2006
DOI: 10.1111/j.1440-1843.2006.00778.x
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Pulmonary arterial hypertension: New insights and new hope

Abstract: Pulmonary arterial hypertension (PAH) is a devastating disorder characterized by abnormal increased vasoconstriction and vascular remodelling. In this review we discuss the pathophysiology, genetic basis and clinical features of this disorder. Current therapy of PAH is based on an understanding of its pathogenesis, and we review current treatment options based on the pathophysiology of the disease. We discuss three promising novel therapies studied in animal models and human tissue. All three therapies appear … Show more

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Cited by 59 publications
(41 citation statements)
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References 93 publications
(95 reference statements)
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“…The 6-min walk test (6MWT), a measure of exercise capacity, and the New York Heart Association (NYHA)/World Health Organization (WHO) functional classification, a measure of severity, are used to follow the clinical course while receiving treatment, and these both correlate with disease severity and prognosis [5]. The aetiology of PAH is incompletely understood; several factors are implicated in its pathogenesis, including genetic predisposition and exposure to toxins and/or inflammatory mediators [6]. Pulmonary vascular tone is maintained by a balance of neural stimuli, oxygen tension, potassium channels and endogenous vasoactive substances, some of which, e.g.…”
Section: Biomarkers In Pulmonary Hypertensionmentioning
confidence: 99%
“…The 6-min walk test (6MWT), a measure of exercise capacity, and the New York Heart Association (NYHA)/World Health Organization (WHO) functional classification, a measure of severity, are used to follow the clinical course while receiving treatment, and these both correlate with disease severity and prognosis [5]. The aetiology of PAH is incompletely understood; several factors are implicated in its pathogenesis, including genetic predisposition and exposure to toxins and/or inflammatory mediators [6]. Pulmonary vascular tone is maintained by a balance of neural stimuli, oxygen tension, potassium channels and endogenous vasoactive substances, some of which, e.g.…”
Section: Biomarkers In Pulmonary Hypertensionmentioning
confidence: 99%
“…Vaistą reikia toliau tirti (3,43). (1,2,8,20,21,33,40,(46)(47)(48)(49) Sildenafilas (žinomas kaip viagra) yra geriamasis vaistas, aktyvus, stiprus ir selektyvus ciklinio guaninmonofosfato (cGMP) -penktojo tipo fosfodiesterazės (PDE) inhibitorius (I, A). Jis ląstelėse didina cGMP koncentraciją.…”
Section: Endotelino-1 Receptorių Antagonistai (Bosentanas Sitaksentaunclassified
“…Elevated pulmonary arterial pressure (PAP) can be observed secondary to heart, lung, or systemic disorders (1). PHT, defined as systolic pulmonary artery pressure (sPAP) ≥35 mmHg at rest as estimated via Doppler echocardiography, has been reported with variable prevalence's in patients with chronic kidney disease (CKD), both predialysis and during hemodialysis (HD) (2).…”
Section: Introductionmentioning
confidence: 99%