肺动脉高压--PAH_Educational_Slide_Kit.pptVIP

* * PAH: how is severity classified? Assessment of PAH usually includes a functional class rating initially devised by the New York Heart Association (NYHA) for Chronic Heart Failure and then adapted for PAH by the World Health Organisation (WHO). WHO Functional class (WHO FC) measures the severity of PAH and reflects the impact on a patients life in terms of physical activity and symptoms. There are four classes, with WHO FC I being the least severe and WHO FC IV being the most advanced.1 References 1. Barst RJ et al. J Am Coll Cardiol 2004;43 (Suppl S): 40S-47S. * WHO classification of functional (WHO FC) status of patients with pulmonary hypertension Reference Barst RJ A et al. J Am Coll Cardiol 2004; 43(Suppl S): 40S-47S. * PAH: how common is it? PAH is a rare disease, with an estimated prevalence of 30?50 cases per million.1 The prevalence of PAH in certain at-risk groups is substantially higher. For example, in HIV-infected patients the prevalence is 0.46%3; in patients with sickle cell disease the prevalence is 20?40%4; and in patients with systemic sclerosis the prevalence has been reported to be up to 16%.5 Idiopathic PAH is more common in young women with a mean age of diagnosis of 36 years2. References Peacock AJ. BMJ 2003; 326: 835?836. Gaine SP et al. Lancet 1998; 352: 719?725. Sitbon O et al. Am J Resp Crit Care Med 2008; 177: 108-113. Lin EE, Rodgers GP, Gladwin MT. Curr Hematol Rep 2005; 4(2): 117?125. McGoon M et al. Chest 2004; 126(1 Suppl): 14S?34S. * PAH: why does it develop? The exact cause behind the development of PAH remains unknown. However, research has lead to a better understanding of the underlying mechanisms. PAH is recognised as a complex, multi-factorial condition involving numerous biochemical pathways and different cell types. Endothelial dysfunction is believed to occur early on in disease pathogenesis, leading to endothelial and smooth muscle cell proliferation and structural changes or ‘remodelling of the pulmona