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目的总结新生儿危重型先天性心脏病(简称先心病)诊断和治疗关键技术在临床应用的经验。方法 2006年1月至2010年12月,新生儿患者369例,平均年龄13.4d,平均体重3.6kg。其中完全性大动脉错位(TGA)310例,完全性肺静脉异位引流(TAPVD)34例,室间隔完整性肺动脉闭锁(PA/IVS)25例。关键技术包括:改进合并冠状动脉畸形TGA的冠状动脉移植方法、改变心上型TAPVD的手术径路、改良PA/IVS的治疗模式;建立胎儿心脏超声,胎儿心脏磁共振诊断技术;健全新生儿围手术期未成熟脏器保护技术。结果 19例TGA合并冠状动脉畸形,大动脉掉转术后因心律失常死亡4例(死亡率21%)。其余291例TGA行大动脉掉转术后因严重低心排血量症死亡7例(死亡率2.4%);心上型TAPVD术后无严重心律失常发生率。PA/IVS心内外科镶嵌治疗组12例,术后因出血死亡1例(死亡率8.3%)。单纯手术治疗组13例,术后因心力衰竭、猝死4例(死亡率31%)。胎儿先心病确诊率由2006年前的70%提高至2010年95%。2004～2010年新生儿心脏术后神经系统并发症如抽搐、手足徐动症的发生率从6%降至0,严重低心排血量综合征发生率从11%降至5%,术后总体死亡率从15%降至4.0%。结论新生儿心脏手术的成功开展,需要有先进的治疗理念、治疗技术及医疗团队。
Objective To summarize the experience of clinical diagnosis and treatment of critical newborn with critical congenital heart disease (CHD). Methods From January 2006 to December 2010, 369 newborns with a mean age of 13.4 days and an average body weight of 3.6 kg were enrolled in this study. Among them, 310 cases of complete aortic atlas (TGA), 34 cases of complete pulmonary venous drainage (TAPVD), and 25 cases of interventricular septum integrity pulmonary atresia (PA / IVS). The key technologies include: improved coronary artery transplantation with coronary TGA; modified surgical approach of supra-cardiac TAPVD; improved the treatment mode of PA / IVS; established fetal cardiac ultrasound and fetal cardiac magnetic resonance diagnosis; completed perinatal neonatal surgery Immature organ protection technology. Results 19 cases of TGA with coronary artery malformations, after the removal of aortic arrhythmias due to death in 4 cases (21% mortality). The remaining 291 TGA patients underwent aorta surgery due to severe low cardiac output of death in 7 cases (mortality 2.4%); no severe cardiac arrhythmia TAPVD incidence. Twelve patients were treated with PA / IVS intracardiac surgery. One patient died of hemorrhage after operation (mortality 8.3%). Thirteen patients were treated by surgery alone, 4 cases died of heart failure (31% mortality) after heart failure. The diagnosis rate of fetal congenital heart disease increased from 70% before 2006 to 95% in 2010. The incidence of neurological complications such as seizures and hand-to-baby asphyxia after neonatal cardiac surgery in 2004-2010 decreased from 6% to 0, and the incidence of severe low cardiac output syndrome decreased from 11% to 5% Overall mortality dropped from 15% to 4.0%. Conclusion Successful neonatal cardiac surgery requires advanced treatment concepts, treatment techniques, and medical team.