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Idiopathic Pulmonary Arterial Hypertension
Seminars in Respiratory and Critical Care Medicine, 2013Idiopathic pulmonary arterial hypertension (IPAH), formerly called primary pulmonary hypertension, is a rare disease (incidence and prevalence rates of approximately one and six cases per million inhabitants, respectively) with different clinical phenotypes.
Rogerio, Souza +2 more
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Idiopathic pulmonary arterial hypertension in children
Current Opinion in Pediatrics, 2005Until recently, the diagnosis of idiopathic pulmonary arterial hypertension was virtually a death sentence, particularly for children. Although there is no cure for idiopathic pulmonary arterial hypertension, recent medical advances have dramatically changed the course of this disease in children.
Erika Berman, Rosenzweig, Robyn J, Barst
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Iloprost for idiopathic pulmonary arterial hypertension
Expert Review of Cardiovascular Therapy, 2005Idiopathic pulmonary arterial hypertension is a rare but serious and life-threatening disease that leads to right heart failure and death within 2.8 years without specific treatment. This review focuses on the stable prostacyclin analog iloprost, its biologic action and pharmacology and, finally, on its clinical development, efficacy and safety in ...
Hanno H, Leuchte, Jürgen, Behr
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Pulmonary Lymphoid Neogenesis in Idiopathic Pulmonary Arterial Hypertension
American Journal of Respiratory and Critical Care Medicine, 2012Patients with idiopathic pulmonary arterial hypertension (IPAH) present circulating autoantibodies against vascular wall components. Pathogenic antibodies may be generated in tertiary (ectopic) lymphoid tissues (tLTs).To assess the frequency of tLTs in IPAH lungs, as compared with control subjects and flow-induced PAH in patients with Eisenmenger ...
Perros, Frederic +11 more
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Idiopathic Pulmonary Arterial Hypertension: Evolving Therapeutic Strategies
Seminars in Respiratory and Critical Care Medicine, 2017AbstractThe field of pulmonary arterial hypertension (PAH) has seen substantial changes in the epidemiology, associated comorbidities, treatment algorithms, and prognosis in the past decade. Specifically for idiopathic PAH (IPAH), there has been a noted increase in the age of initial diagnosis.
Manreet, Kanwar +3 more
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Idiopathic pulmonary arterial hypertension with coexisting CHD
Cardiology in the Young, 2018AbstractSinus venosus atrial septal defect can result in an increase in pulmonary blood flow and vascular resistance, leading to pulmonary hypertension. Rarely, the degree of pulmonary hypertension is out of proportion to the degree of intra-cardiac shunting.
Thomas G. Day +2 more
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Idiopathic Pulmonary Arterial Hypertension in Childhood
Cardiology in Review, 2010The introduction of new medicines to treat pulmonary vascular disease has renewed interest in the management of children with idiopathic pulmonary arterial hypertension. We now have a practical classification of pulmonary hypertension, improved diagnostic techniques, and internationally recognized diagnostic and management guidelines.
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Idiopathic Pulmonary Arterial Hypertension Misdiagnosed as Asthma
Journal of Asthma, 2007Idiopathic pulmonary arterial hypertension (IPAH) is a rare disorder that is progressive and often leads to right heart failure if left untreated. Because of the vague nature of symptoms at presentation, IPAH may take several months to diagnose. The most common presenting complaint in patients with IPAH is dyspnea with exertion, which is also commonly ...
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Idiopathic and Familial Pulmonary Arterial Hypertension
2010Pulmonary arterial hypertension (PAH) is a progressive, fatal disease that is defined hemodynamically. The average life expectancy after diagnosis is short, with death usually due to progressive right ventricular hypertrophy and right heart failure. PAH results from vasoconstriction and structural alterations to the pulmonary vasculature.
Jean M. Elwing +3 more
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Giant Pulmonary Artery Aneurysm Caused by Idiopathic Pulmonary Artery Hypertension
The Annals of Thoracic Surgery, 2020Semih, Kalkan +4 more
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