - - 2010
Acute pulmonary vasoconstriction occurs in a variety of clinical settings relevant for the cardiac intensivist, postoperative pulmonary hypertension being perhaps the most common. Although we know that significant postoperative pulmonary vasoconstriction generally occurs in patients with a pathologically remodeled pulmonary circulation, we know little of its pathophysiology. The following review ...
Humbert M - - 2010
Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2), HIV infection, congenital heart disease, connective tissue ...
Huscher Doerte - - 2010
OBJECTIVES: Pulmonary arterial hypertension in patients with systemic sclerosis is a disease involving multiple organ systems. We investigated the differences in perceptions of how to measure PAH-SSc among cardiologists, pulmonologists and rheumatologists. We also examined how a Delphi exercise can improve agreement among these subspecialties. METHODS: The outcome measures derived ...
Fraisse Alain - - 2010
Pharmacologic strategies to reduce pulmonary vascular tone and to treat pulmonary hypertension originally aimed to enrich vascular smooth muscle cyclic adenosine monophosphate levels. Alternatively, increasing cyclic guanosine monophosphate (cGMP) also reduces pulmonary vascular tone. Inhaled nitric oxide is extremely efficacious in increasing cGMP and selectively reducing mean pulmonary arterial pressure ...
Mayer E - - 2010
Patients with chronic thromboembolic pulmonary hypertension (CTEPH) have fibrous obstructions in their proximal pulmonary arteries, as well as a variable degree of secondary vascular remodelling of distal, surgically inaccessible, small pulmonary vessels. Pulmonary endarterectomy (PEA) is currently the only potentially curative treatment option for CTEPH, although it is not known ...
Nieves Jo Ann - - 2010
Pulmonary hypertension is a potentially lethal condition that may be encountered during the entire life span of patients with many forms of congenital or acquired heart disease, pulmonary disorders, and other diseases. Each pulmonary hypertensive patient requires anticipatory interventions geared to prevent severe exacerbations of the pulmonary hypertensive condition, promote ...
Taylor Mary B - - 2010
In the last several years, there have been numerous advancements in the field of pulmonary hypertension as a whole, but there have been few changes in the management of children with pulmonary hypertension after cardiac surgery. Patients at particular risk for postoperative pulmonary hypertension can be identified preoperatively based on ...
Girgis Reda E - - 2010
IMPORTANCE OF THE FIELD: Pulmonary arterial hypertension (PAH) is a clinical syndrome characterized by structural narrowing of the small pulmonary arteries that often culminates in fatal right heart failure. AREAS COVERED IN THIS REVIEW: PubMed was searched for PAH and treatment. Data from scientific meetings and pharmaceutical websites are also ...
Haworth Sheila G - - 2010
The introduction of new medicines to treat pulmonary vascular disease has renewed interest in the management of children with idiopathic pulmonary arterial hypertension. We now have a practical classification of pulmonary hypertension, improved diagnostic techniques, and internationally recognized diagnostic and management guidelines. In 2009 the UK Pulmonary Hypertension Service for ...
Le Pavec Jérôme - - 2010
Pulmonary arterial hypertension (PAH) is a devastating vascular complication of a number of connective tissue diseases, including systemic sclerosis (SSc), where it has a dramatic impact on the clinical course and overall survival and is the single most common cause of death in patients afflicted with this syndrome. Although remarkable ...
Goldberg Avram - - 2010
Pulmonary arterial hypertension (PAH) is an entity that is known to complicate connective tissue diseases (CTD). PAH in CTD is a very important diagnosis which greatly affects treatment and prognosis. The most commonly affected CTD is scleroderma, although lupus, inflammatory myopathies such as poly and dermatomyositis, and mixed CTD are ...
Stakos Dimitrios A - - 2010
Studies have suggested that collagen accumulation in the aortic wall may contribute to the stiff aorta in arterial hypertension. However, data in human hypertension are limited. In this investigation, relations between markers of collagen metabolism and aortic function in patients with arterial hypertension were evaluated. We studied 72 hypertensive patients ...
Suesaowalak Monnipa - - 2010
BACKGROUND: This article aims to review recent advances in the diagnosis and treatment of pulmonary arterial hypertension in neonates and children with congenital heart disease. DATA SOURCES: Articles on pulmonary arterial hypertension in congenital heart disease were retrieved from PubMed and MEDLINE published after 1958. RESULTS: A diagnosis of primary ...
Singh T P - - 2010
Severe pulmonary hypertension is a debilitating disease with short life expectancy that often affects young people. It is usually idiopathic pulmonary artery hypertension and is characterized by progressive elevation of pulmonary artery pressure and vascular resistance, right ventricular failure and death with a limited median survival time. There is an ...
Macchia Alejandro - - 2010
BACKGROUND: In a previous meta-analysis on the approved treatments for pulmonary hypertension, we reported that all therapies caused small changes in 6-minute walk distance over a short period, with minimal effects on hemodynamics and no effect on survival. Since that last review, 10 new clinical trials with about 1,500 patients ...
Fraisse Alain - - 2010
Limited data are available describing paediatric pulmonary arterial hypertension. To characterize the epidemiology, management and impact on quality of life and outcome of paediatric pulmonary arterial hypertension, excluding persistent pulmonary hypertension of the newborn and pulmonary arterial hypertension caused by congenital heart disease. In this multicentre study, children with pulmonary ...
Fadini G P - - 2010
Pulmonary hypertension is a progressive and disabling disease with as yet unclear pathogenesis, limited treatment options and poor prognosis. This is why the discovery of new pathogenic mechanisms and therapeutic strategies are needed. Endothelial progenitor cells (EPCs) are bone marrow-derived cells involved in endothelial homeostasis, as well as physiological and ...
Ross Alan F - - 2010
Literature about thoracic surgery in patients with pulmonary hypertension is scarce. Perceived high risk has appropriately discouraged any unnecessary operation. However, the medical therapy for pulmonary hypertension has made great advances during the last decade. It is likely that future advances in survival and possibly the need for diagnostic procedures ...
Todd Nevins W NW Department of Medicine, University of Maryland School of Medicine, Baltimore, Maryland, USA. - - 2010
Pulmonary hypertension may occur in patients with interstitial pneumonia and is associated with increased mortality. We sought to determine the prevalence of pulmonary hypertension in sub-groups of patients with interstitial pneumonia and to investigate possible associations between pulmonary vascular hemodynamics and pulmonary function. The presence or absence of pulmonary hypertension ...
Zheng Changlong - - 2010
Pulmonary arterial hypertension (PAH) is a life-threatening disease that leads to progressive pulmonary hypertension, right heart failure, and death. Endothelial dysfunction and inflammation were implicated in the pathogenesis of PAH. Epoxyeicosatrienoic acids (EETs), products of the cytochrome P450 epoxygenase metabolism of arachidonic acid, are potent vasodilators that possess anti-inflammatory and ...
Oda Hirotaka - - 2010
To prevent side branch occlusion during bifurcational lesion stenting, the placement of a wire in both the main branch and side branch is performed for "side-branch protection". However, this procedure does not always prevent side branch occlusion. A procedure for placing two wires in the side branch, called "two-wire protection ...
Miniati Massimo M Dipartimento di Area Critica Medico Chirugica, Università di Firenze, Viale Morgagni 85, 50134 Florence, Italy. - - 2010
Reportedly, fibrin isolated from patients with chronic thromboembolic pulmonary hypertension (CTEPH) is resistant to lysis. Persistence of regions within the fibrin beta chain, which mediate cell signaling and migration, could trigger the organization of pulmonary thromboemboli into chronic intravascular scars. Ascertain whether fibrin resistance to lysis occurs in patients with ...
Grech Victor - - 2010
Disconnected pulmonary arteries are unusual and may result in pulmonary hypertension with acute right heart failure. We report a case of a three-month-old Asian girl who presented with heart failure and severe pulmonary hypertension due to a disconnected right pulmonary artery. An epoprostenol (prostacyclin) infusion was instrumental in lowering pulmonary ...
Karkoulias K - - 2010
Obstructive Sleep Apnea Syndrome (OSAS) is a recognized risk factor for cardiovascular disorders and in some cases is complicated with Pulmonary Arterial Hypertension (PAH), as the endothelium is affected. Recent studies provide strong evidence for endothelial dysfunction in obstructive sleep apnea. The resultant vasoconstriction, abnormal cell proliferation and hyper-coagulability may ...
Yano Toshiaki - - 2010
A 20-year-old female diagnosed as idiopathic pulmonary arterial hypertension at 7 years of age was referred with worsening dyspnea and chest pain. Several imaging studies and right cardiac catheterization showed multiple stenoses in the peripheral pulmonary arteries with severe pulmonary hypertension and multiple systemic arterial stenoses lacking in systemic hypertension. ...
Gillespie Mark N - - 2010
Decades of studies in animal models and in humans with pulmonary artery hypertension have left little doubt that the processes culminating in hyper-tensive pulmonary vascular remodeling and sustained increases in pulmonary vascular resistance are complex. Modulations in phenotype, proliferative state, and survival of multiple lung vascular cell populations, changes in ...
Scallan Ciaran - - 2010
Arterial stiffness plays a critical role in the function of the cardiovascular system as it represents the coupling of the left ventricle and arterial tree. Increased arterial stiffness is associated with a number of cardiovascular complications. Increased stiffness occurs with age and with the development of chronic conditions (e.g., hypertension) ...
Chhina Mantej K - - 2010
Imatinib mesylate is a small molecule inhibitor that selectively inhibits the PDGF receptor kinase as well the cKIT and Abl kinases, among other targets. Various studies have implicated the PDGF pathway in the pathogenesis of pulmonary arterial hypertension (PAH). Inhibition with imatinib mesylate has shown efficacy in human case reports ...
Ishigaki Kazuyoshi - - 2010
Thrombotic microangiopathic hemolytic anemia (TMHA) caused by antiphospholipid syndrome (APS) is generally associated with renal or neurological complications and plasma exchange is the first line of treatment. We present the case of a 72-year-old woman with APS who had chronic TMHA and pulmonary hypertension without other major complications. TMHA and ...
Hovnanian A - - 2010
Schistosomiasis is one of the most prevalent infectious diseases, endemic in more than 70 countries, mainly within the developing world. More than 200 million people might be infected worldwide; about 20 million of those might develop severe disease. The hepatosplenic form of schistosomiasis is the most prevalent form of chronic ...
Marshall G B - - 2010
Misalignment of pulmonary vessels, with or without alveolar capillary dysplasia, is a rare cause of persistent pulmonary hypertension in the newborn. The prognosis is poor, with virtually all patients succumbing to unremitting hypoxaemic respiratory failure and death during the newborn period. We report the CT and histological findings of misplaced ...
Furuya Yoshiaki - - 2010
Interleukin-6 (IL-6) is a pleiotropic cytokine with a wide range of biologic activities in immune regulation, hematopoiesis, inflammation, and oncogenesis. Recent accumulating evidence indicates a pathologic role for IL-6 in promoting proliferation of both smooth muscle and endothelial cells in the pulmonary arterioles, resulting in development of pulmonary arterial hypertension ...
Yang Zhen - - 2010
Endothelial dysfunction is related to reduced arterial elasticity in patients with essential hypertension. Circulating endothelial progenitor cells (EPCs), an important endogenous repair approach for endothelial injury, is altered in hypertensive patients. However, the association between alteration in circulating EPCs and hypertension-related reduced arterial elasticity has not been reported. The purpose ...
Falk Jeremy A - - 2010
Pulmonary hypertension (PH) is found in a vast array of diseases, with a minority representing pulmonary arterial hypertension (PAH). Idiopathic PAH or PAH in association with other disorders has been associated with poor survival, poor exercise tolerance, progressive symptoms of dyspnea, and decreased quality of life. Left untreated, patients with ...
Umar S - - 2010
Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by an increase in pulmonary artery pressure leading to right ventricular (RV) hypertrophy, RV failure, and ultimately death. Current treatments can improve symptoms and reduce severity of the hemodynamic disorder but gradual deterioration in their condition often necessitates a lung transplant. ...
Saleemi Sarfraz - - 2010
Portopulmoanry hypertension (POPH) is a form of pulmonary arterial hypertension (PAH) associated with portal hypertension with or without underlying chronic liver disease. POPH is increasingly recognized and recent evidence suggests that it is one of the leading causes of PAH. The pathophysiology of POPH is poorly understood although the pathological ...
Schuuring Mark J - - 2010
Pulmonary arterial hypertension (PAH) is a progressive disease with poor survival outcome. PAH is classified by the 2009 updated clinical classification of pulmonary hypertension and a major subgroup is PAH due to congenital heart disease (CHD) with systemic-to-pulmonary shunt. CHD-PAH is a result of systemic-to-pulmonary shunting and chronic increased flow ...
Ruan Cheng-Huai - - 2010
In pulmonary arterial hypertension, the blood vessels that carry blood between the heart and lungs are constricted, making it difficult for the heart to pump blood through the lungs. Prostacyclin, a prostanoid metabolized from endogenous arachidonic acid through the cyclooxygenase (COX) pathway, is a potent vasodilator that has been identified ...
Harrington Louise S - - 2010
Pulmonary vascular diseases are increasingly recognised as important clinical conditions. Pulmonary hypertension associated with a range of aetiologies is difficult to treat and associated with progressive morbidity and mortality. Current therapies for pulmonary hypertension include phosphodiesterase type 5 inhibitors, endothelin receptor antagonists, or prostacyclin mimetics. However, none of these provide ...
Yagi Shusuke - - 2010
Pulmonary arterial hypertension (PAH) is a frequent complication in patients with systemic sclerosis. Bosentan is used in patients with symptomatic PAH; however, it has not been established whether or not bosentan ameliorates the progression of PAH in patients with no PAH-related symptoms. We present a case of systemic sclerosis with ...
Morrell Nicholas W NW Department of Medicine Box 157 Addenbrooke's Hospital, University of Cambridge School of Clinical Medicine Hills Road, Cambridge, CB2 8DP - - 2010
Pulmonary arterial hypertension (PAH) is usually a devastating condition with a poor prognosis. Nearly 10 years ago, the underlying molecular basis of heritable PAH was elucidated with the identification of mutations in the gene encoding the bone morphogenetic protein type II receptor (BMPR-II). This discovery is now beginning to suggest ...
Maclean Margaret R MR Integrative and Systems Biology, Faculty of Biomedical and Life Sciences, University of Glasgow, Glasgow, G12 8QQ, Scotland, UK. - - 2010
The serotonin hypothesis of pulmonary arterial hypertension (PAH) arose after an outbreak of PAH in patients taking the anorexigenic drugs aminorex and dexfenfluramine. Both of these drugs are serotonin transporter (SERT) substrates and indirect serotinergic agonists. There is now a wealth of evidence to support a role for serotonin in ...
Rabinovitch Marlene M The Wall Center for Pulmonary Vascular Diseases, Stanford University School of Medicine, Stanford, CA, USA. - - 2010
Peroxisome proliferator-activated receptor gamma (PPARgamma) is a nuclear receptor that functions as a transcription factor to regulate adipogenesis and metabolism by binding to PPAR response elements (PPAREs) in the promoter region of various target genes. Activation of PPARgamma suppresses smooth muscle cell proliferation and migration. This chapter discusses the potential ...
Toporsian Mourad - - 2010
OBJECTIVE: Loss-of-function mutations in genes coding for transforming growth factor-beta/bone morphogenetic protein receptors and changes in nitric oxide(*) (NO(*)) bioavailability are associated with hereditary hemorrhagic telangiectasia and some forms of pulmonary arterial hypertension. How these abnormalities lead to seemingly disparate pulmonary pathologies remains unknown. Endoglin (Eng), a transforming growth factor-beta ...
Arena Ross - - 2010
BACKGROUND: There is an increasing recognition of the potential value of cardiopulmonary exercise testing (CPX) in patients with pulmonary hypertension (PH). Key CPX characteristics in these patients include: (1) a diminished aerobic capacity; (2) an abnormally elevated minute ventilation-carbon dioxide production relationship; and (3) an abnormally diminished partial pressure of ...
Ito Naofumi - - 2010
A 45-year-old man, who had been diagnosed with primary pulmonary hypertension (PPH) 8 years before, was referred to our hospital because of short breath and lower-limb edema. Findings of chest X-ray, electrocardiogram, and cardiac ultrasound showed right atrial and ventricular dilatation/hypertrophy that were compatible with advanced PPH. Chest enhanced computed ...
Montani D - - 2009
Pulmonary hypertension (PH) comprises a heterogeneous group of disorders characterised by increased pulmonary vascular resistance that results in progressive right ventricular failure. In order to translate current evidence into routine clinical practice, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) have recently jointly proposed evidence-based guidelines ...
Confalonieri Marco - - 2009
Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and underdiagnosed disease characterized by progressive hypertension secondary to organized thrombi in pulmonary vessels. The arteriolar lesions of CTEPH are similar to those seen in idiopathic pulmonary arterial hypertension (PAH). Surgical disobliteration of the vessels by pulmonary endarterectomy is the therapy of ...
Jerath Angela - - 2010
Acute pulmonary hypertension is a severe and life-threatening reaction that rarely occurs secondary to protamine administration. Management of systemic hypotension combined with severe pulmonary hypertension causing right ventricular failure is challenging. We describe a case of acute pulmonary hypertension induced by protamine during elective coronary artery bypass surgery refractory to ...
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