新生儿危重先天性心脏病的早期干预

目的：探讨新生儿危重先天性心脏病(先心病)的早期识别及早期干预方法。方法回顾性分析2010年1月至2014年12月阜外医院 PICU 收治的223例出生日龄≤30 d 的新生儿危重先心病病例，分析其畸形种类、临床特征及早期干预经验。结果223例先心病患儿以完全性大动脉转位及完全性肺静脉异位引流最为常见，分别占59%(131例)和17%(39例)。首诊症状以紫绀、心脏杂音和呼吸困难最为常见，分别占91%(204例)、56%(125例)和53%(118例)。术前因严重缺氧、代谢性酸中毒及心力衰竭而急诊收住 PICU 抢救59例。早期干预主要包括维持动脉导管开放、纠正内环境紊乱、治疗心力衰竭等，并尽早手术治疗。14例患儿未接受手术，其中4例术前死亡，另10例放弃治疗并均于出院后12 d 内死亡。最终接受手术209例，手术死亡9例，手术死亡率4.3%。随访3~63个月，远期死亡2例，二期解剖矫治手术2例，再次手术3例。其余术后患儿心功能正常，生长发育接近正常同龄儿童。结论必须在新生儿期进行干预的危重先心病主要包括动脉导管依赖型先心病，其中以完全性大动脉转位、完全性肺静脉异位引流最为多见，如不积极手术治疗，大多于新生儿期死亡。早期识别，适当的术前处理，及时转诊和早期手术是挽救这类先心病患儿的关键。

Early intervention of life-threatening cardiac malformations in the newborn

Objective To investigate the methods of early identification and early intervention for newborn with life-threatening congenital heart disease.Methods Between January 2010 and December 2010,223 neonates with serious congenital cardiac malformations were hospitalized in PICU of Fuwai Hospi-tal.Results The most type of cardiac lesions was complete transposition of the great arteries,accounting for 59%(131 cases),and the second was total anomalous pulmonary venous connection,17%(39 cases).For the primary clinical symptoms,the most common were any cyanosis,dyspnea and cardiac murmur,accounting for 91 %(204 cases),56%(125 cases)and 53%(1 18 cases),respectively.Fifty-nine cases developed into critical conditions such as severe hypoxia,metabolic acidosis and heart failure and were sent to PICU for emergency rescue.Early intervention included maintaining ductus arteriosus open,correcting internal environ-ment disturbances,treatment of heart failure,and surgical treatment as soon as possible.Four cases died before operation and 10 cases were abandoned to continue care,which all died within 12 days after discharge.In 209 cases who received operation,9 cases died.The total operation mortality was 4.3%.Within 3 to 63 month following-up,the late death was in 2 cases,2 cases received two-stage corrective operation,and three for reop-eration.The others all were in normal cardiac function and growth.Conclusion Most of neonatal life-threat-ening congenital cardiac malformations were ductus dependent such as transposition of the great arteries and total anomalous pulmonary venous connection,which the baby needs immediate diagnosis and management for survival.Early recognition,appropriate preoperative management and operation as soon as possible are the key to rescue.