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Sakao Seiichiro - - 2010
Idiopathic pulmonary arterial hypertension (PAH) is a disabling condition characterized by PA vasoconstriction and remodeling as well as in situ thrombosis and eventual right heart failure. Idiopathic PAH occurs more frequently in females than in males. The female:male ratio is 1.64 ∼ 3.88:1. Although endogenous sex hormones including estrogen have ...
Farquhar Michael - - 2010
Pulmonary hypertension is an uncommon but significantly challenging complication of chronic neonatal lung disease [CNLD] as it occurs in the "new bronchopulmonary dysplasia [BPD]". The presence of pulmonary hypertension may be sub-clinical and is often overlooked as it is not considered in all but the more severe cases of children ...
Kluge Stefan - - 2010
Ventriculoatrial (VA) shunts inserted for the treatment of hydrocephalus are known to be a risk factor for pulmonary hypertension. The aim of this study was to evaluate the incidence of pulmonary hypertension among adult patients with VA shunts. All patients who had received a VA shunt at one of two ...
Kobayashi Daisuke - - 2010
We report on an African-American male with Cantu syndrome who required a pericardial window for a significant pericardial effusion in infancy and was subsequently found to have partial pulmonary venous obstruction (PVO) leading to pulmonary hypertension. Measurement of bilateral pulmonary capillary wedge pressures is important to uncover partial PVO.
Humbert Marc - - 2010
BACKGROUND: Novel therapies have recently become available for pulmonary arterial hypertension. We conducted a study to characterize mortality in a multicenter prospective cohort of patients diagnosed with idiopathic, familial, or anorexigen-associated pulmonary arterial hypertension in the modern management era. METHODS AND RESULTS: Between October 2002 and October 2003, 354 consecutive ...
Hui-Li Gan - - 2011
Acute pulmonary arterial hypertension (PAH), which may complicate the course of many complex disorders, is always underdiagnosed and its treatment frequently begins only after serious complications have developed. Acute PAH is distinctive because they differ in their clinical presentation, diagnostic findings, and response to treatment from chronic PAH. The acute ...
Abe Kohtaro - - 2010
BACKGROUND: The plexiform lesion is the hallmark of severe pulmonary arterial hypertension. However, its genesis and hemodynamic effects are largely unknown because of the limited availability of lung tissue samples from patients with pulmonary arterial hypertension and the lack of appropriate animal models. This study investigated whether rats with severe ...
Van Rheen Zachary Z Department of Pediatrics, University of Colorado, Aurora, CO 80045, - - 2011
Interstitial lung disease is a devastating disease in humans that can be further complicated by the development of secondary pulmonary hypertension. Accumulating evidence indicates that the oxidant superoxide can contribute to the pathogenesis of both interstitial lung disease and pulmonary hypertension. We used a model of pulmonary hypertension secondary to ...
Gomberg-Maitland Mardi - - 2010
Pulmonary arterial hypertension (PAH) is a fatal orphan disease. The global epidemiology of PAH is not well known and encourages combined national and international efforts to enhance understanding of the disease. A global database will help unify investigators and patients to foster collaboration and knowledge.
Beghetti Maurice - - 2010
In recent decades, the Fontan procedure and its variations have resulted in markedly improved outcomes of patients with single-ventricle physiology. These improvements are expected to increase greatly the number of surviving patients, particularly those surviving long into adulthood. However, there is still a progressive risk of attrition and failure of ...
Sajkov Dimitar - - 2010
PURPOSE OF REVIEW: Pulmonary hypertension leads to progressive increase in pulmonary vascular resistance, heart failure, and death. Pulmonary arterial hypertension (PAH) is a subset of pulmonary hypertension affecting small pulmonary arteries and not associated with underlying heart or lung disease. Dyspnea and exercise intolerance are hallmarks of PAH and are ...
Galiè Nazzareno - - 2010
Pulmonary hypertension is a heterogeneous hemodynamic and pathophysiological state that is observed in a number of clinical conditions, which have been divided into six diagnostic groups. Although the increase in pulmonary pressure observed in these clinical groups may be similar, underlying disease mechanisms, diagnostic methods, and prognostic and therapeutic consequences ...
Lopes Antonio Augusto - - 2010
Latin America is here defined as all of the Americas south of the United States. In the setting of pulmonary hypertension, there are social inequalities and geophysical aspects in this region that account for a high prevalence of certain etiologies. This review aimed to analyze some of these factors. Data ...
Pritts Chad D - - 2010
PURPOSE OF REVIEW: Patients with pulmonary hypertension who undergo anesthesia and surgery have high morbidity and mortality. Recent advances in our understanding of pulmonary hypertension and its therapy provide an opportunity to improve outcomes. RECENT FINDINGS: Pulmonary hypertension can be classified into several subtypes, each with its own causes, pathophysiology, ...
Adir Y - - 2010
Pulmonary hypertension (PH) is a severe haemodynamic disorder in which the pulmonary artery pressure is persistently elevated, leading to right-sided heart failure and death. Recently, chronic myeloproliferative diseases associated with pulmonary hypertension were included in the group 5 category, corresponding to PH for which the aetiology is unclear and/or multifactorial. ...
Machado Roberto F - - 2010
The inherited hemoglobin disorders sickle cell disease and thalassemia are the most common monogenetic disorders worldwide. Pulmonary hypertension is one of the leading causes of morbidity and mortality in adult patients with sickle cell disease and thalassemia, and hemolytic disorders are potentially among the most common causes of pulmonary hypertension. ...
Vlachopoulos Charalambos - - 2011
Serum uric acid (UA) plays a key role in the development and progression of hypertension. We investigated the association of UA levels and indices of arterial function in a cohort of newly diagnosed, never-treated hypertensive subjects. One thousand two hundred and twenty-five patients with a new diagnosis of mild to ...
Johri Shilpa - - 2010
We describe the case of an 83-year-old man with a family history of pulmonary hypertension (PH) who presented with severe pulmonary arterial hypertension (PAH) and later tested positive for a novel bone morphogenetic protein receptor 2 (BMPR2) gene mutation. To our knowledge, this may be the oldest reported patient with ...
Macnee William - - 2010
Pulmonary hypertension is a common complication in patients with severe hypoxic COPD, but the elevation in pulmonary arterial pressure (PAP) is usually relatively mild, although its presence indicates a poor prognosis. A minority of patients have severe pulmonary hypertension, whose prognosis is very poor with the development of right heart ...
Krasuski Richard A - - 2011
Anemia is a marker of worsened clinical outcome in patients with heart failure from left ventricular dysfunction. Pulmonary hypertension often results in right ventricular dysfunction. Accordingly we sought to examine the association of hemoglobin levels and long-term all-cause mortality in a cohort of patients with pulmonary hypertension. Baseline demographic information, ...
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 ...
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 ...
Shin Jordan T JT Cardiovascular Research Center, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, 149 13th Street, Charlestown, MA 02129, USA. - - 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 SI Pulmonary and Critical Care Medicine, Stony Brook University, Stony Brook, NY 11794 , USA. - - 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 ...
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 ...
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