完全性肺静脉异位引流手术50例治疗经验总结

Summarized the experience on 50 cases treated with total anomalous pulmonary venous drainage operation

目的:探讨梗阻型完全性肺静脉异位引流的解剖、分型、个体化手术方法。方法:2009年6月-2013年11月收治完全性肺静脉异位引流患儿50例,其中心上型26例,心内型19例,心下型3例,混合型2例为心上心内对称型混合,梗阻型33例行急诊手术。术前均行胸片、心电图及超声心动图检查,部分病例经心脏64排CT增强核磁共振检查。术式:典型心上型一般采用左房顶径路吻合,不同亚型采用Warden术式,Stuless吻合技术;心内型缝合心包补片把肺静脉隔到左房,必要时扩大肺静脉开口。心下型采用心脏右侧心右途径;混合型根据类型选择相应的术式。结果:50例均行手术治疗,治愈45例,治愈率90%,院内死亡3例,死亡率6%,死亡原因为吻合口出血、低心排综合征、肺高压危象、肺炎并呼吸衰竭、多脏器功能衰竭。2例花费巨大,肺炎未治愈自动出院。1例合并左侧大脑中动脉栓塞转康复科继续治疗。结论:完全性肺静脉异位引流隔阻率高,急诊手术多见,肺静脉解剖亚型较多,变异复杂,正确认识其亚型,制定个体化手术方案,有助于提高治疗效果。

Objective:To investigate the anatomy,typing and individual operation method of total anomalous pulmonary venous connection with obstruction type.Methods:50 children with total anomalous pulmonary venous drainage were selected from June2009 to November 2013,in which,there were 26 cases of the center type,19 cases of intracardiac type,3 cases of heart type,2 cases of mixed type with up and inside of the heart symmetric mixed.33 cases of obstruction type were taken emergency operation.All the patients underwent chest X-ray electrocardiogram and ultrasound beckoning graph examination before the operation,part of the cases were taken 64 rows of heart CT enhanced mri.Operation:the typical heart type:left atrium pathway anastomosis were generally used,different subtypes using warden operation or stuless anastomosis;intracardiac type:suturing pericardium to isolate the pulmonary vein into the left atrium and pulmonary vein,and expand pulmonary vein opening when necessary.The infracardiac type used on the right side of the heart under right way;mixed type selected procedure according to the types.Results:50 cases were treated with operation,in which 45 cases were cured,the cure rate was 90%,3 cases died in the hospital,and the mortality was6%,the causes of death were anastomotic bleeding,low cardiac output syndrome,pulmonary hypertension crisis,pneumonia and respiratory failure,multiple organ failure.2 cases with immerse expense,pneumonia is not cured and discharged from hospital.In 1cases with left middle cerebral artery embolism transferred to the physiatry department to continue treatment.Conclusion:The blocking rate of total anomalous pulmonary venous connection was high,its very common in the emergency operation,pulmonary vein anatomy with more complex subtype,and always variated,correctly understanding of its subtypes and making individualized operation schemes,can benifit to improving the therapeutic effect.