肺动脉高压是一种慢性持续性肺动脉压升高的疾病。并不伴随明显的左心衰竭。在先天性心脏病中由于左向右的大量分流使肺血流增多而造成。肺动脉高压是渐进的,大部分患者在30～50岁出现症状并死亡。临床上无标准的治疗方案去避免其继续发展。静脉内注射前列腺素,经证明对治疗肺动脉高压有效。肺移植或心肺联合移植对终末期患者或许是最好的治疗方式。但是否有合适的供体是限制这一治疗方案的最主要因素。口服用药对肺动脉高压患者的血流动力学和生存率有较大的改善。本综述通过对成人先天性心脏病并发肺动脉高压的病因与诊断的认识,为其治疗提供指导。 Pulmonary hypertension is a chronic persistent pulmonary hypertension. Not accompanied by significant left ventricular failure. In congenital heart disease due to a large number of shunt left to right to increase pulmonary blood flow caused. Pulmonary hypertension is progressive, most patients 30 to 50 years old symptoms and death. No standard clinical treatment to avoid its continued development. Intravenous injection of prostaglandins has been shown to be effective in treating pulmonary hypertension. Lung transplantation or cardiopulmonary transplantation may be the best treatment for end-stage patients. But whether there is a suitable donor is the most important factor limiting this treatment. Oral administration of pulmonary hypertension in patients with hemodynamic and survival rates have greatly improved. This review provides guidance for the treatment of adult congenital heart disease complicated by pulmonary etiology and diagnosis.