Early prophylactic pulmonary artery banding in isolated congenitally corrected transposition of the great arteries

Objective: Mid-term evaluation of an aggressive surgical management of isolated congenitally corrected transposition of the great arteries (ccTGA) by pulmonary artery (PA) banding in early infancy. Methods: Between 2001 and 2009, 11 asymptomatic patients (seven neonates and four infants) underwent a dilatable, partially adjustable, homemade PA banding for ccTGA with intact ventricular septum. PA band circumference was correlated to body weight (22 mm + 1 mm kg⁻¹) and ideally adjusted to obtain flat septal geometry. Mean age at operation was 1.5 ± 1.4 months. Results: There was no hospital mortality. Mean ventilation time and intensive care unit (ICU) stay were 20 ± 9 h and 2.6 ± 1.5 days, respectively. Five patients required postoperative inotropic support. One late death occurred suddenly at 4 months; normal biventricular function and no tricuspid regurgitation were noted at last follow-up, 1 week before death. Mean follow-up was 21.5 ± 26 months. Mean band velocity increased over time from 2.65 ± 0.7 m s⁻¹ postoperatively to 3.7 ± 0.3 at 6 months and 4.5 ± 0.4 m s⁻¹ at 2 years. Tricuspid regurgitation remained stable in seven patients, decreased in three and worsened in one. Flat septal geometry was obtained in all patients after the third postoperative month. One patient underwent a double-switch procedure at 7 years due to suprasystemic morphologically left ventricular pressure. The postoperative course was uneventful. Conclusions: In neonates with isolated ccTGA, prophylactic PA banding is safe and carries a low morbidity. At mid-term evaluation, tricuspid valve function is stabilised or improved and systemic competence of the left ventricle is maintained, thus allowing double switch if indicated.     

Aortic translocation, Senning procedure and right ventricular outflow tract augmentation for congenitally corrected transposition, ventricular septal defect and pulmonary stenosis

The management of congenitally corrected transposition of the great arteries and associated lesions is frequently challenging. Significant pulmonary stenosis is a contraindication to the conventional double-switch. Instead repair may be accomplished by the Rastelli–Senning procedure, using an extracardiac conduit to achieve continuity between the morphological left ventricle and the pulmonary arteries. This however can be accompanied by conduit and intra-ventricular baffle-related problems that can necessitate surgical re-intervention and lead to late mortality. We describe the use of aortic translocation, Senning procedure and reconstruction of the right ventricular outflow tract using autologous tissue and valved homograft to facilitate anatomical correction in congenitally corrected transposition. The advantages of this technique in this group of patients and the implications for conduction tissue are discussed.     

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。     

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.     

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.     

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。     

Palliative management of a low-birth-weight infant with congenitally corrected transposition of the great arteries,severe restrictive foramen ovale,hypoplasia of the morphologically right ventricle,ventricular septal defect,and steno-insufficiency of the tricuspid valve

A 3-month-old girl weighing 2160 g was diagnosed with congenitally corrected transposition of the great arteries, a ventricular septal defect, severe restrictive foramen ovale, hypoplasia of the morphologically right ventricle, and steno-insufficiency of the tricuspid valve. As her hemodynamic condition (which was comparable to that of the single ventricle with obstructed pulmonary venous pathway) deteriorated progressively, she underwent a palliative operation at 4 months of age. Postoperatively, she was managed with the “low resistance strategy” to achieve a stabilized state of the pulmonary circulation. Currently, 1.5 years after the operation, she is doing remarkably well.     

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.     

双调转术治疗矫正型大动脉转位合并心内畸形

目的总结双调转手术治疗矫正型大动脉转位（cTGA）合并心内畸形的经验。方法2002年4月至2004年6月，行双调转手术治疗cTGA合并心内畸形病儿7例，其中男6例，女1例；年龄4—15岁，平均8．4岁。SLL型6例，IDD型1例。合并室间隔缺损6例，右室双出口1例，继发孔房间隔缺损2例，肺动脉狭窄6例；3例SLL型为右旋心，1例IDD型为左旋心。手术方式包括改良Senning＋Rastelli 4例，Mustard＋Rastelli＋双向Glenn手术、Senning＋Rastelli手术、改良Senning＋Switch手术各1例。结果术后早期死亡1例，为cTGA合并室间隔缺损、肺动脉高压，行改良Senning＋Switch手术者，死因为左心功能衰竭。早期主要并发症有低心排血量综合征、房室分离各1例，胸腔积液和低蛋白血症2例。随访2—24个月，恢复良好，复查多普勒超声心动图、心电图和X线胸片显示，窦性心律5例，结性心律1例，心功能均为Ⅰ级。结论双调转手术可以达到解剖矫治cTGA合并心内畸形，早期手术死亡率低，中、远期心功能效果良好。SLL型病例采用改良Senning心房内调转手术效果优于Senning和Mustard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。     

Hypothermia for cardiogenic encephalopathy in neonates with dextro-transposition of the great arteries

Open in a separate window OBJECTIVESNeonates with dextro-transposition of the great arteries (d-TGA) may experience rapid haemodynamic deterioration and profound hypoxaemia after birth. We report on d-TGA patients with severe acidosis, encephalopathy and their treatment with systemic hypothermia.METHODSThis study is a single-centre retrospective cohort analysis of newborns with d-TGA.RESULTSNinety-five patients (gestational age ≥35 weeks) with d-TGA and intended arterial switch operation were included. Ten infants (10.5%) with umbilical arterial blood pH > 7.10 experienced profound acidosis (pH < 7.00) within the first 2 h of life. Six of these patients displayed signs of encephalopathy and received therapeutic hypothermia. Apgar scores at 5 min independently predicted the development of neonatal encephalopathy during postnatal transition (unit Odds Ratio 0.17, 95% confidence interval 0.06–0.49, P = 0.001). Infants treated with hypothermia had a more severe preoperative course and required more often mechanical ventilation (100% vs 35%, P = 0.003), treatment with inhaled nitric oxide (50% vs 2.4%, P = 0.002) and inotropic support (67% vs 3.5%, P < 0.001), as compared to non-acidotic controls. The median age at cardiac surgery was 12 (range 6–14) days in cooled infants and 8 (4–59) days in controls (P = 0.088). Postoperative morbidity and total duration of hospitalization were not increased in infants receiving preoperative hypothermia. Mortality in newborns with severe preoperative acidosis was zero.CONCLUSIONSNewborn infants with d-TGA have a substantial risk for profound acidosis during the first hours of life. Systemic hypothermia for encephalopathic patients may delay corrective surgery without compromising perioperative outcomes.     

双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

目的 分析和评估纠正型大血管错位伴肺动脉狭窄病儿采用双调转(Double-Switch)手术的治疗效果.方法 2001年8月至2008年12月采用Double-Switch手术行纠正型大血管错位伴肺动脉狭窄21例.其中男11例,女10例;年龄3.5个月至6.3岁,平均(31±18)个月;体重6～28 kg,平均(13.1±6.5)kg.室间隔缺损(VSD)为膜周型15例,远离大动脉1例,肺动脉下5例.轻度二尖瓣反流2例,中度反流2例.采用Senning+Rastelli手术方法 .13例右心室与肺总动脉的连接采用牛颈静脉管道.结果 全组均生存.体外循环转流156～287 min,平均(202.0±35.8)min;主动脉阻断93～161min,平均(138.0±19.8)min.均无完全性房室传导阻滞.随防2～5年,上腔静脉流速增快3例(1.8～2.2)m/s,其中1例术后2.6年再次手术解除上腔静脉梗阻,余2例在随防中;肺静脉回流均无明显梗阻;牛颈静脉管道通畅,解剖右室流出道连接处梗阻2例,压力阶差在30～45 mm Hg(1 mm Hg=0.133kPa),尚在随防中.结论 Double-Switch手术纠治纠正型大血管错位伴肺动脉狭窄的复杂型先心病,取得了较好效果,但仍需进一步随访,以观察这类复杂手术方法 的长期疗效.     

Arterial-level repair of transposition of great arteries without coronary artery transfer

Hypoplastic ascending aorta and interrupted aortic arch (IAA) are rarely associated with dextro-transposition of the great arteries (D-TGA). Severe hypoplastic ascending aorta may preclude coronary artery transfer making arterial switch operation problematic. We report a case of D-TGA with a large subpulmonic ventricular septal defect, IAA, and hypoplastic ascending aorta that underwent successful biventricular surgical repair without coronary artery transfer.     

Modification of the Senning procedure in the double-switch operation: The triangular double-door technique

We developed a modified Senning procedure in the double-switch operation for the patients with congenitally corrected transposition of the great arteries (ccTGA). In our technique, the right atrial (RA) free wall is not used as a baffle for draining systemic venous blood to the left atrium. Instead, a patch material is used for the baffling. A wide communication between the pulmonary venous chamber and RA is created by making the triangular double door with the RA-free wall, and the window is closed with in situ pericardial flap. We have successfully adopted this technique in our recent two consecutive ccTGA patients.     

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.     

合并异位心及左室流出道狭窄的矫正型大动脉转位分流术后再次手术策略研究

目的 探索合并异位心及左室流出道狭窄的矫正型大动脉转位患者姑息性分流术后的二次手术治疗策略.方法 回顾性分析2011年6月至2019年5月连续性收治的姑息性分流术后合并异位心及左室流出道狭窄的矫正型大动脉转位54例患者的临床资料.所有患者均双心室发育均衡,按手术方式分为两组:一个半心室组,行一个半心室矫治术者24例,男...