肺动脉高压是临床表象,实质是肺小动脉增殖生长失控而导致管腔重构性闭塞,其临床结局多以右心室功能衰竭而导致死亡。患者预后取决于治疗策略是否准确。肺动脉内皮功能受损之后,内源性前列环素逐渐分泌下降,因此补充前列环素就成为治疗这种疾病的一个思路。自1992年依前列 Pulmonary hypertension is a clinical manifestation of the essence of uncontrolled proliferation of pulmonary arterioles lead to lumen remodeling occlusion, the clinical outcome of more than the right ventricular failure and death. The prognosis of patients depends on the accuracy of the treatment strategy. Pulmonary artery endothelial dysfunction, the gradual secretion of endogenous prostacyclin decreased, so add prostacyclin has become an idea for the treatment of this disease. Since 1992, according to the forefront