前列腺素E1治疗新生儿完全性大动脉转位的疗效

目的了解前列腺素E1治疗新生儿完全性大动脉转位时在维持血氧饱和度、延长患儿存活时间方面的作用。方法19例大动脉错位患儿分为两组。治疗组11例采用前列地尔静脉24h持续滴注,5~20ng/(kg·min);对照组8例采用一般治疗。用药期间监测患儿体温、血氧饱和度、心率、血压等指标。结果治疗组血氧饱和度为(82.30±3.56)%,对照组为(67·30±4.54)%,二者比较有显著性差异(t=8.232P=0.001),其存活时间明显长于对照组;治疗组体温、心率、血压等指标未见明显波动。结论前列腺素E1对维持完全性大动脉转位患儿的动脉导管开放、改善血氧饱和度、延长存活时间具有良好作用。     

纠正型大动脉转位的治疗进展

纠正型大动脉转位是一种较为罕见的复杂先天性心脏病,常伴有室间隔缺损、肺动脉狭窄或闭锁、三尖瓣Ebstein样畸形以及中位心等.其病理解剖的基本特征是心房心室连接不一致,心室大动脉连接不一致.1957年开展了生理性纠正手术,二十世纪八十年代末期开展了解剖性纠正手术,即双调转手术,包括心房调转加动脉调转手术和心房调转加心室...     

完全性大动脉错位动脉转位术后新主肺动脉发育的随访

目的 报道应用动脉转位术（ASO）治疗完全性大动脉错位（TGA）术后超声心动图的随访结果,评价TGA术后主、肺动脉的发育情况。方法 以2001年3月至2007年3月于复旦大学附属儿科医院心血管中心实施ASO治疗TGA术后存活患儿作为研究对象,于2008年8～9月进行随访,行超声心动图检查,测定新主动脉根部内径、新主动脉瓣环内径、新肺动脉内径和新肺动脉瓣环内径,获取的数据与正常参考值做比较。 结果 研究期间ASO治疗TGA患儿共72例,术中死亡6/72例（8.3％）,存活率为91.7%。失访10/66例,有效数据56例,其中室间隔完整的完全性TGA（TGA/IVS）32例,完全性TGA合并室间隔缺损（TGA/VSD）24例。随访距手术18～168(86.46±23.51)个月。新主动脉根部内径为(16.97±2.71) mm(t=6.936,P<0.001),12/56例(21.4%)>正常参考值90%CI的上限, 44/56例(78.6%)在正常参考值90%CI内,平均Z值为1.10±0.70。新主动脉瓣环内径为(16.27±2.38) mm(t=4.52,P<0.001),56例均在正常参考值90%CI内,平均Z值为0.66±0.65。新肺动脉内径为(14.29±1.92) mm(t=-3.2,P=0.005), 40/56例(71.4%)在正常参考值90%CI内,16/56例(28.6%)<正常参考值90%CI的下限,平均Z值为-0.95±1.33。 新肺动脉瓣环内径为(14.00±1.92) mm(t=0.132,P=0.897), 42/56例(75.0%)在正常参考值90%CI内,14/56例(25.0%)<正常参考值90%CI的下限,平均Z值为0.05±1.85。16/56例(28.6%)存有主动脉瓣轻度反流,均为TGA/VSD病例,平均随访(46.0±22.2)个月;40/56例（71.4％）未见主动脉瓣反流,平均随访(46.8±25.3)个月,两组随访时间差异无统计学意义（P=0.899）。随访中未见主动脉狭窄病例。肺动脉瓣上狭窄7/56例(12.5%),2/56例(3.6%)为轻度狭窄,5/56例(8.9%)为极轻度狭窄。结论 目前在复旦大学附属儿科医院心血管中心开展ASO治疗TGA术后新主动脉根部和瓣环内径均有所扩张,新肺动脉根部内径有所缩减,但其瓣环发育良好。主动脉瓣反流常见于TGA/VSD病例,但其反流程度较轻。肺动脉瓣上狭窄是TGA术后常见的并发症,但大多数病例狭窄程度极轻。     

米力农对小儿先天性心脏病并心力衰竭术前血液动力学干预的研究

目的探讨米力农对小儿先天性心脏病(CHD)并心力衰竭(CHF)术前血液动力学干预的效应。方法将80例患儿随机分为A、B、C、D四组,A、B、C组为米力农组,米力农剂量分别为0.25、0.5和0.75μg/(kg.min),D组为多巴胺组,剂量为5μg/(kg.min),各组持续给药3 d,观察不同维持量的米力农其血液动力学和心功能指标的变化并与多巴胺组进行比较。结果米力农各剂量组于用药后均可使肺动脉压力下降(P<0.01~0.05),以中剂量和大剂量组效果最满意(P<0.01),心率、血压无显著变化(P>0.05),心脏收缩和舒张功能指标LVEF、LVSF、LVSTI、RVSTI及房室瓣E/A值均有显著改善(P<0.01~0.05),有2例出现室性早搏(3.3%);多巴胺组用药前后上述指标均无显著性差异(P>0.05)。结论米力农对CHD并CHF患儿具有正性肌力和扩血管作用,能明显改善心功能和降低肺动脉压力,不影响血压和心率。     

利尿剂的给药方式对心内直视手术后婴儿血液动力学影响

目的　评价利尿剂的不同给药方式对 5kg以下心内直视手术后房、室间隔缺损婴儿围术期血液动力学的影响。方法　将 2 4例 5kg以下心内直视手术后围术期需利尿剂治疗的房、室间隔缺损婴儿分成A、B两组 ,分别采用速尿持续和间断静脉注射两种不同的给药方式 ,在单位时间每公斤体重速尿静脉注射剂量相等 ,观察给药期间婴儿血液动力学指标左房压 (PLA) ,中心静脉压(CVP) ,动脉压 (PAO)的变化。结果　单位时间每公斤体重速尿静脉注射剂量相等情况下 ,B组速尿间断静脉注射后婴儿血液动力学指标PLA ,CVP ,PAO出现显著性变化 (P <0 .0 5 ) ;A组速尿持续静脉注射后PLA、CVP、PAO的变化无显著性差异。结论　采用速尿持续静脉注射的给药方式对血液动力学指标PLA、CVP、PAO的影响小 ,PAO波动不大 ,符合病理生理的要求 ,术后液体平衡容易控制 ,该方法比较适合于 5kg以下婴儿心内直视手术后围术期的利尿剂治疗。     

婴幼儿支气管肺炎合并心力衰竭的血液动力学改变及治疗研究

报告２７例婴幼儿重症肺炎并心力衰竭（简称心衰）与２７例同龄健康儿童配对对比，其心脏指数（ＣＩ）、主动脉最大流速（ＰＦＶＡ）、肺动脉最大流速（ＰＦＶＰ）显著下降，二尖瓣Ｅ峰最大流速（ＰＦＶＭＥ）、三尖瓣Ｅ峰最大流速（ＰＦＶＴＥ）显著增加。２１例肺炎心衰患儿用酚妥拉明治疗前后对比，ＰＦＶＰ、ＰＦＶＡ、ＣＩ显著增加．ＰＦＶＴＥ、ＰＦＶＭＥ无显著改变。２０例肺炎心衰患儿用地高辛治疗前和治疗后２４小时、４８小时对比，Ｃｌ、ＰＦＶＡ、ＰＦＶＰ显著增加，ＰＦＶＭＥ、ＰＦＶＴＥ显著减小。提示对肺炎心衰患儿，除血压下降者外，可先用酚妥拉明，随之开始用地高辛治疗，可取得满意疗效。     

心力衰竭时内分泌改变与血液动力学关系的研究

对２０例健康儿童、２３例心衰病儿检查血浆肾素活性（ＰＲＡ）、血管紧张素Ⅱ（ＡＴⅡ）、醛固酮（ＡＬＤ）、心钠素（ＡＮＦ）。心衰病儿上述四项内分泌素显著高于健康儿童，其升高程度与心衰程度相一致，并分别分析他们与心脏指数（ＣＩ）、主动脉峰值流速（ＰＦＶＡ）、肺动脉峰值流速（ＰＦＶＰ）的关系。通过Ｂａｙｅ判别，Ｙ０＝－１５．９２００＋１２．１５３７ｌｏｇＡＴⅡ＋６．０９９４ｌｏｇＡＮＦ，Ｙ１＝－２７．０６８６＋１５．３５２２ｌｏｇＡＴⅡ＋８．３４６７ＡＮＦ，Ｙ０＞Ｙ１为正常；Ｙ１＞Ｙ０为心衰。     

高容量血液滤过对小儿脓毒症炎症因子及血流动力学影响的研究

目的 研究高容量血液滤过对脓毒症患儿血液中炎症因子的清除效率及血流动力学改善方面的效果,探讨高容量血液滤过治疗小儿脓毒症的可行性.方法 选取34例脓毒症患儿分为两组,一组是进行常规治疗的对照组(17例),另一组是在常规治疗基础上进行高容量血液滤过治疗的观察组(17例),比较两组治疗前后血液中炎症因子及血流动力学指标的变化情况.结果 (1)治疗前对照组和观察组患儿血浆中肿瘤坏死因子(tumor necrosis factor,TNF)-α分别为(103.6±26.1)ng/L、(106.6±31.6) ng/L,白细胞介素(interleukin,IL)-6分别为(772.8±92.4) ng/L、(761.3 ±89.2)ng/L,治疗后对照组和观察组患儿血浆中TNF-α和IL-6水平均显著降低(P＜0.05),其中TNF-α分别降为(85.6±23.7)ng/L、(63.6±26.4) ng/L,IL-6分别降为(109.7 ±38.5) ng/L、(78.1±24.7)ng/L,且治疗后观察组中的TNF-α和IL-6水平比对照组降低更加明显(P＜0.05).(2)治疗前对照组和观察组患儿血流动力学指标,包括平均动脉压、血氧饱和度及心率差异均无统计学意义(P＞0.05),治疗后观察组的平均动脉压(100.4 ±12.1)mmHg(1 mmHg =0.133 kPa)和血氧饱和度(94.2±5.3)％均显著高于对照组的(82.2±11.7) mmHg、(91.4±3.2)％,观察组的心率(85.1±17.3)次/min显著低于对照组的(98.7±18.5)次/min(P ＜0.05).结论 高容量血液滤过治疗能够有效地滤除脓毒症患儿血液中的炎症因子,改善患儿的血流动力学,这对于保护脓毒症患儿的脏器功能以及预后均有十分重要的意义.     

Pulmonary hypertension in patients with complete transposition of the great arteries: Midterm results after surgery

Postoperative results of surgical repair of complete transposition of the great arteries (TGA) with pulmonary hypertension (PH) in 19 patients in whom mean pulmonary arterial pressure was >50 mmHg or the pulmonary/systemic arterial pressure ratio was >0.8 were examined. TGA with intact ventricular septum was diagnosed in 10 patients and TGA with ventricular septal defect in 9. At the time of corrective surgery (arterial switch 10, atrial switch 9), patients ranged in age from 7 months to 14 years (mean 2.4 years). Seventeen patients (89%) survived with New York Heart Association functional class I. Seven patients in whom calculated pulmonary vascular resistance was 10–20 U · m² after surgery survived with subsequent regression of PH. Residual PH was diagnosed in 36% of survivors. Two patients in whom pulmonary vascular resistance was 26 and 36 U · m², respectively, died after surgery. In patients with TGA, severe PH was not necessarily fatal and was potentially reversible after successful surgery.     

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.     

Status of survivors after atrial redirection for transposition of the great arteries: a complete long-term follow-up

All 32 survivors with transposition of the great arteries, born in 1964-83 and operated on at our institution using atrial redirection, were evaluated by cardiac catheterization, echocardiography and Holter monitoring. There were 17 Mustard patients, age 17.1 years (±3.5, 12.0-22.0) and 15 Senning patients, age 9.4 years (±1.6, 7.2-12.1). All but one had simple transposition. Six had caval obstruction, one had pulmonary venous obstruction, three had large atrial shunts, four had considerable pulmonary hypertension, seven had mild ventricular outflow tract obstruction, four had significant tricuspid regurgitation, 11 had systemic ventricle dysfunction (one severe), 14 had sinus node dysfunction (three symptomatic) and two had atrioventricular block (one with pacemaker). Eight Mustard patients (47%) and one Senning patient (7%) had symptomatic cardiac sequelae, and only one patient (Senning) was free from sequelae, illustrating that these patient groups will need continuing medical attention.     

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。     

Aortic atresia and tricuspid atresia occurring in complete transposition of the great vessels

Summary A unique case of aortic atresia and tricuspid atresia associated with transposition of the great vessels is described in a newborn infant. The clinical, echocardiographic, and heart catheterization data are presented. The embryological and surgical implications are discussed.     

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.     

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.     

动脉调转术治疗新生儿室间隔完整型大动脉转位及危险因素

目的 分析动脉调转术治疗新生儿室间隔完整型大动脉转位(D-TGA/IVS)临床结果及危险因素.方法 回顾分析上海儿童医学中心2000年1月至2006年12月共62例行动脉调转术治疗的D-TGA/IVS新生儿围术期临床结果.手术年龄15 h～28 d,平均(8.91±8.04)d,体重2.30～4.40 kg,平均(3.34±0.44)kg.手术均在深低温低流量体外循环下和深低温停循环下进行.结果 全组死亡6例,住院病死率9.7%.其中急诊手术组和非急诊手术组病死率分别为15.6%(5/32)和3.3%(1/30),小年龄组(年龄≤14 d)和大年龄组(年龄15～28 d)病死率分别为8.0%(4/50)和16.7%(2/12),冷晶体停搏液组和冷含血晶体停搏液组病死率分别为4.0%(1/25)和13.5%(5/37).典型冠状动脉组和非典型冠状动脉组病死率分别为8.3%(4/48)和14.3%(2/14).对应各组间病死率差异均无统计学意义.急诊手术组(148.52±193.98)与非急诊手术组(66.38±34.58)的术后插管时间差异有统计学意义(P=0.039),重症监护时间(P=0.088)、术后住院时间(P=0.100)差异无统计学意义;小年龄组与大年龄组、晶体停搏液组与含血停搏液组、典型冠状动脉组与非典型冠状动脉组的术后插管时间、重症监护时间、术后住院时间差异均无统计学意义.结论 新生儿DTGA/IVS行动脉调转术治疗,效果满意.术前明确诊断,纠正酸中毒、维持动脉导管开放,积极改善术前一般情况,术中精心的手术设计和操作,术后应用综合治疗措施,积极防治各种并发症,是降低围术期病死率的关键.早期诊断,早期手术,尽量减少急诊手术,可减少术后呼吸机辅助时间,有利患儿术后顺利康复.