1岁以内完全性肺静脉异位引流外科治疗86例疗效分析

Surgical treatment of total anomalous pulmonary venous connection in infant

目的:回顾性总结婴儿完全性肺静脉异位引流(TAPVC)外科治疗经验及其效果分析,提高手术疗效。方法:回顾分析河北省儿童医院2012年1月至2015年12月TAPVC患儿86例的临床资料,其中男性66例,女性20例,年龄5d^12个月,平均5.11个月;体质量2.3~9 kg,平均(5.67±2.08)kg,均经心脏彩超、胸部X线片、心电图检查确诊,矫治手术均在常规体外循环中低温(28℃左右)下完成。结果:当时住院死亡4例,术后肺静脉狭窄再手术死亡1例,总病死率5.81%。术中心力衰竭死亡2例,严重感染、多器官衰竭1例,低心排出量综合征2例。81例存活者术后复查结果满意,出院后随访3~36个月,身体发育、活动能力正常,无二次手术者。结论:完全性肺静脉异位引流是一种危重先天性心脏病,尤其伴梗阻性的需急症手术治疗,手术矫治可以明显改善患儿的生存质量。

Objective： To review our experience of surgical treatment of total anomalous pulmonary ve- nous connection （TAPVC）. Methods： Retrospectively analyzed the clinical data of 86 patients with TAPVC in Hebei children＇ s hospital during January 2012 and December 2015. There were 66 males and 20 females with a mean age of 5.11 months and a mean body weight of（5.67 ＋ 2. 08 ） kg. All the patients were diagnosed by heart color Doppler ultrasound, X ray and electrocardiogram. The corrections were performed with the aid of car- diopulmonary bypass with moderate systemic hepothermia and HTK cadioplegia. Results： Five cases were dead with a mortality of 5.81%. One of them was performed operation three times because of pulmonary venous steno- sis. Two died in the operating room, because of cardiac failure. One died of infection and multiple orgail dys- function syndrome and two died of low cardiac output syndrome in ICU. The 81 survived patients show a good surgical result by echocardiography examination before hospital discharge. All patients were followed up from 3- 36 months and results were normal. Conclusion： TAPVC is a critical congenital heart disease, which especially with obstruction need to be underwent emergency operations. Radical correction of TAPVC yields good result.