Total correction of transposition of great arteries with atrioventricular septal defect.

A successful anatomical correction of transposition of the great arteries with an atrioventricular septal defect is reported. This combination of anomalies is rare, and the anatomical correction is unusual in that the operation involves all four heart valves.     

Early results for anatomic correction of transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect

Fifty patients, 21 with transposition of the great arteries plus ventricular septal defect, four with double-outlet right ventricle plus ventricular septal defect, and 25 with transposition of the great arteries plus intact ventricular septum, have undergone anatomic correction. There were four hospital deaths, all in the patients with ventricular septal defects (overall mortality rate 8%). The Lecompte maneuver was used in all patients, and in the last 25 patients the coronary arteries were transposed to medially hinged trapdoor flaps created in the neoaorta. In 10 patients (20%) intraoperative adjustments were necessary to the great vessels and coronary arteries to achieve unobstructed coronary artery flow. There have been no late deaths. Postoperative cardiac catheterization in 19 patients has revealed stenosis on the new main pulmonary artery suture line to be the main early problem. Anatomic correction of transposition (with or without ventricular septal defect) and double-outlet right ventricle septal defect would seem to be a good operative alternative to the Mustard or Senning operation, with the advantage of incorporating the left ventricle into the systemic circulation.     

Primary repair of complete transposition of the great arteries with complete atrioventricular septal defect.

We successfully corrected complete transposition of the great arteries associated with complete atrioventricular septal defect in a 50-day-old infant in concomitant arterial switch operation and two-patch repair. The combination of these 2 complex anomalies is very rare, and primary anatomical repair is feasible in such patients.     

Repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: This study was undertaken to compare the outcomes of the Lecompte procedure and Rastelli repair in the transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) and to determine the risk factors associated with unfavorable events. METHODS: Over a 12-year period (April 1990-October 2002), 35 patients underwent complete repair for TGA, VSD, and LVOTO. Twenty-five patients (71%) underwent the Lecompte modification, and mean age and weight were 23.4+/-18.2 months and 10.2+/-3.0 kg. Ten patients (29%) underwent the Rastelli operation, and mean age and weight were 39.1+/-36.1 months and 13.8+/-6.8 kg. RESULTS: One early death (3%) occurred after the Lecompte procedure and no late death. The mean follow-up was 5.9+/-3.8 years. Eight patients in the Rastelli group (80%) underwent a late reoperation for obstruction of the extracardiac conduit, and in four of these patients, a reoperation for LVOTO was concomitantly required. Reoperation was also required in six patients of the Lecompte group (25%); five for right ventricular outflow tract obstruction (RVOTO) including one for LVOTO and two for VSD leakage, and one for mitral regurgitation and left pulmonary artery stenosis. The interval prior to reoperation ranged from 1.6 to 11.1 years, with a mean of 5.7+/-3.1 years. The actuarial figures for freedom from reoperation at 5 and 10 years were 40.0+/-15.5 and 26.7+/-15.0% after the Rastelli operation and 95.7+/-4.3 and 63.5+/-12.6% after the Lecompte procedure (P = 0.02). Multivariate analysis by Cox regression analysis revealed that the risk factors of RVOTO were a younger age at operation, the Rastelli operation, and ductus ligation during the operation. CONCLUSIONS: The Lecompte procedure and Rastelli repair provide satisfactory early and late results. However, substantial late morbidity is more associated with conduit obstruction, and LVOTO in Rastelli repair rather than Lecompte procedure.     

Results of the Damus-Stansel-Kaye procedure for transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect

From 1975 through 1982, the Damus-Stansel-Kaye procedure was performed on 20 patients with complete transposition of the great arteries (TGA) and on 4 with double-outlet right ventricle (DORV) and subpulmonary ventricular septal defect (VSD). The patients ranged from 6 days to 20 years old (median age, 13 months). Associated anomalies included atrial septal defect (24 patients), VSD (14), and others (25). Thirteen patients had had palliative operations previously. Of the 14 hospital deaths (58%), 13 occurred among the 17 patients with one or more risk factors: age less than 18 months, weight less than 10 kg, and left ventricular peak systolic pressure less than 75% of systemic pressure. Follow-up ranged from 12 to 87 months (mean, 51 months). One patient died of cardiac failure two years postoperatively, and 2 required conduit replacement at 40 and 50 months because of stenosis. All 9 survivors are free from major symptoms. The Damus-Stansel-Kaye repair is most suitable for patients with TGA or DORV with subpulmonary VSD who are older than 18 months, weight more than 10 kg, and have a "prepared left ventricle," and whose coronary artery anatomy precludes transplantation.     

Concomitant repair of complete atrioventricular canal defect and transposition of the great arteries in an infant

The case of an infant who was born with the rare presentation of complete atrioventricular canal defect and transposition of the great arteries is described. The patient underwent pulmonary banding at the age of 2 months, followed by concomitant arterial switch and repair of the atrioventricular canal defect at 20 months. Palliative banding allowed for the infant to grow and thus facilitated the later successful repair of these congenital defects.     

Surgery for partial atrioventricular septal defect in the adult

BACKGROUND: We sought to determine the clinical profile, operative results, and long-term outcome for adult patients undergoing operations for partial atrioventricular septal defects. METHODS: Between 1976 and 1996, 50 adults (mean age, 36.6 +/- 13.2 years) underwent surgery for partial atrioventricular septal defects. Thirty-nine of them underwent primary repair for a substantial left-to-right shunt (> or =1.8), associated with symptoms in 29. The remaining 11 patients had previous atrioventricular septal defect repair in childhood, but required reoperation as adults for severe left atrioventricular valve regurgitation (6), subaortic (3) or mitral (1) stenosis, and a residual atrial shunt (1). RESULTS: No patients died in hospital. Of the 39 patients first repaired in adulthood, left atrioventricular valve repair was performed in 37, valve replacement in 1, and no repair in 1. In contrast, left atrioventricular valve replacement was necessary in 2 of the 6 adults undergoing reoperation for left atrioventricular valve regurgitation. At 7 years median follow-up, 8 patients have died (2 from noncardiac causes). Of 42 patients alive in 1997, 39 are New York Heart Association class I or II, and 3 were class III (class improved in 81%). Two patients required left atrioventricular valve replacement (1 week and 5 years after repair, respectively) for valvar failure. CONCLUSIONS: Low operative risk and excellent long-term results support repair of partial atrioventricular septal defect in adults.