小儿室间隔完整型肺动脉闭锁治疗进展

Advances of treatment of pulmonary atresia with intact ventricular septum

室间隔完整型肺动脉闭锁（plumonary atresia with intact ventricular septum，PA／IVS）是一种罕见、危重、复杂的紫绀型先天性心脏病，该病自然病死率高，如不予治疗，50％患儿出生后2周内死亡，约85％半年内会死亡。近年来随着手术、介入等治疗水平的提高，5年生存率提高到75％以上。本文就PA／IVS的手术治疗、介入治疗以及镶嵌治疗的最新进展进行综述。

Pulmonary atresia with intact ventricular septum （PA/IVS） is a rare, critical and complicated cyanotic congenital heart disease, the natural mortality rate is high, if not treated, 50% of newborns died within 2 weeks after birth, about 85 % of newborns will die within six months, In recent years, with cardiac surgery procedures, catheter intervention and other treatment levels improved, the survival rate of newborns after surgery has improved. Over the past decade, the disease 5-year survival rate increased by 75% or more . In this paper, PA/IVS cardiac surgery procedures, catheter intervention and hybrid treatment were reviewed, aimed at providing a reference for clinical treatment.