产前产后一体化诊治大动脉转位畸形5例

目的 总结产前和产后一体化诊治大动脉转位畸形(TGA)的经验.方法 5位孕妇在产前诊断中心确诊胎儿TGA,愿意继续妊娠.孕妇平均年龄(28.4±3.0)岁,产前诊断时胎龄(28.4±4.4)孕周.妊娠(36.5±1.8)孕周分娩,新生儿体重(2468±442)g.新生儿接受超声心动图复查,TGA合并室间隔缺损2例,室间隔完整3例.针对严重缺氧患儿,给予前列腺素和呼吸机辅助呼吸.完善术前准备,均在中低温体外循环下完成大动脉调转术,同时矫治合并心脏畸形.结果 患儿生后2～19天,平均(9.0±6.2)天手术.生存3例.死亡2例,1例早产低体重(1770 g)儿,术后第2天心搏骤停,复苏失败;1例Apgar评分低早产儿术前使用呼吸机和前列腺素,生后第2天急诊手术,术后顽固性低心排血量,第3天死亡.结论 TGA产前和产后一体化诊治有利于减少患儿术前缺氧,避免长途转运,但是需要多科室合作,任何环节的不足将会抵消产前诊断给TGA患儿带来的益处.

Abstract：

Objective To summarize primary experiences of integrated fetal diagnosis and postnatal treatment for the transposition of the great arteries (TGA). Methods Five fetus were diagnozed as TGA at(28.4 ±4.4) weeks of gestation via fetal echocardiography. The mean age of the pregnant women was (28.4 ±3.0) years old. Delivers were taken by caesarean at (36.5 ±1.8) weeks of gestation. The body weight of neonates was (2468 ±442) grams. All neonates were transported to the department of neonatology and re-checked by echocardiography. 3 cases were TGA with intact ventricular septum, 2 cases were TGA with ventricular septal defect. Prostaglandin and mechanical ventilation were applied if oxygen saturation was lower. The standard arterial switch procedure was performed under cardiopulmonary bypass with moderate hypothermia. Results The mean age for patients at surgery was (9. 0 ±6. 2) days ( ranged 2-19 days). Three neonates survived, 2 preterm neonates died. One with body weight 1770 g, 2 days after operation was died of sudden heart arrest and failure of resuscitation. Another was treated by mechanical ventilation and prostaglandin after delivery and underwent operation at the second postnatal day, the neonate appeared low cardiac output and high serum lactate postoperatively and died in the third day. Conclusion The integrated fetal diagnosis and postnatal treatment for TGA could prevent severe cyanosis and emergency transportation after parturition. The lack of any link in the cooperation among the multidisciplinary team could affect the benefits for the TGA neonates provided by prenatal diagnosis.

Integrated fetal diagnosis and postnatal treatment for the transposition of the great arteries

Objective To summarize primary experiences of integrated fetal diagnosis and postnatal treatment for the transposition of the great arteries (TGA). Methods Five fetus were diagnozed as TGA at(28.4 ±4.4) weeks of gestation via fetal echocardiography. The mean age of the pregnant women was (28.4 ±3.0) years old. Delivers were taken by caesarean at (36.5 ±1.8) weeks of gestation. The body weight of neonates was (2468 ±442) grams. All neonates were transported to the department of neonatology and re-checked by echocardiography. 3 cases were TGA with intact ventricular septum, 2 cases were TGA with ventricular septal defect. Prostaglandin and mechanical ventilation were applied if oxygen saturation was lower. The standard arterial switch procedure was performed under cardiopulmonary bypass with moderate hypothermia. Results The mean age for patients at surgery was (9. 0 ±6. 2) days ( ranged 2-19 days). Three neonates survived, 2 preterm neonates died. One with body weight 1770 g, 2 days after operation was died of sudden heart arrest and failure of resuscitation. Another was treated by mechanical ventilation and prostaglandin after delivery and underwent operation at the second postnatal day, the neonate appeared low cardiac output and high serum lactate postoperatively and died in the third day. Conclusion The integrated fetal diagnosis and postnatal treatment for TGA could prevent severe cyanosis and emergency transportation after parturition. The lack of any link in the cooperation among the multidisciplinary team could affect the benefits for the TGA neonates provided by prenatal diagnosis.