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Results 301 - 350 of 1634
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Faruqi S - - 2010
Sitaxentan, a highly-selective endothelin receptor antagonist (ETRA) and bosentan a non-selective ETRA are both approved for the treatment of idiopathic pulmonary arterial hypertension (iPAH). Sildenafil is a phosphodiesterase-5 (PDE-5) inhibitor used in the treatment of iPAH. Tadalafil is a long acting PDE-5 inhibitor largely unexplored for the treatment of iPAH. ...
Olsson Karen M - - 2009
Pulmonary arterial hypertension (PAH) has evolved from an untreatable condition to a disease for which several classes of drugs have now been approved, including various prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors. Because the pathogenesis of pulmonary hypertension is increasingly understood, various new substances are now under clinical investigation, including ...
De Wolf Daniel - - 2009
Pulmonary arterial hypertension is a rare disorder in childhood, the two most common types being idiopathic pulmonary arterial hypertension and pulmonary hypertension associated with congenital left-to-right shunt lesions, together accounting for almost 90% of cases. The clinical presentation of idiopathic pulmonary arterial hypertension (familial and non familial) is essentially non-specific ...
Hughes Michael - - 2009
Left vocal cord palsy is a well-recognised rare complication of SLE. We encountered a gentleman who during an acute flare developed left vocal fold palsy (VCP). Immunosuppression was increased, but this only produced partial improvement. VCP is commonly but not exclusively caused by recurrent laryngeal nerve palsy. Other causes may ...
Hong-liang Zhang - - 2009
OBJECTIVE: Heart rate-corrected QT interval (QTc) and QTc dispersion (QTcd) are increased and associated with ventricular arrhythmia and an increase in sudden death in a variety of diseases. This study aimed to examine QTc and QTcd in pulmonary hypertension and assess their relationship with pulmonary arterial pressure. PATIENTS AND METHODS: ...
Streit Michael - - 2009
Pulmonary arterial hypertension is a complication of systemic lupus erythematosus. Mortality in pregnant patients with pulmonary arterial hypertension related to connective tissue disease is as high as 56%. The authors report the first case of a successful maternal-fetal outcome in a pregnant patient with systemic lupus erythematosus-associated pulmonary arterial hypertension ...
Ferns Sunita Juliana - - 2009
Pulmonary arterial hypertension (PAH) afflicts thousands of children worldwide. The pathophysiology involves intravascular proliferation and remodeling leading to an increase in pulmonary vascular resistance which if left untreated results in right heart failure and death. Signs and symptoms are subtle as the disease progresses to irreversible lung damage. There is ...
Ho Roger C M - - 2009
Since the Food and Drug Administration (FDA) approved the use of buprenorphine hydrochloride (Subutex) for the treatment of opiate dependence in 2002, there has been a global trend of its IV abuse which led to life-threatening medical complications such as infective endocarditis (IE), cardiac failure, and death. First episode IE ...
Aqel Raed - - 2009
Fibromuscular dysplasia (FMD) is predominantly a disease of younger women, but it can occur and cause refractory hypertension in the elderly. We present here classic angiographic and intravascular ultrasound images of FMD in a 70-year-old woman with refractory hypertension. Renal artery FMD should be included in the differential diagnosis of ...
Gowrinath K - - 2009
Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon consequence of acute pulmonary embolism. We report CTEPH in a 58-year-old male who had pleurisy with a small haemorrhagic pleural effusion three months ago. The six-month course of anti-coagulation therapy failed to resolve thromboemboli completely or improve pulmonary hypertension. Computed tomographic pulmonary ...
Tulloh Robert - - 2009
Major advances have been made in the understanding and treatment of pulmonary hypertension in the last few years. Without treatment (medication) for idiopathic pulmonary arterial hypertension, which is a rare and potentially fatal condition, the survival time is only about 3 years after diagnosis. However, if pulmonary hypertension is secondary ...
Hassoun Paul M - - 2009
Pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis, carries a very severe prognosis and is one of the leading causes of death in patients who suffer from it. Indeed, response to modern medical therapy has been disappointing in scleroderma-related PAH compared with other forms of PAH from the ...
Stewart Susan - - 2009
Pulmonary hypertension has a complex pathobiology and the term pulmonary arterial hypertension (PAH) includes a variety of pulmonary hypertensive diseases that have different aetiologies, but which may have a similar clinical presentation and, in some cases, the same response to medical treatment. More recent classifications have been based on aetiology, ...
Castañer Eva - - 2009
Chronic pulmonary thromboembolism is mainly a consequence of incomplete resolution of pulmonary thromboembolism. Increased vascular resistance due to obstruction of the vascular bed leads to pulmonary hypertension. Chronic thromboembolic pulmonary hypertension is clearly more common than previously was thought, and misdiagnosis is common because patients often present with nonspecific symptoms ...
Lloyd Kenneth Scott - - 2009
Pulmonary vascular hypertension in general is a progressive, nearly always fatal condition that until recently has had very few treatment options. Our understanding of the pulmonary vascular disease process has opened the window to earlier screening techniques, diagnosis, and treatment options. However, all current treatment options are complex and expensive ...
Jansa P - - 2009
Pulmonary arterial hypertension (PAH) is a severe chronic disorder of pulmonary arteries with progressive precapillary pulmonary hypertension, characterized by poor life quality and very poor prognosis. Unless treated, it causes death within 2-3 years from diagnosis. PAH affects mainly younger women. The treatment of PAH should not only be symptomatic, ...
Casserly Brian - - 2009
Ambrisentan is an endothelin receptor antagonist (ERA) that was recently approved for treatment of pulmonary arterial hypertension (PAH). Endothelin (ET) is a potent vasoconstrictor with mitogenic, hypertrophic and pro-inflammatory properties that is upregulated in pulmonary hypertensive diseases. The biologic effects of ET are mediated by 2 cell surface receptors termed ...
Roberts Kari E - - 2009
Endothelin receptor antagonism has emerged as an important therapeutic approach in pulmonary arterial hypertension (PAH). Bench to bedside scientific research has clearly shown that endothelin-1 (ET-1) is over-expressed in several forms of pulmonary vascular disease and plays an important pathogenetic role in the development and progression of PAH. Oral endothelin ...
Levy Andrew S - - 2009
This review highlights a number of nitric oxide (NO)-related mechanisms that contribute to coronary vascular function and that are likely affected by hypertension and thus become important clinically as potential considerations in prevention, diagnosis, and treatment of coronary complications of hypertension. Coronary vascular resistance is elevated in hypertension in part ...
Barman Scott A - - 2009
Pulmonary arterial hypertension (PAH) is a devastating disease characterized by progressive elevation of pulmonary arterial pressure and vascular resistance due to pulmonary vasoconstriction and vessel remodeling as well as inflammation. Rho-kinases (ROCKs) are one of the best-described effectors of the small G-protein RhoA, and ROCKs are involved in a variety ...
Sakao Seiichiro - - 2009
Severe pulmonary arterial hypertension, whether idiopathic or secondary, is characterized by structural alterations of microscopically small pulmonary arterioles. The vascular lesions in this group of pulmonary hypertensive diseases show actively proliferating endothelial cells without evidence of apoptosis. In this article, we review pathogenetic concepts of severe pulmonary arterial hypertension and ...
Tan Xiao-Yan - - 2009
Studies on the role of connexins (Cxs) in the pathogenesis of right ventricular (RV) hypertrophy in pulmonary arterial hypertension (PAH) have not been reported to date. Therefore, we established a rat model of PAH induced by monocrotaline (MCT), and they were randomized to three groups: Control, MCT, and MCT+bosentan. Through ...
Lu Ling - - 2008
Portopulmonary hypertension (PPH) is clinically defined as the development of pulmonary arterial hypertension complicated by portal hypertension, with or without advanced hepatic disease. Physical signs may be absent in mild to moderate PPH and only appear in a hyperdynamic circulatory state. Similar signs of advanced liver disease can be observed ...
Ewert Ralf - - 2008
Pulmonary hypertension is a clinically relevant, life-threatening disease. Based pathophysiologically on a dysbalance of vasoactive mechanisms, the therapeutic use of vasodilative agents is one of the major treatment options. Prostanoids, such as iloprost, are widely used in the treatment of patients with pulmonary hypertension. With the focus on pulmonary hypertension, ...
Frachon I - - 2008
The prognosis of postembolic pulmonary hypertension, a rare and serious disease, has been transformed with the curative intervention of pulmonary endarteriectomy. The screening is based on two key non invasive examinations, the cardiac ultrasound and ventilation-perfusion scintigraphy. The confirmation of the diagnosis and the determination of the best therapeutic options ...
Henkens, Ivo Reinier
Right ventricular overload covers a spectrum ranging from volume overload to pressure overload, and often is a combination of these, compromising cardiac function. Part I focuses on right ventricular volume overload in adults with Fallot’s tetralogy corrected in early childhood. We determined which patient characteristics are associated with a more ...
Doran Aimee - - 2008
Pulmonary arterial hypertension is a rare but progressive and life-threatening disease that presents considerable challenges for both the patient and the caregiver. Though complex, intravenous epoprostenol and treprostinil may improve long-term survival, exercise capacity, hemodynamics, and other clinical symptoms of pulmonary arterial hypertension. Recent advances in infusion pump technology offer ...
Popov Aron Frederik - - 2008
OBJECTIVE: Endothelin is the most potent endogenous vasoconstrictor and is involved in several vascular disorders such as arterial hypertension. Its intense interaction with other vasoactive hormone systems revealed the consideration about the endothelin gene as an interesting candidate for influencing the development of essential hypertension and hypertensive endorgan damage. The ...
Said Sami I - - 2008
Pulmonary vascular remodeling and inflammation often coexist in clinical and experimentally induced pulmonary arterial hypertension (PAH). In some instances, the pulmonary hypertension may be the primary, or at least the initial, problem, while inflammatory or autoimmune responses appear to initiate or dominate the picture in other cases. Based on studies ...
Cicekcioglu Ferit - - 2008
Weaning from cardiopulmonary bypass is the most important stage during mitral valve surgery, especially in patients with severe pulmonary hypertension. We report two patients with severe pulmonary hypertension who were operated on because of valvular heart disease. To reduce the pulmonary artery pressure, levosimendan was used because of its vasodilatory ...
Chaouat A - - 2008
Mild-to-moderate pulmonary hypertension is a common complication of chronic obstructive pulmonary disease (COPD); such a complication is associated with increased risks of exacerbation and decreased survival. Pulmonary hypertension usually worsens during exercise, sleep and exacerbation. Pulmonary vascular remodelling in COPD is the main cause of increase in pulmonary artery pressure ...
Hrometz Sandra L - - 2008
OBJECTIVE: To review the role of ambrisentan in the treatment of pulmonary arterial hypertension (PAH). DATA SOURCES: Literature was accessed through MEDLINE (1950-June 2008), Iowa Drug Information Service (1966-March 2008), EMBASE (1966-June 2008), bibliographies of pertinent articles, and unpublished data provided by the manufacturer and the Food and Drug Administration ...
Vonk Noordegraaf A - - 2008
SSc-associated pulmonary arterial hypertension (PAH) has a poorer prognosis than that of other types of pulmonary hypertension. Recent echocardiographic and haemodynamic studies suggest that right ventriculer (RV) pump function and filling characteristics are altered in SSc-PAH as compared with idiopathic PAH. This could be explained by intrinsic myocardial involvement of ...
Kowal-Bielecka O - - 2008
SSc is complicated in approximately 10% of the patients by pulmonary arterial hypertension (PAH), a rare dyspnoea-fatigue syndrome caused by an increase in pulmonary vascular resistance. The prognosis of SSc-PAH is particularly poor, with estimated survival rates of approximately 50% at 2 yrs without pulmonary circulation-targeted therapies. Prostacyclins, endothelin receptor ...
Thistlethwaite Patricia A - - 2008
Pulmonary endarterectomy (PTE) is the definitive treatment for chronic pulmonary hypertension resulting from thromboembolic disease. Chronic thromboembolic pulmonary hypertension is estimated to occur in approximately 4% of patients who have developed an acute pulmonary embolism, though the true prevalence is suspected to be much higher. Chronic thromboembolic pulmonary hypertension is ...
Melly L - - 2008
AIM: Alveolar capillary dysplasia (ACD) is a rare disorder, typically presenting with persistent pulmonary hypertension of the newborn. The aim was to characterise further the histological features of patients suspected of having ACD and to correlate histopathological features with outcome. METHODS AND RESULTS: Three pathologists retrospectively reviewed 21 surgical lung ...
Peinado Víctor I - - 2008
Alterations in pulmonary vessel structure and function are highly prevalent in patients with COPD. Vascular abnormalities impair gas exchange and may result in pulmonary hypertension, which is one of the principal factors associated with reduced survival in COPD patients. Changes in pulmonary circulation have been identified at initial disease stages, ...
Frenckner Björn - - 2008
Increased pulmonary vascular resistance causing pulmonary artery hypertension is a major problem in the treatment of congenital diaphragmatic hernia with a strong association to mortality. We here report a patient with intractable pulmonary hypertension at 4 weeks of age unresponsive to conventional treatment. After administration of the platelet-derived growth factor ...
Kluge Stefan - - 2009
BACKGROUND: The insertion of ventriculoatrial (VA) shunts for the treatment of hydrocephalus is thought to be associated with the development of pulmonary hypertension in adults. OBJECTIVES: It was the aim of this study to describe the frequency and the clinical spectrum of pulmonary hypertension in adults with VA shunts. METHODS: ...
Epling-Burnette P K - - 2008
Large granular lymphocyte (LGL) leukemia is commonly associated with poor hematopoiesis. The first case of pulmonary artery hypertension (PAH) was observed in a 57-year-old woman with natural killer (NK)-LGL leukemia and transfusion-dependent anemia. Using a genetic approach, we demonstrated that killing of pulmonary endothelial cells by patient NK cells was ...
Ghofrani Hossein A - - 2008
The availability of new treatments for patients with pulmonary arterial hypertension has increased awareness and interest in the medical community for pulmonary vascular diseases in general. Many uncertainties exist, however, regarding the diagnosis and treatment of patients with pulmonary arterial hypertension that are particularly pertinent for the management of patients ...
Papakonstantinou E - - 2008
Idiopathic pulmonary arterial hypertension (IPAH) is a fatal disease characterised by elevated blood pressure in the pulmonary circulation. Initial vasoconstriction, proliferation of pulmonary arterial smooth muscle cells (PASMC) and increased deposition of extracellular matrix (ECM) contribute to pathological remodelling of pulmonary arterioles in IPAH. Glycosaminoglycans (GAGs), components of the ECM, ...
Afshar Kamyar - - 2008
PURPOSE OF REVIEW: To examine recent advancements in the clinical characteristics, treatment and prognosis of diffuse parenchymal disease and pulmonary hypertension due to idiopathic pulmonary fibrosis, scleroderma and sarcoidosis. RECENT FINDINGS: Bosentan can be used in delaying the time to progression of disease in biopsy-proven idiopathic pulmonary fibrosis patients. There ...
Haque Abida K - - 2008
CONTEXT: Obesity is associated with sleep disordered breathing and cardiovascular morbidity, but the relationship between pulmonary hypertension, heart disease, and obesity is unknown. OBJECTIVE: To determine the prevalence of pulmonary and cardiovascular disease in obese subjects undergoing autopsy at a large medical center. DESIGN: A search through autopsy records from ...
Babi?ska Anna - - 2008
Chromaffinoma of the adrenal medulla (pheochromocytoma--PHEO) is a rare cause of arterial hypertension which is diagnosed incidentally or run in a family as a component of disease syndromes of the genetic origin. PHEO is diagnosed in about 5-10% of patients with type 1 neurofibromatosis (NF1). In a patient group with ...
Frantz Robert P - - 2008
Endothelin-1 is a potent vasoconstrictor and mitogen that is primarily synthesized and released from vascular endothelial cells. Bosentan is a dual endothelin-receptor antagonist that initially received approval for treatment of WHO group I pulmonary arterial hypertension (PAH) for patients in functional classes III and IV. Analysis of a study conducted ...
Diaz-Guzman Enrique - - 2008
Pulmonary hypertension is an uncommon complication of sarcoidosis, but in severe pulmonary disease it occurs frequently. It is an important cause of cryptogenic dyspnea in sarcoidosis patients and can occur despite the absence of pulmonary fibrosis. The true prevalence is unknown. With the advent of specific therapies for pulmonary hypertension, ...
Lammey Michael L - - 2008
This report describes the diagnosis and treatment of pulmonary arterial hypertension (PAH) in an adult male captive chimpanzee. Although cardiovascular disease in general is common in human and great apes, diagnosis and treatment of PAH in nonhuman primates are uncommon. In the case we present, the adult chimpanzee was diagnosed ...
Mutlu Halil - - 2009
Pulmonary artery dissection (PAD) is a rare diagnosis that is often made postmortem in patients with pulmonary hypertension. It can be visualized by echocardiography, computed tomography, or magnetic resonance imaging. We present a patient with emphysematous chronic obstructive pulmonary disease and secondary pulmonary hypertension in whom a PAD appeared like ...
MacIntyre Iain M - - 2008
Sitaxsentan is the first oral endothelin receptor antagonist (ETRA) with high selectivity for the endothelin-A (ET(A)) receptor to be approved for clinical use by regulatory agencies in Europe for the treatment of pulmonary arterial hypertension (PAH). Clinical trials have shown it to be well tolerated and to improve exercise tolerance, ...
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