左室训练术在婴幼儿大动脉转位分期手术中的应用

目的总结左室训练术在婴幼儿大动脉转位分期手术中的经验。方法2001年1月至2011年12月北京阜外心血管病医院对38例室间隔完整的大动脉转位及合并限制性小室间隔缺损的大动脉转位患儿施行了左室训练手术,其中男26例、女12例,年龄（19．1±7．7）个月,体重（7．6±4．7）埏。术前动脉血氧饱和度72．6％±9．1％。左室训练术包括体动脉肺动脉分流术和肺动脉环缩术。3例需要同期行房间隔开窗术。术后对患儿进行随访观察。结果本组患儿术后动脉血氧饱和度从术前72．6％4±9．1％上升至83．9％±8．1％,左右心室压力比从术前的0．364±O．04上升至0．75±0．09。全组死亡3例,死亡率7．89％。随访35例,随访时间2～11年,随访期间行二期动脉调转手术23例。结论对于超过新生儿期已经发生左心室退化的大动脉转位患儿,左室训练术可以安全有效的施行,为二期做动脉调转手术提供必要的条件。     

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.     

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.     

快速二期动脉转位术纠治新生儿完全型大动脉转位

目的总结快速二期动脉转位术的临床应用效果.方法 2002年9月至2003年5月,5例完全型大动脉转位患者行快速二期动脉转位术.手术平均年龄83.0±72.2 天,平均体重4.7±0.9 kg.由于求诊时已经超过最佳手术年龄,左心室退化,不能承受体循环压力,则先行肺动脉环缩和体肺动脉分流术,术后6～9天行第二期动脉转位术.结果一期手术中,1例术后32小时发生室上性心动过速,少尿,经腹膜透析和药物处理无效死亡;其余4例分别在术后6～9天行二期动脉转位术,无死亡.术后随访2～10个月,生长发育良好,超声心动图检查显示心内无残余分流,主动脉和肺动脉干吻合口通畅,左心室射血分数(EF)0.68～0.77,短轴缩短率(FS)0.24～0.37,1例主动脉瓣轻度反流.结论对失去最佳手术时间的新生儿完全型大动脉转位,行快速二期动脉转位术是最佳的选择.     

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。     

二期手术治疗完全性大动脉错位

目的　总结完全性大动脉错位 (D -TGA)二期手术治疗效果和经验。方法　回顾分析1990年 8月至 2 0 0 0年 12月间 8例进行二期手术D TGA病儿。二期手术年龄为 14d～ 96个月 ,平均(5 0 5± 32 4 )个月 ;体重为 3 7～ 2 3 5kg ,平均 (15 9± 6 1)kg。两次手术间隔时间为 3d～ 75个月。初期手术中球囊房间隔缺损扩张术 2例 ,伴肺动脉发育不良行Blalock Taussig分流术 2例 ;因肺动脉高压行肺动脉环缩术 (PAB) 4例。二期手术中Senning手术 1例 ,Rastelli手术 4例 ,大动脉转换术 (ASO) 2例 ,改良Fontan手术 1例。结果　手术早期死亡 2例 ,1例为Rastelli手术 ,另 1例为改良Fontan手术 ,死亡原因分别为器质性肺动脉高压和左室流出道梗阻。早期生存 6例 ,随访发现 ,Senning手术病儿三尖瓣轻度反流 ,心胸比率 0 6 ;3例Rastelli手术者无左、右室流出道梗阻 ,心功能良好 ,III度房室传导阻滞 1例 ,完全性右束支传导阻滞 2例 ;ASO 2例均无解剖问题 ,心功能正常 ,远期效果满意。结论　初期姑息手术可以对完全大动脉错位病儿的生理进行初步“调整”或“准备” ;二期手术尽量选择大动脉转换术 ,由于解剖或生理因素可选择Rastelli手术或Senning手术 ,避免改良Fontan手术。     

完全性大动脉转位合并重度肺动脉高压增加调转术后病死率

目的 探讨完全性大动脉转位(TGA)术前肺动脉高压与术后近、中期结果的关系.方法 将101例行动脉调转术的病儿根据术前平均肺动脉压力(mPAP)分为3组,肺动脉压力正常(对照)组,轻度肺动脉高压组和重度肺动脉高压组,比较分析不同组间的术后近中期结果.结果 手术后肺动脉高压组病儿肺动脉压力均有明显下降.术后各组间并发症和手术死亡比例差异无统计学意义.但是重度肺高压组中期病死率较高.结论 mPAP＜50 mm Hg(1 mm Hg=0.133 kPa)的TGA合并肺动脉高压病儿可以进行大动脉调转术,并可取得较好的术后结果,但mPAP≥50 mm Hg者虽然行大动脉调转术后肺动脉压力明显下降,且术后早期结果良好,但随访中期病死率较高.     

半旋转动脉干调转术治疗完全性大动脉转位合并室间隔缺损和肺动脉狭窄

目的 报道半旋转动脉干调转术治疗完全大动脉转位(TGA)合并室间隔缺损(VSD)和肺动脉狭窄(PS)的近期疗效.方法 2例病儿男、女各1例,分别为16岁和19个月.均为TGA/VSD/PS.采用半旋转动脉干调转术治疗.结果 2例病儿均生存.女病儿术后发生低心排出量综合征和毛细血管渗漏综合征,治疗后痊愈.男病儿术后恢复良好.术后分别随访10、9个月,心功能Ⅰ级.结论 半旋转动脉干调转术可有效治疗TGA合并VSD和PS.长期效果仍需观察.     

动脉调转术不同年龄手术效果的对比——附142例错过最佳手术年龄者治疗经验

目的 探讨大动脉转位(TGA)病儿接受动脉调转手术(ASO)时年龄对手术效果的影响.方法 2000年5月至2008年9月,264例rIGA病儿行ASO.男194例,女70例;年龄·l d一19岁.其中室隔完整型TCA('rcAJrvs)84例;年龄≤2周28例,>2周56例.伴室隔缺损TCA(TCAJVSO)130例,Tams-ing-B~畸形(TBA)50例,其中≤6月94例,>6月86例.符合最佳手术年龄(≤2周TCA/IVS,≤6个月TGA/IVSD、TBA)者122例,错过最佳手术年龄者(>2周TCA/IVS,>6个月TGA/VSD、TBA)142例.手术方法:18例>2周TGA/IVS先期行左室训练,4例>6个月TGA/VSD先期行肺动脉环缩,余者均一期行ASO,同期矫治合并畸形.结果 全组手术死亡26例,病死率9.85%.平均随访(29.5±7.6)个月,2例分别于术后2个月和1.5年猝死,l例术后1.5年因肺动脉吻合口狭窄再次手术,余者心功能明显改善.无死亡和远期并发症发生.符合最佳手术年龄者病死率13.1l%;错过最佳手术年龄者病死率7.04%.2006年后(共154例),TGA病儿病死率由19.09%降至3.25%(P<0.05),符合最佳手术年龄者由22.64%降至5.80%(P<0.05),错过最佳手术年龄者由15.79%降至1.18%(P<0.05).结论 ASO应用于错过最佳手术年龄TCG者亦可取得满意效果.     

肺动脉环缩术在矫正型大动脉转位形态学左心室功能锻炼中的应用

目的探讨肺动脉环缩术在矫正型大动脉转位形态学左心室功能锻炼的临床应用效果。方法回顾性分析2007年1月至2011年12月上海交通大学医学院附属上海儿童医学中心手术治疗矫正型大动脉转位患者89例中行肺动脉环缩术11例的临床资料,其中男9例,女2例;年龄除1例12岁外,其余为3～42(16.40±11.67)个月;体重6～32(11.70±7.20)kg。所有患者均经超声心动图和心血管造影检查确诊。结果 11例行肺动脉环缩术患者无死亡,术前肺循环与体循环压力比(Pp/Ps)值0.3～0.6(0.44±0.09),术后为0.6～0.8(0.70±0.04),差异有统计学意义(P<0.01)。术前三尖瓣反流轻度2例(18.2%),中度5例(45.4%),重度4例(36.4%);术后无反流2例(18.2%),轻度反流7例(63.6%),轻-中度反流2例(18.2%)。11例中5例术后(15.20±8.31)个月行二期双心室解剖纠治术,其中1例死亡;余6例行单纯肺动脉环缩术后随访(18.83±3.43)个月,超声心动图检查提示:三尖瓣反流轻微2例(33.3%),轻度3例(50.0%),中度1例(16.7%)。结论矫正型大动脉转位通过肺动脉环缩术可减轻三尖瓣反流,锻炼形态学左心室功能,为二期解剖纠治做好准备,手术效果较好。但术后必须定期随访,观察术后形态学左心室功能和三尖瓣反流情况。     

Arterial switch with internal pulmonary artery banding. A new palliation for TGA and VSD in complex cases.

In most cases, one stage repair by arterial switch operation (ASO) is the optimal treatment for neonates with transposition of the great arteries (TGA). Nevertheless, a ventricular septal defect (VSD) associated with TGA remains a major risk factor for early death and reoperation after complete repair in neonates with complex anatomy. A new palliative approach for such specific cases is proposed. An internal pulmonary artery banding (IPAB), as that already used to palliate other cardiac malformations, is performed in association with ASO instead of VSD closure. At the end of ASO, a circular polytetrafluorethylene (PTFE) patch with a 4-mm central hole is oversewn into the neo-pulmonary trunk. We adopted this method in a 17-day-old boy with TGA, VSD, hypoplastic tricuspid valve and diminutive right ventricle. After the operation the child thrived and was doing well without medication. Satisfactory growth of the right ventricle and tricuspid valve was observed by echocardiography during the following months. The patient successfully underwent VSD closure and IPAB removal 2 years after the first procedure. ASO with IPAB could be appropriate in all forms of TGA and VSD in which VSD closure appears too challenging in the neonatal period and in patients with uncertain suitability for biventricular repair. We preferred to use IPAB instead of classic PAB in order to reduce the risk of pulmonary valve damage, pulmonary artery distortion, and above all pulmonary artery dilatation and related coronary compression. In the presented case the strategy as well as IPAB worked according to our expectations.     

Arterial switch operation for transposition of the great arteries without use of prosthetic material]

For transposition of the great arteries (TGA), arterial switch operation (ASO) is theoretically preferable to atrial switch operation, since the left ventricle is established as the systemic ventricle and sinus node function is maintained. However, ASO is a delicate operation requiring the transfer of the coronary arteries. Use of prosthetic material for reconstruction of the neo-pulmonary artery causes postoperative supra-valvular pulmonary arterial stenosis. Five neonates and young infants with TGA underwent ASO without the use of prosthetic material by the technique reported by Pacifico et al. Three patients with simple TGA ranged in age from 10 to 27 days, and in weight from 2.9 to 3.9 kg. Two patients with TGA and ventricular septal defect ranged in age from 41 to 63 days, and in weight from 2.8 to 4.2 kg. There were no deaths, either early or late, and all patients are well 7 to 21 months after surgery. Catheterization and Doppler echocardiographic studies performed in all patients showed that pressure gradient between the right ventricle and pulmonary artery decreased significantly during mean follow-up period of 14 months. These results suggest excellent growth of the pulmonary artery after surgery.     

Surgical management of transposition of the great arteries

From January 1983 through December 1991 470 patients underwent an arterial switch operation (ASO). 281 (59.7%) had transposition of the great arteries (TGA) with intact ventricular septum (IVS) and 189 (40.3%) had a ventricular septal defect (VSD). The overall hospital mortality for ASO was 6.3%, but 0.6% (1/155) in the last 155 consecutive patients with TGA/IVS. Of 9 late deaths (1.9%) 5 were due to coronary artery obstruction. 2 were found related to pulmonary vascular obstructive disease and 2 were unrelated to ASO. Cardiac catheterization in 244 late survivors revealed postoperative, supravalvular pulmonary stenosis in 2% of patients. Residual shunts on ventricular levels greater than QP/QS=1.5/1.0 were measured in 4 patients. No regional wall motion abnormalities were detected and left ventricular function appeared normal in all patients 2 years after surgery. One year after surgery 98% of patients presented in sinus rhythm. The favourable early and midterm results of the ASO as a primary operation continue to make it the preferred approach for the neonate with TGA/IVS and TGA/VSD whenever possible. The rapid two-stage approach (preliminary pulmonary artery banding and shunt followed by ASO after 7 days) is applicable for older patients with TGA/IVS.     

Long-term result of arterial switch operation for corrected transposition of the great arteries or double inlet left ventricle

The purpose of this study is to analyze the operative maneuver and long term outcome of the arterial switch operation (ASO) for congenitally corrected transposition of the great arteries (c-TGA) or double inlet left ventricle (DILV). Since October 1977, 221 patients had undergone ASO in National Cardiovascular Center, Japan. Of these, 8 patients underwent ASO as a part of double switch operation (DSO) for c-TGA, and 1 patient underwent ASO and ventricular septation for the DILV with a rudimentary right ventricle simultaneously. We retrospectively reviewed these 9 patients. Six patients had a past history of the pulmonary artery banding. Age at the time of ASO ranged from 6 months to 5 years (median 3 year). As a reconstruction of the pulmonary artery at the time of ASO, Lecompte maneuver was performed in 7 patients, and original Jatene procedure was performed in 2. Coronary transfer was done as usual in all patients. There was no early death, and 1 patient died 1 year after the operation due to chronic heart failure. Late complication related to the ASO was pulmonary artery stenosis (1 patient after DSO) and aortic regurgitation (1 patient after ventricular septation).     

改良Norwood手术临床应用

目的总结改良Norwood手术的临床应用经验。方法2004年7月和2005年3月分别为1例7月龄单心室伴左心室流出道梗阻女婴,采用主肺动脉和主动脉弓下缘直接吻合、主动脉肺动脉人工血管分流完成改良Norwood手术;1例出生13 d左心发育不良综合征新生儿,采用主肺动脉和主动脉弓下缘直接吻合、右心室肺动脉人工血管分流完成改良Norwood手术。结果例1术后平稳,动脉压为85～100/25～40mmHg（1 mm Hg=0．133kPa）,动脉血氧饱和度为0．75～0．85,术后第3d撤离辅助呼吸,第11 d出院。例2术后平稳,动脉压为65～80／40～60mmHg,动脉血氧饱和度为0．67～0．89,术后第12 d撤离辅助呼吸,第28 d出院。结论改良Norwood手术的先进方法和技术,值得在我国推广应用。     

Preparation of the left ventricle for anatomical correction in patients with simple transposition of the great arteries. Surgical guidelines

Pulmonary artery banding in combination with an aortopulmonary shunt was performed on 16 patients with simple transposition of the great arteries to prepare the left ventricle for anatomical correction. Three groups were identified after operation: Group I (four patients) had increased pulmonary blood flow and tight pulmonary artery banding; Group II (four patients) had increased pulmonary blood flow and moderate pulmonary artery banding; Group III (eight patients) had normal pulmonary blood flow and moderate pulmonary artery banding. Postoperative low cardiac output was present in all patients in Group I, whereas mild heart failure was present in two patients in Group II and in two in Group III. There was one hospital death (6%). The follow-up period was 125 patient-months. Left ventricular systolic pressure rose from 63 +/- 11 torr before the operation to 101 +/- 35 torr after the procedure in Group I (p less than 0.05), from 59 +/- 10 to 93 +/- 33 torr in Group II (p less than 0.05), and from 55 +/- 10 to 84 +/- 16 torr in Group III (p less than 0.005). The increase in left ventricular muscle mass was from 44 +/- 2 gm/m2 preoperatively to 108 +/- 12 gm/m2 after operation in Group I (p less than 0.01), from 43 +/- 3 to 93 +/- 8 gm/m2 in Group II (p less than 0.02), and from 46 +/- 3 to 55 +/- 14 gm/m2 in Group III (p = no statistically significant difference). The postoperative change in left ventricular end-diastolic volume was from 100% +/- 17% to 133% +/- 23% of normal in Groups I and II (p less than 0.05) and from 123% +/- 29% to 107% +/- 36% of normal in Group III (p = no statistically significant difference). In preparing the left ventricle for anatomical correction, avoidance of severe pulmonary artery banding decreases the incidence of postoperative myocardial dysfunction, a moderate degree of volume overload and pulmonary artery banding provides the most effective stimulus for ventricular growth, and a small to moderate atrial septal defect is advantageous because it ensures the volume preload necessary for the development of the left ventricle.     

保留肺动脉瓣的改良REV手术治疗复杂型大动脉转位——附3例报道及文献综述

目的 介绍一种改良REV手术治疗合并室间隔缺损（VSD）、肺动脉瓣狭窄（PS）的复杂型大动脉转位或右心室双出口（DORV）。方法 2005年9月～2006年2月我院收治3例复杂型先天性心脏病患者，在经典REV手术的基础上，我们改良了REV手术，保留了肺动脉瓣和瓣环进行手术治疗。结果 2例痊愈，1例同时用体外膜式氧合器（ECMO）进行抢救后死亡。痊愈患者分别随访4个月和1个月，超声心动图检查提示肺动脉瓣跨瓣压差分别为15mmHg和5mmHg，结果满意。结论 保留肺动脉瓣的改良REV手术对治疗合并VSD、PS的复杂大动脉转位是一种较为理想的手术方法。     

自体主动脉瓣移植纠治完全性大血管错位伴肺动脉瓣狭窄

目的探讨解剖纠治完全性大血管错位（TGA）伴肺动脉瓣狭窄的手术方法。方法1例8月龄、体重6．5kg的TGA伴肺动脉瓣狭窄男病婴，术中将原主动脉瓣移植至肺动脉瓣处形成新的主动脉；再行Switch术，同种带瓣管道连接右心室与肺动脉。另1例10月龄、体重9．8kg的该病男病婴，术中将整个主动脉瓣取下，保留左、右冠状动脉，向后移植，后半部分直接与原肺动脉瓣环连续缝合，前半部分与室间隔缺损之间采用dacron补片连续缝合关闭；肺动脉与右心室切口直接连接。结果2例手术均取得成功。出院时超声检查各吻合口通畅，无残余分流．无主动脉瓣反流。术后随访12个月和6个月，生长发育良好，心胸比率0．75。超声示左心功能良好，前例示主动脉瓣轻度反流．后例左、右心室流出道无残余梗阻．结论采用自体主动脉瓣移植纠治完全性大血管错位伴肺动脉瓣和瓣下狭窄，从解剖上得到彻底纠治．效果良好。由于病例少，随访时间短．还需进一步观察。     

肺动脉环缩术的临床应用

目的总结肺动脉环缩术(pulmonary artery banding,PAB)作为一种姑息治疗手段在治疗先天性心脏病中的临床应用经验。方法 1997年1月至2010年11月北京阜外心血管病医院对138例先天性心脏病患者施行PAB,其中男87例,女51例;年龄(22.2±26.5)个月;体重(7.5±4.6)kg。按施行PAB的目的不同,将138例患者分为3组,心室训练组(组1)55例、单心室矫治过渡组(组2)32例和双心室延迟矫治组(组3)51例。术中、术后分别观察3组患者相应的临床指标,并进行随访。结果 PAB术后院内死亡7例,手术死亡率5.1%。另有3例患者需要再次手术调整环缩带的松紧程度。组1手术死亡1例。55例患者中有36例完全型大动脉转位(D-TGA)患者于年龄(19.6±29.5)个月时接受PAB(29例同期行Blalock-Taussig分流术),平均训练时间42 d,83.3%(30/36)的患者成功施行动脉转位术治疗;另19例矫正型大动脉转位(cTGA)患者,在年龄(45.3±27.2)个月时行PAB治疗,中位训练时间9个月,42.1%(8/19)的患者成功施行双调转术治疗。组2患者手术死亡2例。32例于年龄(14.1±14.9)个月时行PAB治疗,平均肺动脉压较术前明显降低[(23.00±7.40)mm Hg vs.(34.00±10.00)mm Hg],脉搏血氧饱和度(SpO2)亦较术前降低(83.30%±6.30%vs.92.60%±5.90%)。中位间隔时间2年,18.8%(6/32)的患者接受了单心室类矫治术。组3患者手术死亡4例,于年龄(20.6±25.6)个月时行PAB治疗,肺动脉收缩压与体循环收缩压的比值较术前明显降低(0.46±0.15 vs.0.81±0.14),SpO2亦较术前降低(85.00%±10.00%vs.93.10%±7.60%)。中位间隔时间6个月,23.5%(12/51)的患者施行了双心室矫治术。结论 PAB虽然是一种风险较高的姑息治疗手段,但在保护肺血管床、实施心室功能训练、完成复杂先天性心脏病的分期矫治方面仍具有不可缺少的重要作用。     

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.