成人先天性心脏病肺动脉高压的新认识

成人先天性心脏病患者表现为一个正在扩大的并需要终身接受3级护理的群体。一些成人先心病的患者最后发展为不同程度的肺动脉高压,影响患者的生存质量,发病率和病死率。然而近期对肺动脉高压病理生理学理解的提高和靶向治疗的出现使人们在成人先天性心脏病肺动脉高压患者病因学、临床表现、预后和治疗策略方面有了新的认识。

New Recognition about Pulmonary Artery Hypertension in Adults with Congenital Heart Disease

Adults with congenital heart disease represent an expanding patient population requiring life-long tertiary medical care.Some patients with congenital heart disease develop pulmonary arterial hypertension(PAH)of variable severity that affects quality of life,morbidity,and mortality.Recently,however,an improved pathophysiological understanding,and the advent of disease targeting therapies for PAH have led to a new recognition in etiology,clinical presentation,prognosis,and management strategies of pulmonary arterial hypertension in adult patients with congenital heart disease.