双调转术治疗先天性矫正型大动脉转位疗效分析

2002年4月至2003年4月，我们应用双调转手术治疗4例先天性矫正型大动脉转位(cCTGA)病儿，收到满意的治疗效果，现报道如下。     

分期双调转解剖矫治术治疗左心室退化的矫正型大动脉转位的临床疗效分析

目的评价分期双调转解剖矫治术治疗先天性左心室退化矫正型大动脉转位（CCTGA）的临床疗效。方法回顾性分析2005年5月至2011年5月北京阜外心血管病医院14例左心室退化CCTGA患儿经手术治疗的临床资料,男8例,女6例;年龄2．5～72．0（34．4-24．0）个月;体重5．1～23．0（12．7±4．9）kg。术前经超声心动图、心血管造影或心导管检查确诊。主要合并畸形：三尖瓣关闭不全13例,限制型室间隔缺损10例,房间隔缺损或卵圆孔未闭7例,动脉导管未闭4例,肺动脉瓣轻度狭窄4例,Ⅲ度房室传导阻滞1例。在全身麻醉下完成一期左心室训练术,在体外循环下完成二期心房大动脉双调转术,平均间隔时间0．67～34．0（10．23±9．47）个月。结果一期左心室训练术后无住院死亡,随访结束时形态左心室舒张期末内径（mLVEDd）及形态左心室（mLV）后壁厚度均较术前显著增加（P〈0．05）,室间隔位置较术前略向形态学有心室侧（mRV）移位,三尖瓣关闭不会（TR）较术前减轻,跨肺动脉环缩带压差显著增加（P〈0．05）,左心室射血分数（LVEF）无显著变化;形态左心室压力与形态右心室压力比值（PmLV／PmRV）显著增大（P〈0．05）。二期心房大动脉双调转术住院死亡2例,死亡率14．3％（2／14）;死亡原因：严重心律失常、循环衰竭、猝死。术后早期并发症：肺部感染6例,房性心律失常2例,胸腔积液2例,气胸、膈肌麻痹行膈肌折叠术、气道出血、主动脉瓣轻度关闭不全、腹膜透析、体外膜肺氧合辅助、二次气管内插管各l例。生存患者均痊愈出院。术后随访12例,随访时间2～8年。随访期间死亡1例,死亡率8．33％（1／12）,死亡原因：严重心律失常、循环衰竭。心功能I级8例,Ⅱ级3例。主要晚期并发症：左心功能不全3例,主动脉瓣中度关闭不全3例,二尖瓣中度关闭不全1例。结论分期双凋转解剖矫治术治疗左心室退化CCTGA早期临床疗效满意,中远期疗效尚需进一步随访观察,但术后左心功能不良和新出现的主动脉瓣关闭不全应值得关注。     

动脉调转术治疗心室大动脉连接异常的先天性心脏病

目的总结动脉调转术（ASO）治疗心室大动脉连接异常的先天性心脏病（先心病）手术疗效。方法2000年1月至2004年8月，60例病儿实施ASO，早年（2000．1—2003．5）42例，近期（2003．6—2004．8）18例；其中完全性大动脉转位（TGA）49例、Taussig-Bing畸形7例、矫正性大动脉转位（ccTGA）4例；年龄1—6个月15例、7～12个月14例、1-3岁6例、〉3岁6例，其中〉6月龄的TGA/VSD或TGA/PDA18例。行大动脉调转术，同期矫治合并畸形；ccTGA病儿先行心房转流术，后行ASO。结果全组手术死亡10例（16．7％），其中早年9例（21．4％）、近期1例（5．6％），死亡率明显下降（P〈0．05）。生存病儿随访0．5—56．0个月，心功能恢复良好，无死亡及并发症。结论ASO应用于TGA、Taussig-Bing畸形以及ccTGA能取得良好的手术结果。针对国内TGA/VSD或TGA/PDA病儿就诊较晚、年龄较大，肺动脉压力较高等特点，如心导管检查显示肺小动脉阻力不高，ASO仍可获得满意的疗效。     

Double Switch术治疗先天性矫正性大动脉转位二例

目的 介绍解剖矫治先天性矫正性大动脉转位的手术特点。方法 2例矫正性大动脉转位患者,1例9个月,合并室间隔缺损、肺动脉高压,行Senning加动脉调转术;1例23岁,合并室间隔缺损、肺动脉狭窄、三尖瓣关闭不全,行Rastelli加Senning术。结果 2例患者全部成活,其中1例术前出现Ⅲ度房室传导阻滞,安装永久起搏器,术后超声检查心室功能正常,心内隧道和心外管道通畅。无三尖瓣反流。结论 Double Switch手术是一种理想的治疗先天性矫正性大动脉转位的方法。     

心房及大动脉双调转术治疗先天性矫正型大动脉转位

目的 总结先天性矫正型大动脉转位心房及大动脉双调转术治疗的初步经验.方法 先天性矫正型大动脉转位行心房及大动脉双调转手术17例.8例行Senning+大动脉调转术,年龄11个月～11岁,中位年龄3.7岁,其中7例合并肺动脉高压,1例合并肺动脉瓣下狭窄,3例曾行肺动脉环缩术-左心室锻炼.9例行Senning+ Rastelli手术,年龄5～24 岁,中位年龄9.0岁,其中5例行室缺扩大术.结果 Senning+大动脉调转手术组主动脉阻断183～282 min,术后呼吸机辅助14～984 h,引流208～1496 ml.术后完全性房室传导阻滞1例,安装永久性心脏起搏器后病愈出院;右侧膈肌麻痹1例,行膈肌折叠术后病愈出院.Senning+ Rastelli手术组主动脉阻断132～380 min,术后呼吸机辅助18～276 h,引流108～1780 ml,术后ECMO辅助循环1例,因低心排综合征死亡1例.结论 对于年龄较大的先天性矫正型大动脉转位病儿,心房及大动脉双调转术近期治疗效果满意.     

Double—Switch术治疗矫正型大动脉转位

目的：使矫正型大动脉转位（CTGA）不仅从生理上,而且从解剖上得到了纠正。方法：资料为美国波士顿儿童医院心脏外科收治的9例CTGA患者,年龄2－16个月,体重3.1-11kg。术前均行彩色多普勒超声心动图和心导管造影检查,心排血量和心功能测定,9例患者均施行Double-Switch术,合并的其它畸形都予以纠治。结果：9例患者均存活,术后彩色多普勒超声心动图示左、右心功能明显改善。结论：小婴儿CTGA应尽可能早期选做Double-Switch术,使心功能尽可能适合机体的循环机制。     

Rastelli手术治疗大动脉转位伴室间隔缺损

目的介绍Rastelli手术治疗大动脉错位伴室间隔缺损的经验.方法全组49例中男29例,女20例.平均年龄5.6岁;平均体重17.2kg.完全性大动脉转位31例,纠正性大动脉转位18例;伴肺动脉狭窄45例,伴肺动脉高压4例.均在低温体外循环下行Rastelli手术.二期根治5例.体外循环灌注(178.5±52.5)min;主动脉阻断(109.2±38.3) min.结果手术早期死亡6例,死亡率12.2%.死因为肺动脉高压危象、肾衰、心律紊乱和严重低心输出量综合征.术后并发症有心律紊乱、肺动脉高压危象、蛛网膜下腔出血、脑功能紊乱、肾衰及多脏器功能衰竭、心包或胸腔积液、感染等.CICU平均监护7.3 d.随访中因同种带瓣大动脉(VHC)感染死亡1例.结论完全性大动脉转位手术中,右室流出道直切口有利于心内隧道修补室间隔缺损;纠正性大动脉转位手术中,解剖右室径路显露缺损较好且易避开传导系统,但不利于术后心功能恢复.而解剖左室径路修补室间隔缺损的房室传导阻滞发生率高;大于4岁者手术宜选择大号VHC可减少再次手术几率;对无长段左室流出道狭窄的完全性大动脉转位病婴,可在动脉换位术基础上行肺动脉瓣叶交界切开或Konno术以解除左室流出道梗阻.为防止术后功能性二尖瓣反流,对伴肺动脉狭窄的纠正性大动脉转位病儿,提倡心房-大动脉双换位手术.     

左室训练术治疗先天性矫正型大动脉转位的临床疗效分析

目的评价左室训练术治疗先天性矫正型大动脉转位(cCTGA)的临床疗效。方法 2005年5月至2011年5月,阜外心血管病医院对24例左心室退化的cCTGA患者行左室训练术,其中男13例,女11例;年龄0.17～22.00(3.73±4.35)岁;体重5.10～61.00(15.71±10.95)kg。主要合并畸形:三尖瓣关闭不全(TR)23例(轻度11例、中度7例、重度5例),限制型室间隔缺损18例,房间隔缺损5例,卵圆孔未闭5例,动脉导管未闭4例,肺动脉瓣轻度狭窄5例,主动脉弓缩窄1例。术前经超声心动图、心血管造影或心导管检查确诊,形态学左心室舒张期末内径(mLVEDD)8～32(21.56±6.60)mm,形态学左心室(mLV)后壁厚度2～7(4.29±1.52)mm,形态学左心室与形态学右心室(mRV)压力比(PmLV/mRV)0.12～0.65(0.41±0.12)。手术均采用胸骨上端小切口或胸骨正中切口,在全身麻醉下完成肺动脉环缩术。结果术毕测压PmLV/mRV 0.57～0.93(0.76±0.10);全组患者无住院死亡,出院时超声心动图检查提示:双心室结构和功能良好,室间隔位置较术前略向mRV侧移位,mLVEDD较术前略增大,TR较术前减轻。术后随访1～35个月,无远期死亡。所有患者一般情况好,生命体征平稳,心功能分级(NYHA)Ⅰ～Ⅱ级,mLVEDD 14～40(26.17±7.11)mm,mLV后壁厚度4～9(4.95±1.44)mm,PmLV/mRV 0.52～0.98(0.72±0.16),TR较术前明显减轻,其中14例患者完成二期心房大动脉双调转术。结论左室训练术应用于左心室退化的cCTGA患者安全有效,mLV压力负荷及后壁厚度增加,mLV腔扩大,且TR显著减轻。     

小儿ccTGA解剖纠治术中应用双向Glenn/半Mustard心房调转双调转手术的初步研究CSCD

目的 评估先天性矫正型大动脉转位中应用半Mustard心房调转手术及双向Glenn手术完成双调转手术的风险及收益.方法 为了避免完全Senning手术的并发症并且辅助右心血流动力学,采用一种改良的心房调转手术.2014年1月到2016年3月,7例患儿完成此种改良双调转手术的解剖矫治.5例合并肺动脉闭锁患儿施行了Rastelli-心房调转手术,2例施行了动脉-心房双调转手术.心房调转使用半Mustard手术占同期解剖矫治术中的26.9％（7/26）.结果 全组无近期死亡.随访7～21个月,无死亡,无心房板障相关的再次手术或窦房结功能不全.术后三尖瓣反流轻至中度6例,中度1例.右心室-肺动脉管道无明显梗阻.结论 改良心房调转手术完成双调转手术初期结果良好.半Mustard和双向Glenn手术的减少板障及窦房结相关的并发症,手术方法相对简单,能改善右心功能和预后.     

Double switch operation for congenitally corrected transposition of the great arteries after two-staged pulmonary artery banding

We describe a case of congenitally corrected transposition of the great arteries (cc-TGA) successfully performed by the double switch operation after two-staged pulmonary artery banding (PAB). An eleven-year old boy diagnosed with cc-TGA underwent the first PAB at that age, followed by the second PAB one year later. Because of severe ventricular dysfunction and arrhythmia of the anatomic left ventricle, the intension of one-stage PAB was abandoned. Cardiac catheterization data from after the adequate second PAB provided the surgical indication for the anatomical correction and double switch operation (Senning+Jatene procedure) and this was successfully performed at age 14. Although cardioversion was required to treat supraventricular tachycardia in the early period after surgery, the patient was discharged from hospital and remains in good clinical condition at the last follow-up at 5 years with normal sinus rhythm and good biventricular function.     

Double switch with reversed patrick-mcgoon for corrected transposition of the great arteries with double outlet right ventricle

A 4-year-old boy diagnosed with a double outlet right ventricle with transposition of the great arteries in a discordant atrioventricular connection. The anatomical characteristics of the ventriculoarterial relationships resulting from leftward deviation were quite similar to a mirror image of those in the malformation reported by Patrick and McGoon. Hence, it was most appropriate to perform the double switch operation associated with Patrick-McGoon’s intraventricular rerouting and Senning’s atrial switch. This intraventricular rerouting could be performed without damaging the conduction system because of existence of the anterior node. The cardiac catheterization 1 month after the operation showed no pressure gradient of the new reconstructed left ventricular outflow tract, good left ventricular function and no significant pressure gradient of the right ventricular outflow tract. The patient has been staying in class 1 of New York Heart Association for more than 3 years after the operation.     

Functional outcome of anatomic correction of corrected transposition of the great arteries

Objective: Anatomic correction of corrected transposition of the great arteries, utilizing the morphologic left ventricle as a systemic pumping chamber, is considered the preferred method. The purpose of the study was to analyze the intermediate functional outcome following anatomical correction. Methods: Between 1997 and 6/2010, 23 patients with corrected transposition of the great arteries and associated lesions underwent anatomical correction. Seventeen (74%) and six patients (26%) had situs solitus {S,L,L} and situs invs {I,D,D}, respectively. Fifteen patients (65%) had undergone 18 palliations before the corrective operation. The median age at palliation was 0.23 years, with a range of 0.016–8.4 years. A corrective, modified Senning–arterial switch procedure was performed in nine patients, 13 patients underwent a modified Senning–Rastelli procedure, and in one patient a combination of modified Senning and aortic translocation (Bex/Nikaidoh) was used. The median age at the corrective operation was 2 years (from 0.3 to 15.7 years). Results: There was no mortality or heart transplant within the mean follow-up of 3.4 years. Freedom from reintervention was 77% at 5 years. There were no signs of obstruction of the systemic and pulmonary venous tunnels. The function of both ventricles was normal in all patients, even in the four patients who required retraining of the left ventricle. Mild aortic regurgitation was noticed in three patients. Preoperatively detected significant tricuspid regurgitation either disappeared or became trivial after the operation in all the six patients. All patients except two are in sinus rhythm; one patient is pacemaker-dependent preoperatively and one is pacemaker-dependent postoperatively. There were no clinically apparent neurological problems. All patients, but one, are in the New York Heart Association (NYHA) class I. Conclusions: Anatomic correction of corrected transposition of the great arteries can be performed in selected patients without mortality and with acceptable morbidity. The mid-term functional outcome is excellent, resulting in normal ventricular function, even in retrained left ventricles, and minimal incidence of complete heart block. The long-term function of the aortic valve, intraventricular tunnels, conduits, and ventricles requires close surveillance.     

Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries

Objective: To analyse the long-term patency of coronary arteries after neonatal arterial switch operation (ASO). Methods: A retrospective study of the operative reports, follow-up and postoperative catheterisation data of 119 patients, who underwent the great arteries (TGA) repair since 1991, has been carried out. Patient population: Among the 133 survivors of the 137 ASOs performed between 1991 and 2007, 119 patients have been studied by routine control cardiac catheterisation and form the study population. Median time between repair and the coronary angiography was 2.9 ± 1.9 years. A comparison between the eight patients (6.7% out of the entire study population), known to have postoperative coronary obstructions (group I) and the rest of the cohort with angiographic normal coronary vessels (group II) was performed by univariate analysis of variance and logistic regression models. One patient had surgical plasty of the left coronary main stem with subsequent percutaneous angioplasty, three patients had primary coronary stent implantation and four patients had no further intervention at all. In group I, all but one patient denied symptoms of chest pain and echocardiography failed to show any difference between the two groups in terms of left ventricular systolic function (ejection fraction group I 61 ± 2% vs 62 ± 6% of group II, p = 1.0). Results: The association of coronary obstruction with complex native coronary anatomy (Yacoub type B to E) was evident at both univariate (62% of group I vs 22% of group II, p = 0.04) and logistic regression (p = 0.007, odds ratio (OR) 8.1) models. The type of coronary reimplantation (i.e., coronary buttons on punch vs trap-door techniques) was similar between the two groups (punch reimplantation in 25% of patients of group I vs 31% of group II, p = 0.1) as was the relative position of the great vessels (aorta anterior in 100% of patients of group I vs 96% of group II; univariate, p = 0.1). Conclusions: The late outcome in terms of survival and functional status after ASO is excellent. Nevertheless, the risk of a clinically silent late coronary artery obstruction of the reimplanted coronary arteries warrants a prolonged follow-up protocol involving invasive angiographic assessment.     

Anatomical correction of complex forms of transposition of the great arteries in neonates

Objective—The arterial switch operation has become the procedure of choice for the simple transposition of the great arteries (TGA) while in the complex forms of the defect the staged approach is frequently implemented. The aim of this study is to compare groups of patients with simple transposition and children with the complex form of the defect and identify factors affecting early and late outcome.

Design—From 1997 to 2003, 135 consecutive neonates with TGA underwent arterial switch operation and simultaneous reparation of all associated defects. Univariate and multivariate analysis of perioperative variables and follow‐up data was performed. Patients were divided into two groups. Group I (n=84, 62.2%) included neonates with simple transposition (TGA/IVS), Group II (n=51, 37.8%) included children with complex transposition (TGA/VSD).

Results—Overall early mortality was 8.1% and there was one late death (0.7%). One‐month, 1‐year and 5‐year actuarial survival rates were 91.8, 91.1 and 91.1%, respectively. There were no differences in the early and late survival rate between groups. Reintervention rate for right ventricular outflow tract obstruction (RVOTO) was 13.3% (balloon plasty or reoperation). The freedom from reintervention at 1, 3 and 5 years was 98.4, 87.9 and 85.4%, respectively. There were no differences in the need for reintervention between groups. The significant differences between groups concerned: age at operation (p?<?0.001), associated anomalies (p=0.002) including aortic arch anomalies (p=0.002) and coronary artery anomalies (p=0.02), application of delayed chest closure (p=0.015), and occurrence of sepsis (p=0.032). Risk factors for early death were: left ventricule dysfunction related to age at operation (p=0.016) and resternotomy in intensive care unit (p?<?0.001). There were no differences between groups concerning these risk factors as far as circulatory arrest time, aorta clamping time, and early and late morbidity.

Conclusions—The arterial switch operation can be the treatment of choice for various forms of TGA with low early and late mortality and morbidity rates. The main cause of early death is still left ventricular dysfunction. Such well‐known predictors of poor outcome as presence of ventricular septal defect, coronary artery anomalies and aortic arch anomalies did not affect early and late findings. The presented approach of early simultaneous anatomical correction of TGA and all associated anomalies ensures good condition of children with low necessity for reintervention.     

Modification of the arterial switch operation for transposition of the great arteries with complex coronary artery patterns

The arterial switch operation has evolved into the treatment of choice for most forms of transposition of the great arteries (TGA). Recently reported operative mortality of the procedure has fallen to the range of 1.1%–6.0%, even for complex forms of TGA. Despite advancement in the technical aspects of the procedure, certain anatomical variations of the coronary arteries, such as a single coronary orifice and/or intramural coronary artery, are still considered surgical risks in many centers. Optimizing the surgical technique for relocating these challenging variations of the coronary anatomy is key to improving the surgical outcomes for the procedure. In this review, the surgical modifications of the arterial switch operation for TGA associated with complex patterns of the coronary arteries are examined. This review was submitted at the invitation of the editorial committee.     

Results of a new technique of arterial switch operation for transposition of great arteries with single coronary artery

Background Transposition of great arteries (TGA) with Single Coronary artery is one of the high risk groups for Arterial Switch Operation (ASO). To eliminate this risk, we have innovated a new technique of ASO with insitu coronary re-allocation. We present our experience with this new technique. Methods From September 1998 to October 2005, ten consecutive cases of TGA with single coronary artery were operated employing this new technique. Their age ranged from 8 days to 9 months; their weight ranged from 2.6 to 5.8 Kgs. ASO was done by transecting the great arteries just above the commissures. For coronary re-allocation, hockey stick shaped incisions were made in the facing sinuses of the proximal aorta and the pulmonary artery. These flaps were sutured in such a way that the coronary ostium was committed to the neo-aorta. Results There was no in-hospital mortality. The follow-up ranged from 4 months to 7 years. All patients had follow-up echocardiogram at regular intervals, which showed no significant right or left ventricular outflow obstruction, no regional wall motion abnormalities and no, neo-aortic or neo-pulmonary regurgitation. Conclusion This new coronary re-allocation technique avoids problems related to coronary translocation such as traction and kinking. It spares the need for dissection of proximal coronary artery and its branches, and thereby eliminates the risk of development of fibrosis and stenosis. The same technique can be used regardless of the sinus of origin of the coronary artery. It is a reliable and reproducible technique. The early results were excellent.     

大动脉转换术在复杂先天性心脏病治疗中的应用

目的 回顾总结我院近3年采用大动脉转换术治疗小儿复杂先天性心脏病(先心病)的临床经验。方法2000年1月至2003年5月,采用大动脉转换术(Switch手术)治疗小儿复杂先心病61例。其中完全性大血管错位(TGA)45例,包括室间隔完整型(TFGA-IVS)26例,伴室间隔缺损(TGA,VSD)19例;右心室双出口伴肺动脉瓣下室间隔缺损(Taussig-Bing)16例。结果TGA,IVS死亡2例;TGA-VSD死亡4例;Taussig-Bing死亡4例;总手术病死率16％。术后随访3个月-3年,所有病例青紫症状消失,活动能力明显增强。1例Taussig-Bing术前二尖瓣轻～中度返流,术后仍为中度返流。2例TGA术后出现主动脉和肺动脉瓣上狭窄,压差40mmHg,1例肺动脉瓣下狭窄和残余室间隔缺损,3个月后再次手术治愈。结论大动脉转换术应用于完全性大血管错位的纠治,手术效果满意;应用于右室双出口肺动脉瓣下室间隔缺损的纠治,不但可早期纠治,防止其肺血管阻塞性病变发生,而且避免了心内修补左室流出道梗阻的远期并发症。