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Shinohara Hiromi - - 2010
The presence of severe pulmonary arterial hypertension (PAH) is a significant risk factor of major perioperative cardiovascular complications in patients undergoing even non-cardiac surgery under anesthetic management. The most important aspect of perioperative care of PAH patients is to avoid pulmonary hypertensive crisis, which can be induced by alveolar hypoxia, ...
Tonelli Adriano R - - 2010
Limited information is available about the prevalence of pulmonary hypertension diagnosed by right heart catheterization (RHC) in patients with cystic fibrosis being evaluated for lung transplantation. It is unclear whether there are factors that can predict the presence of pulmonary hypertension and whether the presence of pulmonary hypertension influences patient ...
Escribano Subias Pilar - - 2010
Recently, our view of pulmonary hypertension has been changed by the significant progress made in understanding the pathobiology, epidemiology and prognosis of the disease. The increasing number of different conditions now associated with pulmonary hypertension and the appearance of new diagnostic techniques have led to a need for a systematic ...
Mattace-Raso Francesco U S - - 2010
OBJECTIVES: Previous cross-sectional studies found an association between inflammatory markers and measures of arterial stiffness. Recently, some studies investigated whether patients with genotypes associated with high levels of circulating C-reactive protein had high arterial stiffness. These studies reported an association between C-reactive protein levels and measures of arterial stiffness, but ...
Burt Christiana - - 2010
The last 10 years have seen significant advances in the understanding of the pathophysiology and treatment of pulmonary arterial hypertension (PAH). This has included new insights into the genetics, cell-signalling pathways and pathological changes seen in the small pulmonary arteries as well as the introduction of new treatments which have ...
Peacock A - - 2010
Supraventricular tachycardia (SVT) is a rare but important complication of pulmonary arterial hypertension. beta-Adrenoceptor blocking agents are commonly used to treat SVT, but they are potentially dangerous in the context of pulmonary arterial hypertension. Their negative inotropic and chronotropic effects are poorly tolerated in this condition, where cardiac reserve is ...
Firth Amy L - - 2010
Despite improved understanding of the pathobiology of pulmonary arterial hypertension (PAH), it remains a severe and progressive disease, usually culminating in right heart failure, significant morbidity and early mortality. Over the last decade, some major advances have led to substantial improvements in the management of PAH. Much of this progress ...
O'Callaghan Dermot S - - 2010
The last decade has witnessed a remarkable increase in the number of effective treatment options available for the management of patients with pulmonary arterial hypertension. In this regard, agents belonging to the therapeutic classes that specifically target the prostacyclin, endothelin and nitric oxide pathways have shown the greatest efficacy in ...
Dhillon Sonu - - 2010
The interferons are a complex group of virally induced proteins produced by activated macrophages and lymphocytes, which have become the mainstay of therapy for hepatitis C infection. Sustained viral response (SVR) rates in noncirrhotic patients vary from 40-80% with interferon-based therapy. This, along with transplantation, has drastically changed the course ...
Srivastava Mukta C - - 2010
We report the case of a treatment-naive patient with pulmonary arterial hypertension who presented with decompensated right ventricular failure and cardiogenic shock. Unstable hemodynamics, hypoxia and end-organ hypoperfusion limited up-titration of pharmacotherapy. Mechanical circulatory support with veno-venous extracorporeal membrane oxygenation (VV-ECMO) was initiated to permit dose titration of pulmonary vasodilator ...
Alghamdi Mohammed H - - 2010
Both primary pulmonary artery hypertension (PPAH) and autoimmune polyendocrine syndrome (APS) are rare disorders in children. We report a boy who was diagnosed with severe PPAH at 12 years of age. He was treated with prostacyclin for 6 years, briefly with adjunct bosentan, and eventually sildenafil was added. Six years ...
Gu Song - - 2010
BACKGROUND: Pulmonary endarterectomy is safe and effective surgical treatment for chronic thromboembolic pulmonary hypertension. This study aimed to evaluate the efficacy of pulmonary endarterectomy in treatment of thromboembolic pulmonary hypertension. METHODS: A retrospective study of 15 patients who underwent pulmonary endarterectomy in Beijing Chaoyang Hospital was performed. Obvious pulmonary hypertension ...
Melnick Laura - - 2010
Before 2001, intravenous epoprostenol was the only approved drug for patients with idiopathic pulmonary arterial hypertension (IPAH) or familial pulmonary arterial hypertension (FPAH) who were nonresponsive to high-dose calcium channel blockade. The investigators report transitioning select pediatric patients with IPAH or FPAH from intravenous epoprostenol to oral and/or inhaled agents ...
Dewachter Laurence - - 2010
IMPORTANCE OF THE FIELD: Treatments of pulmonary arterial hypertension (PAH) that have so far proven efficacious are all based on the restoration of endothelium control of pulmonary vascular tone and structure, by administration of prostacyclins, endothelin receptor antagonists and phosphodiesterase-5 inhibitors. However, results remain unsatisfactory, with persistent high mortality, insufficient ...
Shin Jordan T - - 2010
When pulmonary hypertension (PH) and right ventricular dysfunction accompany heart failure, the impact on functional capacity and prognosis are ominous. Newer clinical strategies to preferentially lower pulmonary pressures and pulmonary vascular tone improve functional performance and symptoms of heart failure by targeting the nitric oxide signal transduction pathways, as with ...
Said S I - - 2010
Although largely distinct and seemingly unrelated, asthma and pulmonary arterial hypertension (PAH) have important pathological features in common, including inflammation, smooth muscle contraction and remodelling. We hypothesised that these common features could be explained by one shared mechanism of pathogenesis: activation of the transcription factor NFAT (nuclear factor of activated ...
Mathier Michael A - - 2010
Pulmonary arterial hypertension (PAH) is a morbid condition with high mortality if left untreated. Bosentan is an effective treatment option for group 1 pulmonary arterial hypertension. Bosentan improves exercise tolerance and functional class and delays the time to clinical worsening in these patients. Investigation is ongoing to determine its efficacy ...
Vuletin Jose F JF Division of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, - - 2010
This study aim to assess the potential of prenatal predictors of postnatal severe pulmonary artery hypertension (PAH) in isolated left congenital diaphragmatic hernia (CDH) and to define a new prenatal pulmonary hypertension index (PPHI). A retrospective chart review of CDH patients between May 2005 and October 2008 was conducted. Ten ...
Xu Qi - - 2010
Hypertension and aging are associated with large artery structural remodeling and stiffening, which are known to increase cardiovascular risk. Relaxin is a peptide hormone with potent antifibrotic action in multiple organs. Although relaxin is able to reduce peripheral vascular resistance and improve arterial compliance in rats, it remains unclear whether ...
Huang Jing-bin - - 2010
Advanced pulmonary arterial hypertension is characterized by extensive vascular remodeling that is usually resistant to vasodilator therapy. As the major component of the vascular media, decreased apoptosis of pulmonary arterial smooth muscle cell (PASMC) plays key roles during pulmonary vascular remodeling. Recent studies showed that enhancement of apoptosis of PASMC ...
Abman Steven H - - 2010
Pulmonary arterial hypertension in children contributes significantly to morbidity and mortality in diverse pediatric cardiac, lung, hematologic, and other diseases. Pulmonary arterial hypertension is generally a disease of small pulmonary arteries characterized by vascular narrowing due to high-tone and abnormal vasoreactivity, structural remodeling of the vessel wall, intraluminal obstruction, and ...
Stenmark Kurt R - - 2010
Current treatment of pulmonary arterial hypertension, which includes the use of prostacyclins, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors, either alone or in combination, often leads to improvements in functional capacity and modest decreases in pulmonary artery pressure. Disappointingly, however, two recent meta-analysis reviewing the controlled trials in pulmonary ...
Kim N H - - 2010
Although pulmonary endarterectomy remains the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (CTEPH), not all patients will benefit from or receive this highly specialised surgery. Patients whose CTEPH is deemed inoperable by an experienced centre and patients with persistent pulmonary hypertension after surgery are candidates for trial ...
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 ...
Todd Nevins W - - 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 ...
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 ...
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 - - 2010
RATIONALE: 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. OBJECTIVES: Ascertain whether fibrin resistance to lysis occurs in ...
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 ...
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