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.     

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.     

Transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Rastelli or Lecompte procedure?

During a 10-year period (1980 to 1990), 62 patients underwent complete repair for transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Twenty-two patients (35%) (mean age 8.1 +/- 7.2 years) underwent the Rastelli operation: The ventricular septal defect was enlarged anteriorly in eight patients, and right ventricular-pulmonary artery continuity was established with an extracardiac valved (9/22) or nonvalved (13/22) conduit. Forty patients (65%) (mean age 3.3 +/- 3.2 years) underwent the Lecompte modifications: The conal septum was extensively excised when present (30/40), anterior translocation of the pulmonary bifurcation was performed in 32 patients, and right ventricular-pulmonary artery continuity was established by direct anastomosis without a prosthetic conduit. There were seven early deaths (11%; 70% confidence limits, 7% to 17%): two after the Rastelli procedure (9%; 70% confidence limits, 3% to 20%) and five after the Lecompte operation (12.5%; 70% confidence limits, 7% to 20%). Four patients were lost to follow-up, yielding a 93% complete follow-up (mean follow-up 55 months). There were two late deaths (one in each group). Actuarial probability of survival (+/- standard error) at 5 years was 83% +/- 9% after the Rastelli operation and 84% +/- 6% after the Lecompte procedure. All long-term survivors (except one in the Rastelli group) were in functional class I. Five patients in the Rastelli group underwent late reoperation for obstruction of the extracardiac conduit (28%; 70% confidence limits, 16% to 42%). Three late reoperations (10%; 70% confidence limits, 4% to 19%) were required after the Lecompte operation (one for residual ventricular septal defect and two for residual pulmonary outflow tract obstruction). At most recent examination, residual pulmonary outflow tract obstruction was present in seven patients of the Rastelli group (39%; 70% confidence limits, 26% to 53%) and in six patients of the Lecompte group (19%; 70% confidence limits, 12% to 29%). The combined likelihood of reoperation for pulmonary outflow tract obstruction and residual pulmonary outflow tract obstruction was significantly higher in the Rastelli group (67% versus 26%; p = 0.005). Both procedures provide satisfactory early and late results. The Lecompte operation allows complete repair in infancy, is feasible in patients with anatomic contraindications to the Rastelli operation, and may reduce the need for reoperation and the prevalence of residual pulmonary outflow tract obstruction.     

Transposition of the great arteries and ventricular septal defect: results with the Senning operation and closure of the ventricular septal defect in infants

From May, 1978, to July, 1982, 46 infants ranging in age from 12 days to 12 months and in weight from 2.1 to 8.4 kg underwent repair of dextrotransposition of the great arteries (D-TGA) and ventricular septal defect (VSD) using a Senning repair and closure of the VSD. Ventricular septal defects were classified as membranous (47.8%), malaligned (28.3%), atrioventricular (AV) canal type (13.0%), subarterial (2.2%), muscular (2.2%), and multiple (6.5%). Hospital mortality was 15.2% and late mortality, 5.1%. Postoperative complications included tricuspid regurgitation (mild in 3 and severe, requiring tricuspid valve replacement, in 3), residual VSD (pulmonary/systemic flow ratio of greater than 2:1) in 3 patients (2, AV canal type and 1, multiple VSDs), pulmonary venous obstruction in 3 patients, and permanent complete heart block in 4 patients (2, AV canal type of VSD also requiring tricuspid valve replacement). Lung biopsy studies showed reversible Heath-Edwards and morphometric changes. No patient was seen with Heath-Edwards III or greater changes. In 10 patients, right ventricular end-diastolic pressures and pulmonary artery pressures at rest were within normal limits one year after operation. As the operative mortality of atrial inversion and arterial switch operations for D-TGA with VSD tends to become comparable, more extensive follow-up data, including cardiac catheterization and coronary arteriography in a large number of patients, will be necessary to establish the superiority of one approach over the other.     

Rastelli operation as one stage anatomical correction for infants with complete transposition of the great arteries and ventricular septal defect

We recently adopted the Rastelli operation as a one stage anatomical correction for three infants with transposition of the great arteries and associated with ventricular septal defect (TGA + VSD). In all, ventricular septal defect was enlarged by excision of the conus septum to avoid obstruction of intraventricular tunnel and Hancock valved conduits of 18 mm in diameter were used for reconstruction of pulmonary trunk. The postoperative function of the left ventricule proved to be better than that of the right ventricle, as the systemic ventricle, after the Mustard procedure for TGA + VSD. Our results show that the Rastelli operation is effective as a one stage anatomical correction in infants with TGA + VSD.     

Transposition of the great arteries associated with ventricular septal defect: surgical results and long-term outcome.

OBJECTIVES: To identify potential risk factors influencing early and late outcome following the arterial switch operation (ASO) for transposition of the great arteries associated with ventricular septal defect including double-outlet right or left ventricle. METHODS: All patients who underwent ASO in our department until August 2000 (n=105) were included in this study. There were 77 transpositions of the great arteries with ventricular septal defect, 22 Taussig-Bing hearts and six patients with double-outlet morphology. The median age at operation was 24 days. Aortic arch obstruction was present in 25 patients; in 13 of these patients, a repair with aortic arch reconstruction was done before ASO. The usual coronary artery pattern was present in 59% of the patients. In six patients, we found an intramural course of at least one coronary artery. The ventricular septal defect was closed with a patch through the right atrium (n=35), the aorta (n=25), the pulmonary artery (n=25) or the right ventricle (n=3); in 17 patients a combined approach was necessary. RESULTS: There were five hospital deaths (4.7%, 95% confidence limit 2-11%). The median duration of follow-up was 72 months. Fourteen patients underwent 15 reoperations 33 months after repair (median), eight for right ventricular outflow tract obstruction or neopulmonary stenosis. Four late deaths occurred, two due to complications related to coronary artery anomalies. Statistical analysis revealed no significant risk factor whatsoever correlating with death or need for reoperation. Survival after 12 years was 91.6%, and freedom from reoperation was 82.6%. Latest follow-up data showed that 13% of patients were in NYHA class II and/or required medical treatment; 87% were in NYHA class I. CONCLUSIONS: ASO associated with patch closure of ventricular septal defect can be performed early in life with a low risk of mortality (<5%), low incidence of reintervention (<15%) and promising long-term outcome.     

Apical ventricular septal defects: follow-up concerning anatomic and surgical considerations

BACKGROUND: Apical ventricular septal defects (VSDs) are difficult to visualize and close transatrially. We described their distinctive anatomic features, which have seldom been documented angiocardiographically and pathologically, in order to develop an effective approach for their surgical management. METHODS: Fourteen postmortem cases, two explanted hearts, 9 successfully operated patients, and 1 unoperated living patient were included in this report. Angiocardiographic documentation of the apical VSD was available in 14 of 16 (87.5%) of the postmortem and transplanted cases, and in 6 of 10 (60%) of the living patients. Echocardiograms were available in 23 of all 26 cases (88%). RESULTS: Severe associated malformations were present in 14 of 16 (87%) of the pathologically documented cases. Large VSDs allowed extensive communication between the left ventricular and the right ventricular sinuses in 4 patients. In 12 of the pathologically documented cases and in the 10 living patients, the left ventricular apex communicated with the right ventricular apical infundibular recess. CONCLUSIONS: Extremely large apical VSDs with severe biventricular dysplasia and dysfunction may require cardiac transplantation. Large apical VSDs can be successfully closed through a small apical infundibulotomy. This approach, applicable even in small infants, can avoid pulmonary artery banding or left ventriculotomy.     

Left ventricular wall stress and contractile function in transposition of the great arteries after the Rastelli operation

Left ventricular wall stress and contractile function were determined by echocardiographic methods in 11 patients studied 0.7 to 13.8 years (mean +/- standard error of the mean = 5.6 +/- 1.2 years) after undergoing the Rastelli operation for transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction. Age at operation ranged from 4.6 to 11.3 years (mean +/- standard error of the mean = 7.4 +/- 0.7 years). Data were compared with data of 24 normal subjects of similar age and heart rate. Left ventricular end-diastolic dimension and end-diastolic volume were significantly higher than normal, averaging 134% +/- 8% of normal dimension (p less than 0.004) and 106 +/- 13 ml/m2 versus a normal volume of 60 +/- 3 ml/m2) (p less than 0.007). In addition left ventricular wall mass was 215 +/- 40 gm/m2 versus a normal value of 72 + 6 gm/m2 (p less than 0.004). Both meridional and circumferential end-systolic and peak systolic stress values were not significantly different between normal subjects and Rastelli patients. Estimates of ventricular pump function including shortening fraction, rate-corrected velocity of circumferential fiber shortening, and ejection fraction were all depressed when compared with normal values. Velocity of fiber shortening, evaluated as a function of end-systolic stress, demonstrated abnormal contractile function in eight of 11 (73%) patients. These data indicate that left ventricular function is usually abnormal and residual left ventricular dilation and wall hypertrophy remain despite successful use of the Rastelli operation for repair in patients with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction.     

The Rastelli operation for transposition of the great arteries. Early and late results.

Fifty-nine patients had corrective operation of the Rastelli type for transposition of the great arteries between 1968 and 1975. In 121 patients (35 per cent), the ventricular septal defect was enlarged by excising a portion of the septum. During the first 30 days after the operation, II patients (19 per cent) died. The risk of repair in infancy was greatly increased. There were 5 late deaths, and reoperation was required in 11 patients. Sixty-eight per cent of the survivors are in New York Heart Association Class I and 29 per cent are in Class II. Some late complications related to deteriorations of the earlier aortic homograft conduit may be avoided by use of a Dacron conduit with a porcine valve, as suggested by short-term favorable results in 25 recent cases. The current operative mortality rate of 8 per cent (last 25 operations) and the observation that all but one of the late survivors in this series are either asymptomatic or only mildly symptomatic tend to verify the Rastelli operation as the procedure of choice for repair of transposition of of the great arteries when associated with ventricular septal defect and pulmonary stenosis.     

Surgical anatomy of the infundibular septum in transposition of the great arteries with ventricular septal defect

Anatomic variation of the infundibular septum was studied in transposition of the great arteries with ventricular septal defect in 23 hearts and double-outlet right ventricle with anterior position of the aorta in two hearts. Anterior displacement of the infundibular septum (i.e., "false" Taussig-Bing heart) was associated with coarctation or interruption of the aortic arch in 88% of the cases, whereas posterior displacement resulted in subpulmonary narrowing in 100% of the cases. Anterior displacement makes intraventricular rerouting from the left ventricle to the aorta difficult because of a long oblique route. In addition, the right ventricular cavity becomes smaller after closure of the ventricular septal defect. Therefore, arterial switch accompanied with transatrial or transpulmonary closure of the defect without ventriculotomy is recommended. In hearts with posterior displacement of the infundibular septum, the anterosuperior rim of the defect is difficult to approach through the tricuspid valve, and the route from the left ventricle to the aorta is rather straight. Hence, the Rastelli procedure is preferable. In hearts without displacement of the infundibular septum, either arterial or atrial switch with transatrial closure of the ventricular septal defect is applicable.     

Rastelli operation as one stage anatomical correction for infants with complete transposition of the grat arteries and ventricular septal defect

We recently adopted the Rastelli operation as a one stage anatomical correction for three infants with transposition of the great arteries and associated with ventricular septal defect (TGA+VSD). In all, ventricular septal defect was enlarged by excision of the conus septum to avoid obstruction of intraventricular tunnel and Hancock valved conduits of 18 mm in diameter were used for reconstruction of pulmonary trunk. The postoperative function of the left ventricle proved to be better than that of the right ventricle, as the systemic ventricle, after the Mustard procedure for TGA+VSD. Our results show that the Rastelli operation is effective as a one stage anatomical correction in infants with TGA+VSD.     

两种不同手术方式矫治完全性大动脉错位伴有室缺和肺动脉狭窄的效果分析

目的 回顾性分析两种不同手术方式矫治完全性大动脉错位(TGA)伴有室间隔缺损(VSD)和肺动脉狭窄(PS)的效果.方法 对46例TGA/VSD/PS的患者进行手术矫治,其中采用主动脉根部移位/重建双室流出道术,即Nikaidoh术27例(N组),Rastelli手术19例(R组).N组采用自身心包补片重建右室流出道(RVOT),其中1例用同种异体带瓣管道Homograft;R组使用Homograft重建RVOT.两组均无手术前姑息手术史.结果 N组因术后严重心功能衰竭死亡1例(3.7%),R组无死亡.术后早期并发症的发生率两组相近.手术平均年龄N组(16.3±16)个月,R组(51±20)个月,N组明显小于R组(P=0.028).N组术后无明显残余左、右心室流出道梗阻(LVOTO、RVOTO),而R组有37%患者分别存在LVOTO或RVOTO(P＜0.05),但N组术后89%患者存在轻-中度肺动脉血反流现象,R组仅1例患者存在轻度反流(P＜0.05),两组手术早期心功能状况差异无统计学意义;随访期两组均无死亡,但R组有4例(23.6%)因LVOTO、RVOTO再手术治疗.结论 Nikaidoh术矫治TGA/VSD/PS患者,在解剖上更胜一筹,适宜于小的年龄患者.     

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.