室间隔完整型肺动脉闭锁的诊断及介入治疗进展

Advances of diagnosis and catheter intervention of pulmonary atresia with intact ventricular septum

室间隔完整型肺动脉闭锁(PA/IVS)是新生儿期危重性青紫型先天性心脏病(CHD),自然病死率极高,需在出生后早期干预治疗。随着产前诊断技术及内外科治疗技术的提高,PA/IVS患儿的生存率及预后有了明显的改善。该文回顾并总结了PA/IVS诊断及治疗的新进展,旨在为该病的临床治疗提供参考。

Pulmonary atresia with intact ventricular septum （PA/IVS） is a critical cyanotic congenital heart disease with high mortality in neonates and infants. Interventions must be given at early stage after birth. The survival rate and prognosis of patients with PA/IVS have been significantly improved with the development of prenatal diagnosis techniques and therapeutic techniques of medicine and surgery. This paper reviews and summarizes the progresses in diagnosis and treatment of PA/IVS to provide a reference for the clinical treatment of the disease.