Search Results
Results 451 - 500 of 1634
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White R James - - 2007
Severe pulmonary arterial hypertension (PAH) occurs in idiopathic form and in association with diverse diseases. The pathological hallmarks are distal smooth muscle hypertrophy, obliteration of small pulmonary arteriole lumens, and disorganized cellular proliferation in plexiform lesions. In situ thrombosis is also observed. A detailed understanding of the disease progression has ...
Domenighetti Guido - - 2007
Pulmonary hypertension (PH) has been partially reclassified during the 2003 Third World Symposium on Pulmonary Arterial Hypertension held in Venice. PH is a common disorder that may complicate a variety of cardiopulmonary diseases, including severe COPD, left ventricular failure and chronic thromboembolic obstruction of the pulmonary arteries. Pulmonary arterial hypertension ...
Stewart Douglas R - - 2007
Neurofibromatosis type 1 (NF1) is a common disorder of dysregulated tissue growth secondary to mutations in the tumor suppressor gene NF1. Pulmonary arterial hypertension (PAH) in patients with NF1 is hypothesized to be secondary to an underlying vasculopathy. We describe the entity we term NF1-associated PAH (NF1-PAH) in four new ...
Rosenkranz Stephan - - 2007
Pulmonary hypertension (PH) is a devastating disease that - if untreated - is characterized by a poor prognosis. According to the current classification (Venice, 2003), pulmonary arterial hypertension (PAH) is distinguished from other forms of PH. Recent advances in drug therapy have led to a dramatic improvement of medical care ...
Zhang Bo - - 2007
BACKGROUND: Severe pulmonary hypertension is a common complication of congenital cardiac defects with large left to right shunt, and the closure of a large ventricular septal defect (VSD) with elevated pulmonary vascular resistance (PVR) is associated with significant morbidity and mortality. A unidirectional monovalve homologous aortic patch was designed to ...
Jain Suma - - 2007
The mechanisms leading to elevations in precapillary pulmonary vascular resistance are complex and likely involve multiple pathways, but the histopathologic sequelae of these processes are restricted to a few findings, primarily neoangiogenesis, intimal and smooth muscle proliferation, vasoconstriction, and/or in situ thrombosis. Regardless of the etiology of pulmonary hypertension, abnormalities ...
Ioachimescu O C - - 2007
Portopulmonary hypertension (PPHTN) and hepatopulmonary syndrome (HPS) are distinct clinical entities that may accompany liver disease. While PPHTN and HPS have been infrequently described as occurring in the same patient, to the present authors' knowledge, the order of occurrence has always been the initial onset of HPS, with pulmonary hypertension ...
Zhang Lizhi - - 2007
Mixed connective tissue disease (MCTD) is a systemic disease seen in a group of patients with overlapping clinical features of lupus, scleroderma, polymyositis, and rheumatoid arthritis. A defining feature of MCTD is the presence of antibodies against the U1-ribonucleoprotein (U1-RNP) complex. Pulmonary hypertension is the major cause of death in ...
Wallace Sharon M L SM Clinical Pharmacology, University of Cambridge, Addenbrooke's Hospital, Cambridge, United - - 2007
Isolated systolic hypertension is associated with increased cardiovascular risk. It is thought to result from large artery stiffening, which is determined by structural components within the vasculature but also by functional factors including NO and endothelin-1. We hypothesized that endothelial dysfunction would account for increased arterial stiffness in patients with ...
SUZUKI, Eiji
We encountered a 32-year-old woman presenting with high-grade fever and swollen lymph nodes. Echocardiography showed a pericardial effusion and progressive pulmonary hypertension (PH). Although the criteria for systemic lupus erythematosus (SLE) were met, typical laboratory values and symptoms of SLE were not present. Anti-ribonucleaoprotein antibodies were negative. Computed tomography (CT) ...
Espiritu Joseph D - - 2007
Pulmonary hypertension can occur from obstruction of the distal pulmonary arteries by thrombus, ova and parasites, and foreign material. We report a 62-year-old patient who had fatal pulmonary hypertension from an 8-cm fragment of a totally implantable venous access device (TIVAD) retained in the pulmonary artery. Despite long-term therapeutic anticoagulation, ...
Barman Scott A - - 2007
Although one of the common characteristics of pulmonary hypertension is abnormal sustained vasoconstriction, the signaling pathways that mediate this heightened pulmonary vascular response are still not well defined. Protein kinase C (PKC) and Rho-kinase are regulators of smooth muscle contraction induced by G protein-coupled receptor agonists including endothelin-1 (ET-1), which ...
Bonderman Diana - - 2007
BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by intraluminal thrombus organization and fibrous obliteration of pulmonary arteries. Recently, associated medical conditions such as splenectomy, ventriculoatrial shunt for the treatment of hydrocephalus, permanent central intravenous lines, inflammatory bowel disease, and osteomyelitis were found to be associated with the development of ...
Sehgal Pravin B - - 2007
Histological and electron microscopic studies over the past four decades have highlighted "plump," "enlarged" endothelial, smooth muscle, and fibroblastic cellular elements with increased endoplasmic reticulum, Golgi stacks, and vacuolation in pulmonary arterial lesions in human and in experimental (hypoxia and monocrotaline) pulmonary arterial hypertension. However, the contribution of disrupted intracellular ...
Griffin N - - 2007
AIM: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. MATERIAL AND METHODS: From the National Pulmonary Hypertension Database, we ...
LaRaia Anne V - - 2007
Pulmonary arterial hypertension (PAH), a rare disease involving the pulmonary vascular circuit, is defined as an elevation in pulmonary arterial pressures and is characterized by symptoms of dyspnea, chest pain, and syncope. If left untreated, the disease carries a high mortality rate, with the most common cause of death being ...
Yu Min - - 2007
Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease of unknown etiology. The exact pathogenesis of pulmonary arterial hypertension is still not well known. In the past decades, many protein molecules have been found to be involved in the development of IPAH. With proteomic techniques, profiling of human plasma proteome ...
Colle Isabelle - - 2007
Hepatopulmonary syndrome (HPS) is found in 4-47% of patients with cirrhosis and is characterized by intrapulmonary vascular dilatations especially in the basal parts of the lung. Liver injury and/or portal hypertension trigger the release of endothelin-l, TNF-alpha, cytokines and mediate vascular shear stress and release of nitric oxide and carbon ...
Zhang Yanhang - - 2007
We applied a statistical mechanics based microstructural model of pulmonary artery mechanics, developed from our previous studies of rats with pulmonary arterial hypertension (PAH), to patient-specific clinical studies of children with PAH. Our previous animal studies provoked the hypothesis that increased cross-linking density of the molecular chains may be one ...
Kato Gregory J - - 2007
Pulmonary arterial hypertension (PAH), once considered a rare complication of sickle cell disease (SCD) and thalassemia, appears to be more common in adults with hemoglobinopathy than previously appreciated. On prospective screening of adults with SCD, approximately one-third of adults are found on echocardiography to have a tricuspid regurgitant jet velocity ...
Benza Raymond L - - 2007
Pulmonary arterial hypertension (PAH) is a rare but frequently fatal condition marked by vasoconstriction and vascular remodeling within small pulmonary arteries. The pathobiology of PAH involves imbalances in a multitude of endogenous mediators, which promote aberrant cellular growth, vasoconstriction and hemostasis within the pulmonary vascular tree. The mechanisms promoting these ...
Dorfmüller Peter - - 2007
Pulmonary arterial hypertension is a severe complication of connective tissue diseases. It is currently well established that pulmonary arterial hypertension associated with connective tissue diseases such as systemic sclerosis is frequently less responsive or even refractory to pulmonary vasodilator therapies. In that setting, pulmonary venoocclusive disease is believed to contribute ...
Peacock A J - - 2007
All hospitalisations for pulmonary arterial hypertension (PAH) in the Scottish population were examined to determine the epidemiological features of PAH. These data were compared with expert data from the Scottish Pulmonary Vascular Unit (SPVU). Using the linked Scottish Morbidity Record scheme, data from all adults aged 16-65 yrs admitted with ...
Keenan N G - - 2008
Pulmonary tumour thrombotic microangiopathy (PTTM) presents as acute pulmonary hypertension and sudden cardiovascular collapse in individuals with adenocarcinoma, usually of the stomach. The illness follows a fulminant course often before the adenocarcinoma has become clinically apparent. Frequently the diagnosis is made only at necropsy. The diagnosis can be made by ...
Park Jae Hyeong - - 2007
In developed countries, the incidence of cardiac beriberi is rare. It can lead to high output cardiac failure and pulmonary hypertension. We hereby report an atypical case of beriberi heart with reversible right heart failure and severe pulmonary hypertension. Thiamine deficiency can cause reversible pulmonary hypertension, and it must be ...
Hardziyenka Maxim - - 2007
AIMS: To study whether pre-operative assessment, using echocardiography, of the timing of a particular feature in the pulmonary flow (pulmonary flow systolic notch) may predict in-hospital mortality and mid-term haemodynamic improvement after pulmonary endarterectomy (PEA) for chronic thrombo-embolic pulmonary hypertension (CTEPH). METHODS AND RESULTS: Fifty-eight of 61 consecutive CTEPH patients ...
Penaloza Dante - - 2007
More than 140 million people worldwide live >2500 m above sea level. Of them, 80 million live in Asia, and 35 million live in the Andean mountains. This latter region has its major population density living above 3500 m. The primary objective of the present study is to review the ...
- - 2007
Pulmonary arterial hypertension (PAH) is a life-threatening disorder characterized by elevated pulmonary artery pressure and pulmonary vascular resistance. Continuous infusion of a prostanoid, which acts as a vasodilator and anti-proliferative agent, is indicated in the treatment of patients with severe PAH. Two prostanoids are approved for intravenous (IV) use in ...
Trow Terence K - - 2007
Accurate diagnosis of pulmonary arterial hypertension is a challenging and complex process that requires a high index of clinical suspicion from even the most astute clinician. This article discusses the use of a variety of noninvasive tests that can help define the population of patients in whom invasive cardiac catheterization ...
Smedema J P - - 2007
Hereditary spherocytosis consists of a group of haemolytic anaemias caused by defects in the proteins involved in the vertical interactions between the membrane skeleton and the lipid bilayer of the red blood cell. Inheritance is most commonly autosomal dominant with variable expression. Splenectomy may be indicated because of anaemia or ...
Lode Holger N - - 2007
The development of pulmonary arterial hypertension (PAH) is the leading cause of mortality in patients with thalassemia and sickle cell anemia and was reported to occur in hemolytic anemias such as hereditary stomatocytosis, and paroxysmal nocturnal hemoglobinuria. Here, we report for the first time on the development of PAH in ...
Landzberg Michael J - - 2007
Pulmonary arterial hypertension associated with congenital heart disease, although common (15%-30%) in all-comers with congenital heart disease, is variable in terms of clinical manifestations, severity of associated pulmonary arterial hypertension, and response to therapy and outcomes (depending on lesion anatomy, pulmonary circulation flows and pressures, and presence and timings of ...
Ravipati Gautham - - 2007
Since the discovery of sildenafil in 1989 as a highly selective inhibitor of the phosphodiesterase type-5 (PDE-5) receptor, 2 additional PDE-5 inhibitors, tadalafil and vardenafil, have emerged as safe and effective treatments of erectile dysfunction (ED). Enzymes in the PDE family catalyze the hydrolysis of the intracellular signaling molecules cyclic ...
Alam Shoaib - - 2007
After half a century of clinical experience and research, management of pulmonary arterial hypertension remains a challenge. Currently, data to support the use of standard therapies for pulmonary arterial hypertension (oxygen supplementation, diuretics, digoxin, anticoagulation, and calcium channel blockers) are mostly retrospective, uncontrolled prospective, or derived from other diseases with ...
Sallach Susan M - - 2007
Pulmonary artery hypertension is a rare disease with significant morbidity and mortality. Initial and serial noninvasive assessment of these patients can be accomplished with transthoracic echocardiography and/or cardiac magnetic resonance imaging. These complementary techniques provide the structural and functional information required to care for patients with pulmonary artery hypertension and ...
Taichman Darren B - - 2007
Progress in understanding the basic biology and the development of new therapies for pulmonary arterial hypertension have led to improvements in survival. This article reviews clinically important changes in the classification of the pulmonary hypertensive diseases, as well as the epidemiology of various forms of pulmonary hypertension. The risk factors ...
Tuder Rubin M - - 2007
The secondary role of pathology in the present clinical management of pulmonary hypertension (PH) reflects to some extent the limitations of the current understanding of the disease. Ample room exists for the diagnostic translation of the pathobiologic studies, with the goal of improving the diagnostic and prognostic power of the ...
Snow Jennifer L - - 2007
Recent discoveries in the disease pathophysiology of pulmonary arterial hypertension have been translated into effective therapies tested in clinical trials. The studies have focused on surrogate and intermediate end points, thought to reflect quantity and quality of life, respectively. The authors present the necessary requirements for establishing the reliability and ...
Eder Lihi - - 2007
Only a limited number of patients with amyloidosis and pulmonary hypertension have been reported in the literature. We report a 73-year-old female with AL type amyloidosis who developed respiratory insufficiency and right heart failure because of severe pulmonary hypertension. There were no signs of cardiac involvement with amyloid or findings ...
O'Callaghan Dermot S - - 2007
This review assesses the available evidence supporting the use of drug combinations for the management of the various forms of pulmonary arterial hypertension (PAH). Ongoing and forthcoming randomized trials evaluating this strategy are also highlighted. Furthermore, new types of agents to treat PAH in the future are explored.
Said Sami I - - 2007
BACKGROUND: Vasoactive intestinal peptide (VIP), a pulmonary vasodilator and inhibitor of vascular smooth muscle proliferation, has been reported absent in pulmonary arteries from patients with idiopathic pulmonary arterial hypertension (PAH). We have tested the hypothesis that targeted deletion of the VIP gene may lead to PAH with pulmonary vascular remodeling. ...
Hoeper M M - - 2007
Blood gas abnormalities in patients with idiopathic pulmonary arterial hypertension (IPAH) may be related to disease severity and prognosis. The present authors performed a 12-yr retrospective analysis assessing arterialised capillary blood gases, haemodynamics, exercise variables and survival in 101 patients with IPAH. At baseline, arterial oxygen tension (P(a,O(2))) and carbon ...
Ulrich Silvia - - 2007
Pulmonary hypertension (PH) is often difficult to diagnose and many different disorders may result in elevated pulmonary arterial pressure requiring therapy. Left untreated, PH usually has a dismal prognosis culminating in right ventricular failure and death. Besides conservative therapeutic strategies such as anticoagulation and diuretics, the past decade has brought ...
Suntharalingam Jay J Pulmonary Vascular Diseases Unit, Papworth Hospital NHS Trust, Papworth Everard, Cambridgeshire CB3 8RE, - - 2007
Although pulmonary endarterectomy (PEA) is potentially curative in chronic thromboembolic pulmonary hypertension (CTEPH), some patients have distally distributed disease that is not amenable to surgery. The aetiology and characteristics of this patient group are currently not well understood. This study compares the baseline demographic features and outcomes in subjects with ...
Sayin Tamer - - 2007
Primary pulmonary hypertension is a rare disease with an ominous prognosis for which new therapeutic options are being developed. Recently, there have been case reports indicating that sildenafil may be of benefit in short- to midterm follow-up. However, long-term clinical data of sildenafil in primary pulmonary hypertensive patients is lacking. ...
Yang Ying - - 2007
Calcium signaling has been reported to be involved in the pathogenesis of hypertension. Verapamil, one of the calcium antagonists, is used to characterize the role of calcium signaling in the development of pulmonary arterial hypertension syndrome in broilers. The suppression effect of verapamil on pulmonary arterial hypertension and pulmonary vascular ...
Nathan Steven D - - 2007
Idiopathic pulmonary fibrosis (IPF) has a poor prognosis and a course that is unpredictable. Pulmonary hypertension may complicate the course of IPF and potentially impact prognosis. There are multiple factors that might influence the onset and severity of pulmonary hypertension in IPF. The relationship between the physiologic and pathobiologic manifestations ...
Sahara Makoto - - 2007
BACKGROUND: Recent evidence suggests that bone marrow (BM)-derived cells may differentiate into vascular cells that participate in arterial repair and/or lesion formation. However, it remains uncertain whether BM-derived cells also can participate in vascular remodeling associated with pulmonary arterial hypertension. METHODS AND RESULTS: The BM of Sprague-Dawley rats was reconstituted ...
van Wolferen S A - - 2007
Pulmonary hypertension is a rare disease with a poor prognosis. It was first described in the late 19th century as a clinical-pathological syndrome characterised by obstruction of the small pulmonary arteries and right ventricular hypertrophy in patients presenting with severe dyspnoea and cyanosis. After the development of right heart catheterisation ...
Leibovitch Leah - - 2007
Pulmonary hypertension is characterised by a progressive increase in pulmonary vascular resistance and a poor prognosis. The exact underlying mechanisms are still poorly understood; however, it is hypothesised that pulmonary medial hypertrophy and endothelial dysfunction lead to impaired production of vasodilators such as nitric oxide (NO) and prostacyclin, and increased ...
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