室间隔完整型大动脉错位的手术治疗

目的　探讨室间隔完整型大动脉错位 (TGA/IVS)的手术治疗方法。　方法　回顾性总结 1999年 8月～ 2 0 0 1年 12月 ,手术纠治TGA/IVS 12例的临床资料。 3例年龄 >2个月的患儿行Senning手术 ,9例年龄 <1个月的采用大动脉转换术 (switch)。　结果　本组采用switch手术治疗者 1例死亡 ,死亡原因为单根冠状动脉。 11例存活患者 ,随访 2个月～ 2年 ,平均 (12± 7)个月 ,效果良好。结论　室间隔完整型大动脉错位必须早期做出诊断 ,以便及时得到治疗。switch手术的最佳手术年龄应在婴儿 2周内 ,最好不超过 1个月     

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.     

Neonatal anatomic repair of transposition of the great arteries and ventricular septal defect

From January 1985 to March 1992, 64 consecutive neonates with transposition of the great arteries (TGA) and ventricular septal defect (VSD) underwent an arterial switch operation and VSD closure. The mean age at operation was 18.5 ± 12 days and the mean weight was 3.3 kg. Seventeen patients had an associated aortic coarctation, of whom 15 underwent single-stage repair through median sternotomy. Coronary artery distribution was: type A: 45 patients; type B: 2; type D: 11 and type E: 6 patients. The location of the VSD was perimenbranous in 42 patients, trabecular in 13, infundibular in 5, and 4 presented with the Taussig Bing heart anomaly. The hospital mortality was 9.3% (n = 6). There were four late deaths (one TGA-VSD and three TGA-VSD and coarctation). Nine patients required reoperation. The mean follow-up of all survivors was 36 ± 19 months. They were in NYHA class I without medication. Six patients developed mild-to-moderate aortic insufficiency. The actuarial survival and freedom from reoperation at 5 years were 81.06% and 84.6%, respectively. We conclude that neonatal anatomic repair of TGA and VSD offers good medium-term results and avoids iterative operations.     

主动脉移位术纠治婴幼儿完全性大动脉错位伴室间隔缺损和肺动脉狭窄

目的探讨主动脉移位术纠治完全性大动脉错位伴室间隔缺损和肺动脉狭窄(TGA/VSD/PS)的临床应用。方法2004年8月到2005年7月,采用主动脉移位术连续纠治6例TGA/VSD/PS,其中男5例,女1例;年龄4~24个月,平均(11.33±6.86)个月;体重5.6~11.0kg,平均(8.43±2.19)kg。结果无手术死亡。体外循环转流112~204min,平均(153.83±33.70)min,主动脉阻断73~139min,平均(89.16±18.61)min。术后随访2~12个月。6例病儿活动良好,X线胸片示心影较术前略大,肺血增多;心电图示窦性心律,心脏超声检查示主动脉瓣反流轻微2例,轻度1例。射血分数0.74~0.86,短轴缩短率0.34~0.52。结论当前TGA/VSD/PS普遍采用Rastelli手术,但长期随访发现术后并发症较多,结果尚不理想。主动脉移位术重建左心室流出道和右心室流出道,避免了Rastelli手术后左心室流出道梗阻和心外管道梗阻的并发症。     

Surgical management of transposition of great arteries associated with multiple ventricular septal defects.

OBJECTIVE: The presence of associated multiple ventricular septal defects (VSDs) increases the risk of the anatomic repair for transposition of the great arteries (TGA). The aim of this study was to define the optimal management of this complex anomaly. METHODS: Between January 1988 and December 1998, 45 patients underwent anatomic repair of TGA associated with multiple VSDs. The median age was 50 days and the median weight 4 kg. Eighteen (40%) had undergone previous palliation including 17 pulmonary artery banding procedure (PAB), seven associated with coarctation repair and one isolated coarctation repair. The perimembraneous septum was involved in 24 patients, the trabecular in 43, the inlet in seven and the infundibular in two. Closure of the VSDs included Dacron or pericardial patchs and matress sutures. The initial approach was through right atriotomy which was sufficient in 15 patients. VSDs were closed through right ventriculotomy in 13 patients, through pulmonary artery in six, through the aorta in one and in the remaining (n = 10) combined approaches were used. Only one patient required left apical ventriculotomy. RESULTS: There were five hospital deaths (11%; 70% CL: 6-18%) including the one early reoperation for residual VSD closure. Five patients had successful early reoperation for secondary PAB for residual VSD. Three late deaths occurred (7%; 70% CL: 3-13%). At the last visit, 95% of survivors were asymptomatic and without any cardiac medication. CONCLUSION: Mid-term survival with good quality of life can be achieved following either one or two-stage repair of this complex anomaly. In the presence of VSD closure failure a secondary PAB may be the procedure of choice.     

Anatomic correction of transposition of the great arteries with ventricular septal defect. Experience with 118 cases

One hundred eighteen patients, 100 with transposition of the great arteries plus ventricular septal defect and 18 with double-outlet right ventricle and subpulmonary ventricular septal defect have undergone arterial switch and patch closure of the ventricular septal defect since February 1983. In transposition of the great arteries the ventricular septal defect was perimembranous in 70 cases, trabecular in 28, and infundibular in 10. Eleven patients had multiple ventricular septal defects. In addition to 18 patients with double-outlet right ventricle, malalignment of the conal septum was present in 19 cases. Coronary type A distribution was recognized in 79 cases, type C in one, type D in 24, and type E in 14. Great arteries were side by side in 19% of cases. Aortic coarctation was present in 31 cases, and subaortic obstruction in 9. Age at operation ranged from 4 days to 4 years (mean, 3.5 +/- 8.3 months), and mean weight was 4.0 +/- 1.6 kg. Thirty-seven infants were younger than age 1 month. Thirty-six patients underwent previous operations: pulmonary artery banding alone (n = 13), pulmonary artery banding and coarctation repair (n = 13), and coarctation repair alone (n = 10). Mean time between the first procedure and the switch was 2.2 months. Six patients with aortic coarctation underwent one-stage repair, through median sternotomy, aortic reconstruction, closure of the ventricular septal defect, and arterial switch. Perioperative mortality was 13.5% (70% confidence limit 10% to 17.6%, n = 16). It was directly related to coronary artery kinking in 50% of deaths and to anatomy and size discrepancy of the great arteries in the remaining deaths. Univariate analysis could not find any significant risk factor of in-hospital mortality. Mean follow-up of 30.3 +/- 23.5 months was achieved in all but 2 survivors. There was one late death. Ten patients underwent 11 reoperations for recoarctation (n = 1), pulmonary stenosis (n = 7), residual ventricular septal defect (n = 2), and stenosis of superior vena cava (n = 1). Two patients needed a permanent pacemaker. Actuarial survival and freedom from reoperation at 5 years were 84.5% +/- 3.6% and 85.7% +/- 4.6%, respectively. We conclude that anatomic correction of complex transposition is a safe method that offers good early and midterm results.     

A new method of closing the ventricular septal defect in corrected transposition of the great arteries

A new method of closing a perimembranous malalignment ventricular septal defect (VSD) in corrected transposition of the great arteries (TGA) of the [S,L,L] type is presented. The method consists of combined approaches to the VSD through both a right atriotomy and an aortotomy without a ventriculotomy. The VSD is patched obliquely from the morphological right ventricular side of the septum, cranially through the aortic valve to the left ventricular side of the septum, caudally through the mitral valve. Although this method has been successfully applied in only one adult patient, some advantages may be expected: (1) prevention of trauma to the His bundle, which runs along the anterosuperior rim of the VSD on the left ventricular side; and (2) prevention of trauma to the tricuspid, mitral, and aortic valves without having to open the ventricles. We believe that this new method warrants a further trial as possibly better for closure of the VSD in corrected TGA of the [S,L,L] type.     

Half-turned truncal switch operation for transposition of great arteries with ventricular septal defect and pulmonary regurgitation

A three-month-old girl weighing 4.2 kg, diagnosed with transposition of the great arteries (TGA) and ventricular septal defect (VSD) was referred to us. She had normal-sized pulmonary annulus and moderate pulmonary regurgitation. Because her pulmonary valve was not suitable for systemic circulation due to valvular incompetence, the half-turned truncal switch operation was selected. The postoperative course was uneventful without left or right ventricular outflow obstructions over a year of follow-up. Our report demonstrated that the TGA and VSD with normal pulmonary annulus is not contraindicated for half-turned truncal switch operation.     

Trans--pulmonary artery repair of ventricular septal defect associated with congenitally corrected transposition of the great arteries.

This report documents successful trans-pulmonary artery repair of ventricular septal defect (VSD) in 2 patients with congenitally corrected transposition of the great arteries, one patient having associated subpulmonic stenosis. In selected cases, the method of repair that is described offers significant advantages over techniques employing transventricular or transatrial access to the VSD.