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Results 351 - 400 of 1660
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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, ...
Nathan Aruna T - - 2008
The presence of pulmonary arterial hypertension (PAH) is a significant predictor of major perioperative cardiovascular complications in patients undergoing cardiac diagnostic or interventional procedure or non cardiac surgery under sedation and/or anesthesia. Factors that precipitate a pulmonary hypertensive crisis include hypoxia, hypercarbia, acidosis, hypothermia, pain and airway manipulations. Pain management ...
Hennigan S - - 2008
Pulmonary hypertension is a common but underdiagnosed complication of systemic lupus erythematosus, which can be associated with significant morbidity and early mortality. Although often associated with anti-phospholipid antibodies, the etiology remains poorly understood. In case reports and small open trials, the anti-CD20, B-cell targeted therapeutic antibody, rituximab, has been reported ...
Boutet K - - 2008
Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction, in situ thrombosis, and vascular remodeling of small pulmonary arteries inducing increased pulmonary arterial resistance. Conventional treatment is based on life style modification and nonspecific treatment (warfarine, diuretics, oxygen). Calcium channel blockers are vasodilatators that have been shown to be of great ...
Dahoui Hanane A - - 2008
Infantile pyknocytosis is a rare condition characterized by transient neonatal hemolytic anemia associated with increased pyknocyte count on blood smear. We describe three siblings with infantile pyknocytosis, born to consanguineous parents. The first and third siblings had neonatal hemolytic anemia that resolved spontaneously at 6 months. The second sibling presented ...
Achouh L - - 2008
Once the diagnosis of pulmonary arterial hypertension is established, wheezing and chronic cough are rarely described during the course of the disease. The present study reports on two nonsmoking patients with severe pulmonary arterial hypertension, confirmed by right-heart catheterisation, who developed chronic cough, wheezing and irreversible obstructive lung disease masquerading ...
Henkens I R - - 2008
A 54-year-old female was referred to our centre for further evaluation of recently established severe pulmonary hypertension. Six months prior to presentation to the cardiologist of the referring centre, the patient had first experienced exertional dyspnoea. At the time of presentation to the referring cardiologist, the patient's ECG showed signs ...
Ventetuolo Corey E - - 2008
Pulmonary arterial hypertension is a rare and often devastating disease, although various effective therapies are now available. Clinical trials have used hemodynamic, cardiac imaging, laboratory, and exercise measurements as surrogate and intermediate end points in pulmonary arterial hypertension. Yet, based on the current literature, it is difficult to surmise which ...
Gomberg-Maitland Mardi - - 2008
This article elaborates on the phases of drug development and trial design for pulmonary arterial hypertension, emphasizing the need for learning trials to assess efficacy signals before confirming trials. In this new era in pulmonary arterial hypertension, we need to reconsider innovative trial designs to maximize efficiency and pursue agents ...
Le Pavec Jérôme - - 2008
RATIONALE: Portopulmonary hypertension (PoPH) can be defined as elevation of pulmonary arterial pressure and pulmonary vascular resistance in the setting of portal hypertension. Survival results in PoPH are contrasting, and prognostic factors need to be identified. OBJECTIVES: To analyze long-term survival in a large cohort of patients with PoPH with ...
Schrier Robert W - - 2008
In this article, the pathophysiology of left ventricular failure is reviewed. By contrast, the paucity of information about pulmonary arterial hypertension and right ventricular failure is acknowledged. The potential mechanisms whereby renal sodium and water retention in right ventricular failure secondary to pulmonary arterial hypertension can occur, despite normal left ...
O'Callaghan Dermot S - - 2008
INTRODUCTION: Pulmonary arterial hypertension (PAH) is a rare, progressive disease for which no cure exists. However, improved understanding of underlying pathophysiological mechanisms has led to the development of several effective treatments that improve haemodynamics and functional status. OBJECTIVE: An overview of emerging pharmacological approaches to the management of PAH is ...
Aqel Raed A - - 2008
A 61-year-old white female, a Jehovah's Witness, with severe pulmonary hypertension, presented with worsening heart failure symptoms. She had a pericardial effusion with left ventricular (LV) diastolic collapse on transthoracic echocardiography. She was not a candidate for surgical pericardial window and therefore underwent pericardiocentesis and percutaneous balloon pericardiotomy with remarkable ...
Sakuma Masahito - - 2008
BACKGROUND: The pulmonary vascular changes induced by epoprostenol in patients with idiopathic pulmonary artery hypertension (IPAH) have not been reported by a clinical study. METHODS AND RESULTS: Analysis 1 compared the wedged pulmonary angiography (PAG) findings prior to initiation of epoprostenol therapy (n=24) with those after initiation (n=16). Analysis 2 ...
Overbeek M J - - 2008
The platelet-derived growth factor receptor inhibitor imatinib has demonstrated clinical and haemodynamical improvement in both animal models of pulmonary hypertension (PH) and patients with PH. It has been suggested that anti-proliferative effects on pulmonary vascular smooth muscle cells are responsible for these beneficial effects. The current study describes a patient ...
Rabinovitch Marlene M Department of Pediatrics, Stanford University School of Medicine, Stanford, California 94305-5162, USA. - - 2008
Recent investigations have suggested that it might be possible to reverse the pathology of pulmonary arterial hypertension (PAH), a disorder that can be rapidly progressive and fatal despite current treatments including i.v. prostacyclin. This review will address the cellular and molecular processes implicated in clinical, genetic, and experimental studies as ...
Oduber Charlene E U - - 2009
We report four patients with chronic thromboembolic pulmonary hypertension (CTEPH) presumably due to recurrent pulmonary embolism from low-flow vascular malformations, and give a review of the literature. Venous malformations, such as those observed in Klippel- Trenaunay syndrome (KTS) can be associated with hypercoagulability, thrombosis and recurrent pulmonary embolism and ultimately ...
Thomas de Montpréville Vincent - - 2008
To assess the histological bases of lymphadenomegaly, which has been reported as a frequent radiological finding in pulmonary veno-occlusive disease (PVOD), we have reviewed pulmonary and mediastinal lymph nodes resected during lung transplantations in 19 patients suffering from PVOD and related pulmonary capillary haemangiomatosis (PCH). Lymphatic congestion was common and ...
Dempsie Y - - 2008
Pulmonary arterial hypertension (PAH) is characterized by a sustained and progressive elevation in pulmonary arterial pressure and pulmonary vascular remodelling leading to right heart failure and death. Prognosis is poor and novel therapeutic approaches are needed. The serotonin hypothesis of PAH originated in the 1960s after an outbreak of the ...
Rosenzweig Erika B - - 2008
PURPOSE OF REVIEW: With rapid advances in the understanding and treatment of pulmonary arterial hypertension, navigating the pediatric literature becomes challenging. A comprehensive review of the most recent literature over the past year on available and emerging novel therapies as well as an approach to target pediatric populations will provide ...
Porres-Aguilar Mateo - - 2008
Antiphospholipid syndrome is an autoimmune disease characterized pathophysiologically by the presence of antiphospholipid antibodies and > or =1 clinical manifestation, the most common being venous or arterial thrombosis. We describe the case of a 40-year-old male with unexplained severe pulmonary arterial hypertension with a seven-day history of progressive shortness of ...
Skoro-Sajer Nika - - 2008
BACKGROUND: Pulmonary hypertension (PH) is a severely disabling disorder characterized by sustained elevations of pulmonary arterial pressure, ultimately leading to right-heart failure and death. Pulmonary arterial hypertension (PAH) usually occurs in the absence of an evident cause (idiopathic PAH) or may be associated with connective tissue disease, HIV infection, congenital ...
Aliyu Zakari Y - - 2008
Pulmonary artery systolic hypertension is common and associated with increased mortality among adult sickle cell disease (SCD) patients in the United States. Although the prevalence of SCD is highest in sub-Saharan Africa, the frequency of pulmonary artery systolic hypertension and the risk factors for the development of pulmonary hypertension have ...
Zhu Shu S Department of Pharmacology and Toxicology, Medical College of Georgia, Augusta, Georgia 30912, - - 2008
BKCa channels regulate pulmonary arterial pressure, and protein kinase C (PKC) inhibits BK(Ca) channels, but little is known about PKC-mediated modulation of BKCa channel activity in pulmonary arterial smooth muscle. Studies were carried out to determine mechanisms of PKC modulation of BKCa channel activity in pulmonary arterial smooth muscle cells ...
Villavicencio Karrie K Department of Cardiology, University of Colorado at Denver Health Sciences Center, Denver, CO, - - 2008
Pulmonary hypertension is a survival-limiting complication in adults with homozygous sickle cell disease (SS), but little is known about the development, course, or best treatment of pulmonary hypertension in children with SS. We report the clinical course and treatment of a child with SS-associated, hemolysis-driven pulmonary hypertension.
Vonk M C - - 2009
The prevalence and incidence of systemic sclerosis (SSc) in The Netherlands is unknown. The same holds true for its leading causes of death: pulmonary fibrosis and pulmonary arterial hypertension (PAH), for which effective treatment options have recently become available. OBJECTIVE: To establish the prevalence and incidence of SSc and its ...
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