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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。
Adults with congenital heart disease manifest as an expanding group that needs life-long Level 3 care. Some adult patients with congenital heart disease eventually developed to varying degrees of pulmonary hypertension, affecting the quality of life of patients, morbidity and mortality. However, the recent understanding of the pathophysiology of pulmonary hypertension and the emergence of targeted therapies have led to a new understanding of the etiology, clinical presentation, prognosis and treatment strategies of patients with pulmonary hypertension in adults with congenital heart disease.