Search Results
Results 251 - 300 of 1648
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Toonkel Rebecca L - - 2010
Pulmonary hypertension, a common manifestation of advanced sarcoidosis, is thought to result from fibrosis with chronic hypoxia and destruction of small vessels, extrinsic compression of pulmonary arteries, or granulomatous vasculitis. We report a case of sarcoidosis-associated pulmonary hypertension due to fibrosing mediastinitis. Our patient presented with cough and dyspnea on ...
Melby Spencer J - - 2009
Aortopulmonary window is a rare abnormal congenital communication between the pulmonary artery and the ascending aorta with intact aortic and pulmonary valves. Because pulmonary hypertension and premature death are the natural history of the uncorrected left-to-right shunt physiology that occurs with aortopulmonary window, surgical correction, which is the gold standard ...
- - 2009
Jennifer Taylor MPhil, talks to Nazzareno Galiè , FESC, FRCP, chairman of the committee for the 2009 guidelines. What's new, changes from previous recommendations, which decisions were difficult, what they mean and should do.
Jensen Kurt W - - 2009
BACKGROUND: The currently recommended treatment for chronic thromboembolic pulmonary hypertension is pulmonary thromboendarterectomy (PTE). No convincing evidence for the use of pulmonary hypertensive medical therapy (PHT) exists in operable candidates. We sought to determine the prevalence of the use of PHT on referral for PTE and the effects on pre-PTE ...
Vachiéry J-L - - 2009
The onset and progression of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) can be particularly aggressive; however, effective treatments are available. Therefore, early identification of patients with suspected PAH, confirmation of diagnosis, and intervention is essential. PAH may be challenging to diagnose in its earliest stages, particularly ...
Hemnes A R - - 2009
Understanding the haemodynamical profile of the right ventricle and pulmonary circulation is critical to not only the initial evaluation of, but also the continued management of pulmonary hypertension. Despite advances in non-invasive imaging techniques, right heart catheterisation (RHC) remains the gold standard for diagnosis of pulmonary hypertension and its various ...
Stepien Rebecca L - - 2009
Pulmonary arterial hypertension is a description of a physiological finding rather than a diagnosis. Pulmonary arterial pressure is the result of interactions among pulmonary blood flow (right ventricular cardiac output), pulmonary vascular impedance and post-capillary pressure (typically reflecting left atrial pressure). When elevations in pulmonary arterial pressure (systolic/diastolic pulmonary arterial ...
Duffels M G J - - 2009
Background. In patients with pulmonary hypertension, it is unknown whether the treatment effect of bosentan is dependent on the duration of pulmonary vessel changes. Therefore, we studied the response to bosentan in patients with life-long pulmonary vessel changes (pulmonary arterial hypertension (PAH) due to congenital heart disease (CHD)) and in ...
Mubarak Kamal K - - 2010
Pulmonary arterial hypertension is a chronic, progressive disease characterized by elevation of pulmonary artery pressure and pulmonary vascular resistance that ultimately results in right ventricular failure and death. Multiple mechanisms are involved in the pathogenesis of pulmonary arterial hypertension, including prostacyclin, endothelin-1, and nitric oxide pathways amongst others. The first ...
Steiner Ivo - - 2010
Pulmonary hypertension is clinically defined as a pulmonary arterial pressure greater than 25 mmHg at rest or greater than 30 mmHg during exercise. We report a unique case of an infant with congenital heart disease causing pulmonary hypertension and necrotizing pulmonary arteritis but affecting only one lung. In conclusion, in ...
Thakare Manisha - - 2013
Adult-onset Still's disease (AOSD) is a rare condition diagnosed by a combination of clinical and laboratory features and after ruling out other conditions. Pulmonary manifestations, apart from pleuritis, are uncommon and pulmonary arterial hypertension (PAH) in extremely uncommon. We have described a case of AOSD with severe PAH. There have ...
Naclerio Caterina - - 2010
This study aimed to investigate the efficacy of bosentan in the treatment of severe pulmonary hypertension in a young female patient with mixed connective tissue disease (MCTD) associated with antiphospholipid syndrome. A 27-year-old woman presented with sudden onset of dyspnea. She had not experienced any dyspnea before this period, and ...
Safi Morteza - - 2009
Compression of left main coronary artery (LMCA) secondary to pulmonary trunk dilatation is a newly recognized entity that has been associated with severe pulmonary hypertension. In this paper we present a case of extrinsic compression of LMCA caused by dilated pulmonary trunk secondary to pulmonary hypertension documented using 64-slice multidetector ...
Selimovic Nedim - - 2009
BACKGROUND: The endothelin-1 (ET-1) system plays a pathophysiologic role in patients with pulmonary arterial hypertension (PAH). Results from previous studies assessing the transpulmonary gradient of ET-1 have been inconsistent. The influence of an intravenous epoprostenol infusion on the transpulmonary ET-1 gradient is unknown. METHODS: In a prospective investigation, serum concentrations ...
Rubenfire Melvyn - - 2009
To our knowledge, there are no specific and validated measures of quality of life (QoL) or degree of disability for pulmonary arterial hypertension (PAH). A review of the literature shows that, with the exception of one recently designed specifically for pulmonary hypertension, QoL questionnaires used in PAH studies are generic ...
Labombarda Fabien - - 2009
Mortality in children with idiopathic pulmonary arterial hypertension is high, emphasizing the need for novel therapeutic approaches. A surgical approach consisting in the creation of anastomosis between descending aorta and left pulmonary artery, the Potts shunt, has been proposed to decompress right ventricle. We reported two cases of severe idiopathic ...
Tuder Rubin M - - 2009
The pathological features of pulmonary arterial hypertension are highlighted in this review, which can serve as a guide to diagnosis and identification of potential cellular targets for novel lines of therapy. It also emphasizes the variability of pathological processes found in pulmonary hypertensive lungs collected by the Pulmonary Breakthrough Initiative ...
Beghetti Maurice - - 2009
Pulmonary hypertension complicates the course of many children and adults with congenital heart diseases (CHDs). The increase in pulmonary pressure associated with CHD is secondary to either increased pulmonary blood flow or increased postcapillary pressures. Pulmonary arterial hypertension is in the vast majority associated with congenital cardiac shunts. Despite major ...
Hoeper Marius M - - 2009
Pulmonary arterial hypertension (PAH) is a distinct subgroup of pulmonary hypertension that comprises idiopathic PAH, familial/heritable forms, and PAH associated with connective tissue disease, congenital heart disease, portal hypertension, human immunodeficiency virus (HIV) infection, and some other conditions. The hemodynamic definition of PAH was recently revised: PAH is now defined ...
Souza Rogério - - 2009
Recent years have witnessed a significant increase in the knowledge about the pathophysiology of pulmonary arterial hypertension (PAH) and the availability of multiple drugs specifically aimed at pulmonary circulation. Although most of this is related to the idiopathic form of PAH, this development has also turned attention to other forms ...
Corris Paul A - - 2009
The development of specific disease-targeted therapy for pulmonary arterial hypertension has offered a major advancement in the management of patients, and these medical treatments have been shown to improve both quality and quantity of life. Nevertheless, many patients deteriorate with time and will require consideration of other approaches. This article ...
Saggar Rajan - - 2009
Pulmonary hypertension (PH) can occur as either a primary or a secondary process, and in general, its presence increases overall morbidity and mortality. Importantly, the majority of prior studies have been in the setting of idiopathic pulmonary arterial hypertension (IPAH); thus the following discussion focuses on IPAH. Because the majority ...
Hassoun Paul M - - 2009
Pulmonary arterial hypertension (PAH) may complicate diverse connective tissue diseases (CTDs) such as systemic sclerosis (SSc), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and mixed CTD (MCTD) and is an important cause of morbidity and mortality in this context. From a histological standpoint, the pulmonary vascular lesions in PAH complicating ...
Thachil J - - 2009
Pulmonary hypertension is a common and debilitating condition which is increasingly being recognized in haematological patients. This is particularly so in haemolytic diseases like thalassaemia and myeloproliferative conditions like idiopathic myelofibrosis. Recently, splenectomy, which may be required for some of these conditions, have been linked to this complication although the ...
Csiszar Anna A Reynolds Oklahoma Center on Aging, Department of Geriatric Medicine, University of Oklahoma Health Science Center, Oklahoma City, OK 73104, - - 2009
Proliferation of pulmonary arterial smooth muscle cells, endothelial dysfunction, oxidative stress, and inflammation promotes the development of pulmonary hypertension. Resveratrol is a polyphenolic compound that exerts antioxidant and anti-inflammatory protective effects in the systemic circulation, but its effects on pulmonary arteries remain poorly defined. The present study was undertaken to ...
Pawar Ravindra - - 2009
We report the use of Bosentan in the post-operative period of a neonate with obstructed infradiaphragmatic total anomalous pulmonary venous connection and severe pulmonary arterial hypertension. To our knowledge, this is the first report of use of Bosentan in this situation.
McLaughlin Vallerie V - - 2009
New and emerging therapies might provide benefit in patients with pulmonary arterial hypertension. Their efficacy and safety will be compared with existing combination therapies in randomized clinical trials. Appropriate end points for these trials need to be identified: these will include exercise testing, the composite end point of time to ...
Badesch David B - - 2009
The diagnosis and assessment of pulmonary arterial hypertension is a rapidly evolving area, with changes occurring in the definition of the disease, screening and diagnostic techniques, and staging and follow-up assessment. The definition of pulmonary hypertension has been simplified, and is now based on currently available evidence. There has been ...
Keogh Anne M - - 2009
Most patients with chronic thromboembolic pulmonary hypertension are operable, and pulmonary endarterectomy is the treatment of choice. Pulmonary endarterectomy should not be delayed for medical therapy, and risk stratification helps to define patients likely to achieve the best outcome. Inoperable patients should be referred for trials of medical agents. Atrial ...
Simonneau Gérald G Centre National de Référence des Maladies Vasculaires Pulmonaires, Université Paris-Sud, Hôpital Antoine Béclère, Clamart, France. - - 2009
The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches. In 2003, during the 3rd World Symposium on Pulmonary Hypertension, the clinical classification of PH initially adopted in 1998 during the 2nd ...
Ghofrani Hossein A HA Pulmonary Hypertension Division, University of Giessen Lung Center, University Hospital Giessen, Giessen, Germany. - - 2009
Over the past 2 decades, pulmonary arterial hypertension has evolved from a uniformly fatal condition to a chronic, manageable disease in many cases, the result of unparalleled development of new therapies and advances in early diagnosis. However, none of the currently available therapies is curative, so the search for new ...
Dimitroulas Theodoros - - 2010
OBJECTIVES: Systemic sclerosis-related pulmonary arterial hypertension (SSc PAH) is a major complication of both limited and diffuse systemic sclerosis and leads to substantial morbidity and mortality. Natriuretic peptides (NP) are clinically useful markers of right ventricular dysfunction and pulmonary hypertension. The aim of our review was to examine the evidence ...
Nakwan N - - 2009
The sophisticated and expensive treatment modalities of persistent pulmonary hypertension of the newborn (PPHN), such as nitric oxide, are limited in developing countries. Alternative (less expensive) treatments are being sought and bosentan, an oral dual endothelin-1 receptor antagonist, may be an option for the treatment of PPHN. We report our ...
Naoman Shahla G - - 2010
Pulmonary hypertension is a complication of sickle cell disease that is associated with increased mortality. Whether this complication is associated with hemolysis has been questioned. Systolic pulmonary artery blood pressure can be estimated from echocardiography-determined tricuspid regurgitation velocity (TRV). A velocity of 2.5 m/s or higher suggests possible pulmonary hypertension. ...
Tissot Cecile - - 2009
Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by progressive obliteration of the pulmonary vasculature, leading to right heart failure and death if left untreated. Prior to the current treatment era, pulmonary hypertension carried a poor prognosis with a high mortality rate, but its prognosis has changed over the ...
Pitsiou Georgia G - - 2009
Pulmonary arterial hypertension (PAH) is a life-threatening disease of the pulmonary arterioles, which, in the absence of effective therapy, progresses rapidly to right heart failure and death. Opening of a patent foramen ovale (PFO) is common in patients with severe pulmonary hypertension (PH), resulting in resistive hypoxemia. We report the ...
Opitz C F - - 2009
Despite limited evidence from clinical studies, anticoagulant drugs such as vitamin K antagonists (VKA) (e.g., warfarin or phenprocoumon) are widely used in the background treatment of patients with pulmonary arterial hypertension (PAH). According to current guidelines, they are generally accepted as efficacious drugs, although their efficacy is neither supported by ...
Ocak S - - 2009
Although pulmonary arterial hypertension is usually associated with advanced stages of sarcoidosis, its occurrence in early stage disease is rare. Herein, a case of associated pulmonary arterial hypertension in the setting of Hashitoxicosis and stage II pulmonary sarcoidosis is reported. The case of associated pulmonary arterial hypertension occurred in a ...
Cavusoglu Yuksel - - 2009
Currently, prostacyclin, endothelin receptor antagonists and phosphodiesterase inhibitors are recommended therapy in idiopathic pulmonary arterial hypertension. However, each of these drugs has limited efficacy in the improvement of clinical symptoms, pulmonary hemodynamics and long-term survival. Levosimendan, a novel calcium-sensitizing inodilator agent, has been shown to have pulmonary vasodilatory action. However, ...
Guillevin L - - 2009
Vasculitis can occur either as a primary condition or secondary to CTDs, infection, medication or malignancy. This article reviews the clinical presentation and management of vascular disease associated with SLE and SS, as well as the primary necrotizing vasculitides. Although pulmonary arterial hypertension (PAH) has traditionally been considered a rare ...
Engel Peter J - - 2009
Significant progress has been made in the medical therapy of pulmonary hypertension in recent years, but long term results are still disappointing. We describe the theoretical underpinnings of a surgical procedure which we suggest might prevent or delay the occurrence of right ventricular failure and thereby improve long-term survival in ...
Zheng Yi - - 2010
Pulmonary arterial hypertension (PAH) is a severe clinical and pathophysiologic syndrome with no effective treatment at present. Mycophenolate mofetil (MMF), a prodrug of mycophenolic acid (MPA), has been applied widely to the treatment of connective tissue diseases with the effect of immunosuppressant. Its anti-proliferation has been found recently. Thereby, we ...
Shaikh Ayaz Hussain - - 2009
The case of a 65 years old lady, diabetic, hypertensive, reduced functional class with history of episodic dyspnoea lasting for few weeks for the last two years. On presentation she was tachypnoiec with reduced oxygen saturation. Jugular venous distension was present with bibasal crackles. Her ECG was normal. Echocardiogram revealed, ...
Palmer Michael J - - 2009
BACKGROUND: Knowledge of the causative reasons for pulmonary arterial hypertension, a major category of pulmonary hypertension, has expanded dramatically over the past 10 years. This has led to heightened research across a range of potential new mechanistic approaches and resulted in the identification of further treatment options, together with several ...
Faughnan M E - - 2009
Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder, characterised by the presence of vascular malformations. The pulmonary vascular complications of HHT include pulmonary arteriovenous malformations, pulmonary hypertension associated with high-output heart failure and liver vascular malformations and, finally, pulmonary arterial hypertension secondary to HHT. In the present review, ...
Brun Henrik - - 2009
We examined inflammatory mediators in patients with pulmonary hypertension related to congenital systemic-to-pulmonary shunts and the change in these markers during treatment with bosentan. Inflammatory mechanisms probably play a pathogenic role in idiopathic pulmonary arterial hypertension. Their involvement in pulmonary hypertension related to congenital systemic-to-pulmonary shunts is largely unknown. Plasma ...
- - 2009
(1) In patients with mildly symptomatic pulmonary hypertension (stage III), there is no firm evidence that ambrisentan reduces mortality or slows disease progression. In contrast, ambrisentan has frequent and sometimes serious adverse effects; (2) In patients whose physical activity is markedly restricted by pulmonary hypertension, it is better to continue ...
Kang Beodeul - - 2009
We describe a 54-year-old woman with isolated pulmonary arterial hypertension accompanied by hyperthyroidism due to Graves' disease. Her pulmonary artery hypertension resolved spontaneously after restoration of euthyroidism. This case suggests that hyperthyroidism should be considered a reversible cause of pulmonary arterial hypertension.
Beghetti Maurice - - 2009
In order to provide an overview of current knowledge, the literature was systematically examined for clinical studies, which evaluate the safety and effectiveness of bosentan in pediatric pulmonary arterial hypertension (PAH). 3 databases (MEDLINE, EMBASE and BIOSIS) were searched for the period January 2000 - October 2007 using the key ...
MacLean Margaret R - - 2009
The serotonin hypothesis of pulmonary arterial hypertension (PAH) arose owing to anorexigens, acting as indirect serotinergic agonists, causing PAH. However, it is now thought that serotonin plays an important role in the pathobiology of PAH per se. The rate-limiting enzyme in the synthesis of peripheral serotonin is tryptophan hydroxylase 1 ...
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