Serino G, Giacomazzi F
Dipartimento Medicina Interna e Medicina Vascolare IRCCS Policlinico San Donato (Milano), Italy.
Pediatr Med Chir. 2010 Nov-Dec;32(6):274-9.
Pulmonary Hypertension (PH) is definited by a mean pulmonary artery pressure (PAPm) >25 mmHg at rest. The Dana Point 2008 Revised Classification System represents the most recent classification system update with respect of various etiologies of PH. About 10 % of adolescents or adults with uncorrected congenital heart disease (CHD) with left-to-right shunt and high pulmonary blood flow develop Pulmonary Arterial Hypertension (PAH) . Progressive vascular remodeling and increase in pulmonary vascular resistance (PVR) may ultimately lead to reversal of the shunt (pulmonary to systemic) causing cyanosis and determining the so-called Eisenmenger Syndrome (ES). Recent advances in the early diagnosis and medical targeted treatment of adult patients with CHD-PAH and ES can improve PAP, PVR and exercise tolerance, together with NYHA Class and survival, and may potentially reverse the vascular remodeling process in selected patients.
肺动脉高压（PH）定义为静息时平均肺动脉压（PAPm）>25 mmHg。《2008年达纳点修订分类系统》是关于PH各种病因的最新分类系统更新。约10%未矫正的左向右分流且肺血流量高的先天性心脏病（CHD）青少年或成人会发生肺动脉高压（PAH）。进行性血管重塑和肺血管阻力（PVR）增加最终可能导致分流逆转（从肺到体循环），引起发绀并导致所谓的艾森曼格综合征（ES）。成人CHD-PAH和ES患者早期诊断和医学靶向治疗的最新进展可改善PAP、PVR和运动耐量，以及纽约心脏协会（NYHA）心功能分级和生存率，并且可能在部分患者中逆转血管重塑过程。
