Single-Stage Arterial Switch With Aortic Arch Enlargement for Transposition Complexes With Aortic Arch Obstruction

Background. Patients with transposition complexes and aortic arch obstruction are a surgical challenge with significant mortality. We have adopted an aggressive approach of concurrent aortic arch repair and arterial switch operation with excellent results.

Methods. Since 1989, 12 of 13 patients with aortic arch obstruction and transposition of the great arteries or double-outlet right ventricle with subpulmonary ventricular septal defect have undergone complete single-stage repair. One patient underwent a two-stage repair because of hemodynamic instability. The median age of repair was 27 days and the median weight was 3.5 kg. Surgical technique involved the arterial switch operation and ventricular septal defect closure when present in 12 patients. One patient with severe subaortic stenosis underwent a modified Damus-Kaye-Stansel operation with concomitant aortic arch enlargement. The aortic arch was enlarged in 12 of 13 patients with a pulmonary homograft patch.

Results. There have been no early deaths and only one late death at 39 months postoperatively from hepatoblastoma. The mean follow-up is 42 months. There have been no reoperations for recurrent aortic arch obstruction. All survivors are currently well from a cardiac point of view.

Conclusions. Concomitant single-stage repair for transposition complexes with aortic arch obstruction achieves excellent survival and should be the surgical procedure of choice.     

Clinical results of arterial switch operation for double-outlet right ventricle with subpulmonary VSD.

OBJECTIVE: An arterial switch operation is considered a good alternative for the repair of double-outlet right ventricle (DORV) with atrioventricular concordance connection and subpulmonary ventricular septal defect (VSD) when intraventricular rerouting is not feasible. The clinical results of an arterial switch operation with ventricular septal defect closure for this anomaly were studied. METHODS: Between 1986 and 1997, 27 patients ranging from 10 days to 5 years of age (mean 0.4 years) underwent an arterial switch operation with ventricular septal defect closure for the correction of double outlet right ventricle with subpulmonary VSD. The 50% rule was used to define double-outlet right ventricle. Arch anomalies were associated in nine cases, and were corrected either previously or simultaneously. A subarterial muscle resection was performed in 14 without any subsequent stenosis of the ventricular outflow tract. The relationship of the great arteries was mostly anteroposterior in 15 and mostly side by side in 12. The left coronary artery (main trunk or circumflex artery) courses behind the pulmonary artery in 15/27 (six/15 in the anteroposterior relation and ten/12 in the side by side relation). The Lecompte maneuver was used to reconstruct the pulmonary artery in all but five cases with a side by side relationship of the great arteries. RESULTS: There was one operative death (3.7%) and three late deaths. The actuarial survival rate was 83 +/- 8% at 9 years. Right ventricular outflow tract obstruction including peripheral pulmonary stenosis developed in seven cases operated on in the early era. The reoperation free rate was 46 +/- 20% at 9 years. CONCLUSION: Although double-outlet right ventricle with subpulmonary VSD has complex features, including an aortic arch obstruction and coronary artery anomalies, an optimal definitive surgical repair using an arterial switch operation can be performed safely with a thorough understanding of this variable anomaly. The prevention of right ventricular outflow tract obstruction at the time of an arterial switch operation may thus help improve the rate of late morbidity.     

Surgical management of the straddling mitral valve in the biventricular heart

BACKGROUND: The straddling mitral valve in the biventricular heart is a rare condition that may complicate biventricular repair. METHODS: Treatment and outcomes in 5 consecutive patients who underwent primary repair between 1992 and 1997 were reviewed. Their ages at repair ranged from 2 months to 8 years. Three patients had a double-outlet right ventricle with a subaortic (n = 2) or subpulmonary (n = 1) ventricular septal defect. Two patients had transposition of the great arteries (S,D,D), a ventricular septal defect, and left ventricular outflow tract obstruction. The attachments of the papillary muscles of the straddling mitral valves were located on the right ventricular aspect of the ventricular septum. Four patients underwent baffle partitioning of the ventricular cavity. The baffle suture line was used to secure the chordae tendineae crossing the ventricular septal defect, or was intentionally omitted at the papillary muscle. The right ventricular outflow tract was reconstructed with patch augmentation, an extracardiac conduit, or an arterial switch operation. One patient with transposition who had a giant papillary muscle to the straddling mitral valve associated with abnormal insertion of the tricuspid valve on the conal septum underwent univentricular repair. RESULTS: There were no early or late postoperative deaths. There was no mitral valve dysfunction, left ventricular outflow tract obstruction, or heart block in the 4 patients who underwent biventricular repair. CONCLUSIONS: Although there are several exceptional situations in which ventricular partitioning may result in early and late complications, a straddling mitral valve does not preclude biventricular repair.     

Arterial switch operation for a complex transposition of the great arteries in dextrocardia.

A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,D,D}, ventricular septal defect, aortic arch hypoplasia, juxtaposition of the morphologically right atrial appendage and superoinferior ventricles is herein presented. He successfully underwent arterial switch operation, ventricular septal defect closure and aortic arch reconstruction with end to end extended direct anastomosis.     

What is necessary for improvement of arterial switch operation?

We report long-term results of the arterial switch operation (ASO) and postoperative complications related to operative procedure. Between 1998 and 2007, ASO were performed in 42 patients [transposition of the great arteries (TGA) with intact ventricular septum:21, TGA with ventricular septal defect (VSD):13, Taussig-Bing anomary (TBA):7, and double outlet right ventricle with noncommitted VSD:1]. Hospital death occurred in 1 patient (2.4% mortality rate) due to low cardiac output syndrome (LOS) caused by prolonged aortic clamp, who had TGA with VSD, coactation of aortic arch and right ventricular outflow obstruction. Four patients required re-operation (freedom from re-operation rate was 84.7%). Two had pulmonary stenosis and 1 needed right ventricular outflow tract (RVOT) reconstruction with transannular patch. His pulmonary valve used for previous VSD closure was diminished. The other required muscle resection of RVOT. Five patients presented grade II or more neo-aortic insufficiency and 2 of them were TBA. Both these TBA, rerouting of left ventricular outflow tract (LVOT) to the aorta was challenging, they showed LVOT obstruction postoperatively. One underwent aortic valve plasty and the other had residual VSD closure. None had coronary event or abnormality in coronary arteriography. Selection of surgical procedure should be based on morphologic features in order to improve surgical outcome.     

The role of aortic translocation in the management of complex transposition of the great arteries

The surgical management of transposition of the great arteries with a ventricular septal defect and left ventricular outflow tract obstruction still remains a significant challenge despite major advances in pediatric cardiovascular surgery. Almost four decades after its inception, the Rastelli procedure continues to be the standard surgical technique used in the management of these patients, despite less than optimal long-term results. Aortic translocation with biventricular outflow tract reconstruction is an alternative surgical technique that creates anatomically sound connections between the ventricles and their respective great arteries, which could result in better long-term outcomes. This technique is especially useful in patients with transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction who have an inlet or restrictive ventricular septal defect, a small right ventricle, a straddling atrioventricular valve, or an aberrant coronary artery crossing the right ventricular outflow tract.     

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。     

Anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect. II. Clinical results in 50 patients with pulmonary outflow tract obstruction

From November 1980 to November 1985, 50 patients underwent anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect and pulmonary outflow tract obstruction. The technique used was one that we have previously described, which we call REV. The principles of this technique are resection of the infundibular septum, construction of a tunnel connecting the left ventricle to the aorta, and direct anastomosis, without a prosthetic conduit, of the pulmonary arterial trunk with the right ventricle. The tunnel is situated beneath the aortic valve and occupies very little space in the right ventricular cavity. Age at operation ranged from 4 months to 13 years (mean 3.5 years). Twenty-six patients had a classic type of transposition of the great arteries; all other patients had various types of anomalies of ventriculoarterial connection in which it was impossible, after the intraventricular connection of the left ventricle to the aorta, to use the natural pulmonary orifice for the pulmonary outflow tract reconstruction. There were nine hospital deaths (18%) and one late death. Twenty-six of 29 patients whose follow-up time exceeded 1 year had an excellent clinical result. No stenosis of the aortic outflow tract was found. Four patients had significant pressure gradients on the pulmonary outflow tract. Our present experience with REV suggests that this technique allows anatomic repair in a wide variety of anomalies of ventriculoarterial connection associated with ventricular septal defect and pulmonary outflow tract obstruction, even in infants, with an acceptable rate of mortality and morbidity.     

Results of arterial switch operation for primary total correction of the Taussig-Bing anomaly

BACKGROUND: This study evaluates the results of the arterial switch operation for early total repair of double-outlet right ventricle with subpulmonary ventricular septal defect (the Taussig-Bing heart). METHODS: From 1986 through April 2003, 27 patients with Taussig-Bing anomaly underwent arterial switch operation. Twenty patients were neonates (n = 11) or infants younger than 3 months (n = 9). Obstruction of aortic arch (n = 19) or subaortic right ventricular outflow tract obstruction (n = 20) and unusual coronary artery patterns (n = 19) were common. Total correction as a single procedure was performed in 21 patients. Events are depicted by Kaplan-Meier curves. RESULTS: There was 1 patient hospital death at 2 months after repair. One patient died late that was not cardiac related. Survival was 92% +/- 6% at 8 months and remained constant thereafter. Four patients underwent reoperation (1 for residual aortic arch obstruction and 3 for subvalvular and valvular pulmonary stenosis). Freedom from reoperation decreased to stabilize at 83% +/- 8% after 2 years. The risk to have right ventricular outflow tract obstruction develop was 33% +/- 10% at 1 year, increasing slowly and leveling out at 57% +/- 12% at year 5 and thereafter. Statistical analysis revealed no significant risk factor for death or need for reoperation. CONCLUSIONS: The Taussig-Bing anomaly should be corrected in the neonatal period or in early infancy by arterial switch operation, closure of the ventricular septal defect, and simultaneous correction of associated cardiovascular anomalies as a one-stage procedure. Right ventricular outflow tract obstruction often complicates the postoperative course and is the main cause for reintervention.     

Reverse subclavian flap repair of hypoplastic transverse aorta in infancy

BACKGROUND: Management of hypoplastic aortic arch associated with coarctation in infancy can be challenging. Reverse subclavian flap aortoplasty plus coarctation resection offers simplicity without needing foreign material or cardiopulmonary bypass. METHODS: Since 1988, 46 of 162 infants less than 3 months undergoing coarctation repair had hypoplastic arch enlargement with reverse subclavian flap aortoplasty. Median age was 11 days; mean weight was 3.2 kg. Thirty-seven patients (80%) had associated cardiac defects including single or multiple ventricular septal defects (14 infants), transposition of the great arteries (7), aortic or mitral stenosis (5), and complete atrioventricular septal defect (5 infants). Twenty-eight patients had pulmonary artery banding; 2 had an arterial switch operation through a separate median sternotomy. RESULTS: There were two hospital deaths: one 4 months postoperatively in a patient requiring a Norwood procedure the next day for underestimated left ventricular hypoplasia; the other of sepsis more than 1 month postoperatively. On follow-up from 1 to 129 months (mean, 38 months), there were five recurrent obstructions: three at the coarctation site treated with balloon dilatation and two at the arch site. Twenty-six children had their heart defects corrected with 29 subsequent operations including an arterial switch operation for transposition of the great arteries/ ventricular septal defect (3 infants), relief of aortic or mitral stenosis +/- ventricular septal defect closure (5), multiple ventricular septal defect closure (3), a bidirectional Glenn (2), complete atrioventricular septal defect (2), and anomalous left coronary with ventricular septal defect repair (1 infant). Four children await debanding and ventricular septal defect closure or Glenn anastomosis. There have been two late deaths (overall survival, 91%). CONCLUSIONS: Reverse subclavian flap aortoplasty is excellent for relief of arch hypoplasia and coarctation in infants with low recurrence rates and acceptable operative and intermediate survival.     

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.     

Ventricular septal defect with tricuspid pouch with and without transposition. Anatomic and surgical considerations.

In a 10-year review, patients operated on for ventricular septal defect and tricuspid valve pouch were divided into two groups, because the effect of the tricuspid valve pouch is influenced by which ventricle has the higher pressure. Group I comprised patients with ventricular septal defect without transposition of the great arteries and group II, ventricular septal defect with transposition. In 72 of 392 group I patients, the septal tricuspid valve leaflet was incised to expose the edges of the hidden ventricular septal defect to accomplish proper anatomic repair. Forty-eight patients had a tricuspid valve pouch, the diagnosis being established by angiography, echocardiography, or at operation. Ages at operation ranged from 5 months to 22 years and the pulmonary-systemic flow ratio ranged from 1 to 3.4, with 16 being less than 1.5. In one patient the pouch produced a 40 mm Hg pressure gradient in the right ventricular outflow tract. At operation, through a transatrial approach, the tricuspid valve pouch was opened radially, the actual ventricular septal defect patched, and the tricuspid valve leaflet repaired. There were no deaths, no significant intraoperative or postoperative morbidity, and no tricuspid valve dysfunction. The average postoperative hospital stay was 4.8 days. In group II, six of 83 patients operated on for transposition with ventricular septal defect had significant left ventricular outflow tract obstruction from the tricuspid valve pouch. Five of six had a Mustard procedure, two requiring a left ventricular-pulmonary artery conduit, and in two of the six the ventricular septal defect was closed through the pulmonary artery. One patient had heart transplantation after a Mustard repair and tricuspid valve replacement. The sixth patient in group II had a successful arterial switch at 9 years of age, after the presence of left ventricular outflow tract obstruction was proved to be due to the pouch. The presence of a tricuspid valve pouch in group I may lead the surgeon to close false small openings produced by the pouch rather than the actual ventricular septal defect. Incising the pouch is safe and essential for proper exposure and secure closure of the true defect. In group II, the systemic right ventricular pressure can push the pouch into the left ventricular outflow tract, causing significant obstruction, and may contribute to tricuspid valve insufficiency after atrial baffle repair. Arterial switch is preferred because it returns the obstructive tricuspid valve pouch and abnormal tricuspid leaflet to the lower pressure pulmonic right ventricle.     

The potential for subpulmonary obstruction in complete transposition after the arterial switch procedure. An anatomic study

Increasing experience with the arterial switch procedure has revealed that, in some instances, obstruction of the newly created sub-pulmonary outflow tract is a major problem. In this study, we examined the right ventricular outflow tract in 51 specimens of complete transposition, 18 of which were associated with a ventricular septal defect. We focussed our attention on the musculature which made up the outflow region and considered variations from that found in the normal right ventricle. Our gross observations showed differences in morphology which produced a sharper angulation between inflow and outflow when compared to the normal. Discrete outflow obstruction was found in 11 hearts (8 with ventricular septal defect and 3 with an intact ventricular septum). The obstruction was due to deviation of the outlet septum in 5, and anomalous muscle bundle in 1, hypertrophy of septomarginal and septoparietal trabeculations in 3 and circumferential infundibular hypertrophy in 2 hearts. Coexisting obstruction of the left ventricular outflow was present in 3 hearts. The anatomical substrates of discrete obstruction should be identified by preoperative cross-sectional echocardiography and/or angiography. Modifications in surgical techniques or radical resection of the obstructing musculature could then be performed to avoid the problem of subpulmonary obstruction after the arterial switch procedure.     

Anatomic correction for complete transposition and double-outlet right ventricle

Between February, 1981, and December, 1984, 30 patients underwent anatomic correction of transposition of the great arteries with intact ventricular septum (n = 8), transposition with ventricular septal defect (n = 15), and double-outlet right ventricle with subpulmonary ventricular septal defect, the Taussig-Bing anomaly (n = 7). At operation, ages ranged from 18 hours to 6 years (mean 11.3 months) and weights ranged from 2.6 to 16.4 kg (mean 6.1 kg). The group with transposition and intact ventricular septum on average was younger (mean 1.2 months) and smaller (mean 3.5 kg) than the other two groups. Associated congenital heart defects were seen in 12 patients, including five with coarctation, three with multiple ventricular septal defects, two with right ventricular hypoplasia, two with juxtaposed atrial appendages, and one each with interrupted aortic arch, Wolff-Parkinson-White syndrome, and left ventricular outflow tract obstruction. All 10 patients who had undergone prior palliative operations had pulmonary artery banding. In addition, four of these patients had coarctation repairs, four had atrial septectomy, and one had systemic/pulmonary shunting. All recognized patterns of coronary anatomy were encountered. The aorta and pulmonary artery were side by side in 14 patients and anteroposterior in 16 patients. The Lecompte maneuver to establish right ventricular-pulmonary arterial continuity was successfully used in 12 of 13 patients with anteroposterior great vessels but in none of those with side-by-side arteries. Seven patients had subvalvular right ventricular outflow tract obstruction, recognized either at operation (five) or postoperatively (two). This was responsible for death in three patients. The 30 day hospital mortalities were as follows: one death (12.5%) in the group with transposition and intact ventricular septum, six deaths (40%) in the group with transposition plus ventricular septal defect, and one death (14.3%) among patients with double-outlet right ventricle and subpulmonary ventricular septal defect; the overall mortality was eight deaths (26.7%). There have been no late deaths (mean follow-up 17.2 months). Ninety-five percent of the survivors are in New York Heart Association Functional Class I. Postoperative catheterization in 13 patients has shown normal left ventricular function, no coronary stenosis, and no aortic incompetence. Sixty-nine percent of these patients had clinically unsuspected gradients across the right ventricular outflow tract, which may be prevented by avoiding the Lecompte maneuver or the use of conduits.     

The Damus-Stansel-Kaye procedure: anatomical determinants and modifications

Seven of 119 patients undergoing anatomical correction for transposition of the great arteries and Taussig-Bing anomalies without pulmonary stenosis had the Damus-Stansel-Kaye procedure and the rest, the arterial switch. The age of the patients having the Damus-Stansel-Kaye procedure ranged from 0.5 year to 5 years (mean age, 2.2 +/- 1 years). Four patients had transposition, 2 had Taussig-Bing anomaly, and 1 had corrected transposition. Indications for the Damus-Stansel-Kaye procedure were side-by-side great arteries associated with difficult coronary anatomy (5 patients), single coronary system (1 patient), and subaortic stenosis (1). A graft between the ascending and descending aorta for interrupted aortic arch made mobilization and posterior displacement of the ascending aorta for the arterial switch difficult. Subaortic stenosis (1 patient), subpulmonary ventricular septal defect (2 patients), and restrictive ventricular septal defect (4) precluded the Rastelli procedure. In 6 patients, the main pulmonary artery was transected at the band, a proximal main pulmonary artery to aorta anastomosis was complemented with a synthetic patch, and a right ventricle to distal main pulmonary artery valved conduit was inserted. Four patients had closure of the aortic outflow. Two patients had postoperative bleeding and 2, heart block. The only patient who did not have transection of the main pulmonary artery, an omission that led to an obstructed conduit at the distal anastomosis, died late. Two patients subsequently needed aortic outflow closure for critical aortic insufficiency. The Damus-Stansel-Kaye procedure has a definite role and can be safely performed in patients with transposition of the great arteries and Taussig-Bing anomalies.(ABSTRACT TRUNCATED AT 250 WORDS)     

Internal right ventricular band for multiple ventricular septal defects in a neonate undergoing arterial switch and aortic arch repair

A neonate presented with d-transposition of the great arteries, aortic arch hypoplasia, aortic coarctation, and multiple ventricular septal defects. During the arterial switch procedure and the aortic arch repair, a fenestrated Gore-Tex disk (W.L. Gore & Assoc, Flagstaff, AZ) was sewn into the right ventricular outflow tract to restrict pulmonary blood flow. The internal right ventricular band successfully controlled the pulmonary blood flow, maintaining a systemic oxygen saturation of 88% to 92%, and allowing growth from 3.5 to 10.5 kg. At 8 months of age, the internal band in the patient was removed, and the ventricular septal defects were successfully closed.     

动脉转位术的临床应用

目的 总结动脉转位术(arterial switch operation，ASO)治疗完全型大动脉转位(transposition of the great arteries，TGA)和右心室双出口伴肺动脉瓣下室间隔缺损(VSD)的临床经验。方法 采用ASO治疗小儿先天性心脏病32例，其中TGA22例，伴室间隔完整型(intact ventricular septum，IVS)9例，伴VSDl3例；右心室双出口伴肺动脉瓣下VSD(Taussig—Bing)10例。结果TGA／IVS9例中死亡1例，TGA／VSD13例中死亡4例，Taussig-Bing10例死亡3例，总手术死亡率25％(8／32)。术后随访3个月～2年，所有患者紫绀消失，活动能力明显增强。1例Taussig—Bing术前二尖瓣轻-中度反流，术后仍为中度反流；2例TGA主动脉和肺动脉瓣上狭窄，压差40mmHg(1kPa=7．5mmHg)，1例肺动脉瓣下狭窄和残余VSD，3个月后再次手术治愈。结论 ASO已广泛应用于TGA的纠治，手术效果满意；应用于右心室双出口肺动脉瓣下VSD的早期纠治，不但可防止发生肺血管阻塞性病变，而且避免了心内修补左心室流出道梗阻的远期并发症。     

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.     

Arterial switch operation with ventricular septal defect repair and aortic arch reconstruction

The arterial switch operation for transposition of the great arteries or double outlet right ventricle with ventricular septal defect (VSD) and aortic arch obstruction is a challenging procedure. One-stage neonatal repair is preferred; however, palliation may be indicated in the newborn who presents with Swiss cheese septum or the patient with extracardiac complications such as necrotizing enterocolitis or subarachnoid hemorrhage. The aortic arch repair is performed with continuous cerebral perfusion and includes patch enlargement of the transverse aortic arch and ascending aorta to control for the important diameter mismatch between the aorta and the pulmonary root. The VSD is no longer closed through the pulmonary valve. Instead, the VSD is approached through the aortic valve after harvesting of the coronary buttons and/or through the tricuspid valve and, if necessary, through an infundibulotomy. The coronary artery transfer is often complex because of the presence of unusual coronary artery patterns and requires precise technique. Subaortic obstruction is often present and requires correction. The right ventricle can be mildly hypoplastic but is not a contraindication to repair unless there is important tricuspid stenosis.     

Early palliation of univentricular hearts with subaortic stenosis and ventriculoarterial discordance. The arterial switch option.

The optimal Fontan-type operation greatly depends on appropriate initial palliation. Several surgical techniques have been used in infancy to palliate patients with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis. The two most common are pulmonary artery banding and Damus-Norwood procedures. Palliative arterial switch operation is another surgical option that was used in this early series of seven infants. The principle of this operation is to "switch" the subaortic obstruction into a subpulmonary obstruction; the coronary artery relocation on the large pulmonary trunk creates a harmonious aortic root and the connection of the rudimentary ventricular chamber to the pulmonary artery trunk creates a natural protection of the pulmonary vascular bed through the restrictive bulboventricular foramen. Seven infants with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis underwent a palliative arterial switch operation. All infants had an associated aortic arch obstruction of various degrees, including one with interrupted aortic arch, five with coarctation with severe arch hypoplasia, and one with isolated arch hypoplasia. There were three with double-inlet left ventricle, three with tricuspid atresia, and one with transposition of the great arteries with ventricular septal defect and severe right ventricular hypoplasia. The subaortic obstruction was patent at birth in five patients who underwent a palliative switch operation in the first 2 months of life, and rapidly occurred following a previous neonatal pulmonary artery banding associated with arch repair in two patients who underwent a switch operation at 5 and 8 months of age, respectively. The operation includes aortic arch repair without prosthetic material, an atrial septectomy, and the arterial switch. An associated pulmonary shunt was required in five patients and a pulmonary artery banding in one. There was one early death in a patient with [S,L,L] anatomy and congenital atrioventricular block, leading to an early mortality of 14% (95% confidence limits: 1% to 28%). There was one late death. Four survivors are waiting for a Fontan-type procedure, and one survivor had satisfactory right ventricular growth. Early palliative arterial switch operation offers several advantages: reconstruction of a harmonious aortic root, natural protection of the pulmonary bed through the restrictive bulboventricular foramen, prevention of deleterious myocardial hypertrophy, and arch reconstruction without the introduction of a foreign material. This aggressive technique may provide a satisfactory palliation in infants with univentricular hearts and ventriculoarterial discordance, when the bulboventricular foramen/aortic anulus ratio is less than 0.8 or when the subaortic stenosis is severe enough to be associated with an arch obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)