58例大血管错位儿童临床分析

目的：探讨儿童大血管错位的诊断和治疗措施。方法：利用彩色多普勒超声心动图和心血管造影对58例大血管错位儿童进行比较、观察。结果：彩超显示卵圆孔未闭8例,心血管造影显示3例;心血管造影显示肺动脉分支狭窄4例,左肺动脉缺如1例,彩超未能显示;降主动脉侧支循环9例,彩超显示4例,1例生后用前列腺素E1持续静脉滴注,1例行房隔造口术,2例行肺动脉环缩术,治疗后血氧饱和度均有改善。结论：彩超顺序分段诊断,大大提高了诊断率,但不能代替心血管造影。采用前列腺素E持续静脉滴注、房隔造口术及肺动脉环缩术能增加血氧饱和度,促进左室发育,对提高根治术成功率可能有重要意义。     

先天性纠正型大动脉错位不同手术方法结果的比较

目的 比较先天性纠正型大动脉错位(ccTGA)的各种手术治疗结果方法,为临床治疗ccTGA提出更合理的手术方式.方法 1999年6月至2009年12月外科手术治疗95例ccTGA,包括传统纠治手术25例,功能性单心室纠治手术26例,双调转术(Double Switch)手术26例,姑息手术15例,另有3例接受其他手术.结果 术后死亡7例,其中传统手术组死亡2例,Double Switch手术组死亡4例,其他手术组死亡1例,单心室手术组全部存活.结论 纠正型大动脉错位的手术方法的选择取决于解剖条件和生理参数.在各类手术结果的比较中,功能性单心室纠治手术的死亡率低、并发症少、再手术率低,可适当放宽功能性单心室纠治手术指征,可能得到更好的治疗效果.但长期的手术疗效还有待于进一步随访.

Abstract：

Objective To study the clinical outcomes of various corrective surgeries for congenitally corrected transposition of the great arteries (ccTGA) in children. Methods Between June 1999 and December 2009, 95 patients with ccTGA who underwent corrective surgeries at this center were enrolled in this study. Among the 95 patients, 25 patients were performed conventional surgical repairs, 26 had functionally univentricular surgical repair, 26 had double switch operation, 15 had palliative surgeries, and 3 underwent other surgical treatments. Results Seven deaths after surgery were recorded, including 2 patients who had conventional surgical repairs, 4 with double switch operation and 1 with other surgical treatments. No death of the patients who underwent functionally univentricular surgical repair was noted. Conclusions The surgical strategy to correct ccTGA should be made according to patient's individual anatomic anomalies and pathophysiology. Short term outcome study revealed that functionally univentricular surgical repair is safe and effective.     

Long-term psychological outcome of children after surgery for transposition of the great arteries

To assess the psychological consequences of a single congenital heart defect, we tested intellectual function, self-perception,'body image", child psychiatric symptoms and the family climate in 21 boys and 10 girls, at a mean age of 13.2y, on average 11.5 y after surgery for transposition of the great arteries. Where applicable, test norms were used for comparison. WISC-R IQ tended to be slightly lower than that of the general population. Self-perception, as reflected on the"I think I am"test, was normal."Body image"as measured by the"Draw-a-man"test was poor in the boys, but did not show a relationship with any other test tapping mental health. Six children (19%) had clinically significant child psychiatric symptoms, which is slightly more than expected, and were overrepresented in patients with poorer cardiac function. Five of these represented"internalizing"disorders. The patients'families scored higher than expected on the family climate"chaos"subscale, which has been demonstrated to be associated with the development of psychiatric symptoms. Overall, however, the children and their families were regarded as socially and psychologically well-functioning.     

Management of congenital diaphragmatic hernia with transposition of the great arteries

Congenital diaphragmatic hernia (CDH) is a relatively rare malformation, but it has a high mortality rate. Its association with congenital heart disease lowers survival rate due to severe acidosis and desaturation caused by persistent pulmonary hypertension of the neonate. We describe herein a case of CDH with transposition of the great arteries and intact ventricular septum, in a patient who was prenatally diagnosed, managed with extracorporeal oxygenation (ECMO) support and successfully treated with both CDH repair and Jatene procedure, with no respiratory or circulatory sequelae. In conclusion, precise prenatal estimation is essential, and ECMO is a useful therapeutic option in these complex cases.     

手术治疗纠正型大动脉错位

目的　总结治疗纠正型大动脉错位的手术方法和经验。方法　 1990年 1月～ 1998年12月 ,手术治疗纠正型大动脉错位 (C TGA) 31例 (占同期手术 0 .6 % )。男性 2 1例 ,女性 10例。手术年龄 2～ 9岁 (平均 5 .2 8± 1.76岁 ) ,体重 7～ 2 2 .5kg(平均 15 .96± 3.78kg)。其中伴左室流出道梗阻2 7例 ,肺动脉高压 4例。 2 4例做双心室修补术 ,其中 12例经形态左心室切口修补室缺 ,死亡 3例 ;12例经形态右心室切口修补室缺 ,死亡 1例。Fontan纠治手术 5例 ,死亡 1例。双向腔肺动脉吻合术 2例 ,无死亡。结果　全组手术死亡率 16.1%。长期随访的疗效不理想 ,主要形态右心室不能长期耐受体循环压力 ,易发生三尖瓣反流。结论　C TGA伴肺动脉高压 ,必须在 1岁以内早期手术。目前双心室手术方法的远期结果尚不理想 ,应以解剖纠治方法为好。     

纠正性大动脉转位病理解剖类型分析

目的 总结16年来收治的73例纠正性大动脉转位(C-TGA)的临床资料，就其解剖类型、合并的心内畸形种类作一分析。方法 根据心脏二维超声心动图、心导管造影检查结果及心脏手术时直视下所见，判断C-TGA的解剖类型及合并的心内结构畸形结果73例患儿中心房正位{S，L.L.}62例，心房反位{I，D，D，}11例；合并其他心内结构畸形72例(99％)．其中室隔完警型8例(11％)，伴有室间隔缺损(VSD)型64例(89％)。室隔完整的C-TGA最多合并的畸形为三尖瓣形态和功能异常．而C-TGA／VSI)最常合并肺动脉狭窄或闭锁。73例患儿中术前发生心律失常13例，30例接受外科手术或介入治疗．术后发生心律失常19例(63％)，其中Ⅲ^* A-VB 13例，6例在术后2周内恢复正常，余7例安装永久性起搏器.结论 C-TGA最常见的解剖类型是{S．L.L．}型，解剖右心室的三尖瓣功能异常和肺动脉狭窄或闭锁是最为多见的并发畸形。     

Nitric oxide in neonatal transposition of the great arteries

Three newborn infants with transposition of the great arteries (TGA) and intact ventricular septum (IVS) developed postnatal persistent pulmonary hypertension of the newborn (PPHN) and were successfully treated with inhaled nitric oxide (iNO). Intervention with balloon atrial septostomy (BAS) was performed in two of the infants before the iNO treatment, but they continued to be severely hypoxic with metabolic acidosis. However, the iNO immediately improved oxygenation and the clinical condition. The third neonate had a moderately large atrial communication and echocardiographic signs of PPHN. He received iNO before BAS with dramatic clinical improvement, which therefore postponed BAS.

Conclusion: Early diagnosis of PPHN and treatment with iNO may improve final outcome in neonates with TGA and IVS. In the presence of moderately large atrial communication and PPHN, treatment with iNO might be considered before BAS.     

完全性大动脉转位患儿外科术后早期并发症和近期随访

Objective To present the short-term outcomes of the patients with transposition of the great arteries after repair. Methods Between 2008 and 2009,71 patients with transposition of the great arteries (TGA) underwent corrective surgeries at this center. The 71 TGA patients were divided into TGA/IVS group whose ventricle septum was intact, and TGA/VSD group who complicated with ventricle septum defect. Their clinical data and postoperative short-term outcomes were analyzed and compared. Results The postoperative complications included 7 (9. 9% ) patients presented with low cardiac output syndrome, 12 ( 16. 9% ) with pulmonary stenosis, 21 ( 29. 6% ) with arrhythmia, 6 (8. 5%) with diaphragmatic paralysis, 6 (8. 5%) with airway abnormalities, and 13 ( 18. 3% ) with bacteria in sputum culture. Three patients (4. 2%) died after surgery. Fifty four out of the 68 patients were followed up for an average period of 12. 0 ± 7. 4 months. Four (7. 4%) patients died during follow-up. Three patients (5. 6%) underwent interventional therapies for pulmonary stenosis. The survival rate of 57 patients was 87. 7% at the end of the first postoperative year. No differences of the postoperative complications and survival rate were noted between the TGA/IVS and TGA/VSD group.Conclusions The postoperative short-term outcomes of the patients with transposition of the great arteries are good.     

Anomalous semilunar valve relationships in transposition of the great arteries

Summary We compared semilunar valve relationships of 26 normal hearts with 66 specimens with transposition of the great arteries (TGA). The TGA hearts exhibited a spectrum of dextroposition of the aorta, which brought the point of fibrous continuity between aortic and pulmonary valves 15 to 120° closer to the tricuspid annulus than in the normal specimen. Aortic dextroposition was associated with malalignment of the posterior semilunar (pulmonary) valve in relation to the aortic valve, the tricuspid and mitral valves, and to the membranous ventricular septum. Supported by HL 20132 from the National Institutes of Health.     

新生儿完全性大动脉错位的急诊球囊房间隔造口术

目的 评价急诊球囊房间隔造口术（BAS）对新生儿大动脉错位患儿抢救生命和姑息治疗的价值。方法 2002年10月至2006年1月，我中心急诊行新生儿大动脉错位房间隔造口术12例，其中11例在心导管室X线透视下完成，1例在超声心动图图像实时导引下完成。对比手术前后心脏超声、动脉血气结果及临床症状。结果 12例患儿术后心脏超声监测示房间隔缺损增大，直径达7～10mm，即刻动脉血氧饱和度升高，达77％～91％，代谢性酸中毒明显改善或得以纠正，临床缺氧症状改善。结论 急诊球囊房间隔造口术对挽救大动脉错位新生儿的生命具有重要价值，并可为行大动脉调转术争取最佳手术时机和尽可能好的术前状态。     

完全性大动脉转位患儿外科术后早期并发症和近期随访

目的 探讨完全性大动脉转位(TGA)患儿术后早期并发症及近期预后情况.方法 对2008年至2009年行手术治疗的71例TGA患儿基本资料进行回顾性分析,并根据室间隔发育情况分为室间隔完整组(TGA/IVS)和室间隔缺损组(TGA/VSD),探讨术后早期并发症及近期预后情况.结果 术后早期出现低心输出量综合征7例(9.9%)、肺动脉狭窄12例(16.9%)、心律失常21例(29.6%)、膈肌麻痹6例(8.5%)、气道异常6例(8.5%)、下呼吸道分泌物培养细菌阳性13例(18.3%);术后院内死亡3例(4.2%).随访68例出院患儿,失访14例(20.6%),余54例(79.4%)随访时间(12.0±7.4)个月,其中4例(7.4%)死亡,3例(5.6%)因肺动脉狭窄需要介入治疗.可随访的57例患儿术后1年存活率87.7%.TGA/IVS组和TGA/VSD组在术后早期并发症和近期存活率方面差异均无统计学意义.结论 TGA患儿术后尽管可能出现一些并发症,但其预后良好,近期存活率较高.     

新生儿完全性大动脉错位的急诊球囊房间隔造口术

目的评价急诊球囊房间隔造口术(BAS)对新生儿大动脉错位患儿抢救生命和姑息治疗的价值。方法2002年10月至2006年1月,我中心急诊行新生儿大动脉错位房间隔造口术12例,其中11例在心导管室X线透视下完成,1例在超声心动图图像实时导引下完成。对比手术前后心脏超声、动脉血气结果及临床症状。结果12例患儿术后心脏超声监测示房间隔缺损增大,直径达7~10mm,即刻动脉血氧饱和度升高,达77%~91%,代谢性酸中毒明显改善或得以纠正,临床缺氧症状改善。结论急诊球囊房间隔造口术对挽救大动脉错位新生儿的生命具有重要价值,并可为行大动脉调转术争取最佳手术时机和尽可能好的术前状态。     

Atrial natriuretic peptide and atrial pressures in newborns with transposition of the great arteries

The relation of atrial pressure to atrial plasma concentration of atrial natriuretic peptide (ANP) was studied in four newborns undergoing catheterization because of transposition of the great arteries. In three patients, mean left atrial pressure clearly exceeded right atrial pressure (12 +/- 7 vs. 2 +/- 3 mmHg; mean +/- SD). In one patient mean atrial pressures were identical (4 mmHg). In all patients the ANP concentration in the left atrium exceeded that of the right (833 +/- 464 vs. 415 +/- 366 pg/ml; mean +/- SD). There was a significant (r = 0.97; p less than 0.01) correlation between pressure and ANP concentration in the left atria. In the right atria, no linear correlation existed between pressure and ANP concentration. ANP concentration in the left atrium decreased after a lowering of the left atrial pressure by atrial septostomy. In these patients, the left atrium seems to be the main source of circulating ANP.