完全性大动脉转位重症新生儿临床综合救治策略

Comprehensive management strategy for critical newborns with transposition of great arteries

目的总结内外科联合救治完全性大动脉转位（TGA）重症新生儿的经验。方法2010年10月至2012年10月NICU、小儿心脏专科联合救治17例TGA危重新生儿，回顾性分析其临床救治情况。结果TGA患儿共17例。男14例，女3例；手术年龄1～45d；体质量2．7～4．6kg；9例合并VSD，3例接受前列腺素E治疗，2例入院时存在难以纠正休克、严重低氧血症，急诊大动脉调转术（ASO）治疗；8例为室间隔完整型TGA，全部接受前列腺素E治疗；4例未明显好转，急诊ASO治疗；术后共12例延迟关胸，其中5例为急诊手术患儿；术后腹膜透析6例，体外膜肺氧合（ECMO）辅助1例；胸骨愈合不良6例，急诊ASO患儿均胸骨愈合不良，13例患儿顺利出院，死亡4例，其中l例死于术后手术切口感染并发脓毒症，肺动脉吻合处心包补片感染不愈合，肺动脉大出血死亡。1例术中发现合并有体静脉畸形引流，术后出现严重低心排综合征，手术次13死亡。1例术后手术切口感染，愈合不良，死于脓毒症、弥散性血管内凝血。1例由于术中冠状动脉畸形，冠状动脉移植困难，主动脉开放后心脏心肌供血不良死亡。结论NICU中TGA重症新生儿，术前处理的目的是增加EC—MO，避免急诊手术；如持续存在无法纠正低氧血症或心力衰竭，及时急诊ASO是救治生命的有效手段。

Objective To summarize the clinical experience of comprehensive management of critical newborns with transposition of great arteries（TGA）by neonatologists and cardiac surgeons. Methods Seventeen critical newborns with TGA rescued by combined efforts of neonatologists and pediatric cardiac surgeons from Oct. 2010 to Oct. 2012 were retrospectively analyzed. Results Of the 17 newborns（ 14 cases were male,3 cases were female） ,9 cases were TGA with ventricular septal defect（TGA/VSD）, 8 cases TGA with intact ventricular septal defect （TGA/IVS）. The babies aged from 1 -45 days,weighed from 2.7 -4.6 kg at operation. Of the 9 cases TGA/VSD babies,3 cases were treated with prostaglandins E （PGE） for hypoxemia correction and selective operation opportunity;2 cases underwent emergency arterial switch operation （ASO） for severe cardiac shock. All of the 8 cases TGA/IVS babies received PGE treatment preoperatively for life threatening hypoxemia, and 4 cases of them underwent emergency ASO. There were 12 cases delayed sternal closure and 5 cases of them were emergency babies. Peritoneal dialysis was employed for 6 cases and extracorporeal membrane oxygenation （ECMO） for 1 case. There were 6 cases with postoperative incision infection,inclu- ding 5 emergencies. Thirteen newborns survived and 4 cases died at hospital： one case complicated with postoperative septicemia and severe mediastinitis which leaded to pericardium patch rupture and fatal pulmonary arterial hemorrhage; one case with abnormal inferior vena cava connection and severe low cardiac output syndrome;one case with disseminated intravascular coagulation （DIC） due to septicemia and severe mediastinitis postoperatively, one case with abnormal coronary artery during surgery and difficult coronary artery transplantation and insufficient blood supply after aorta was open. Conclusions Correcting hypoxemia and stabilizing the severe babies are critical in NICU to avoid emergency surgery. Emergent ASO is the optimized medical therapy for those with persistent severe hypoxemia and heart failure.