Primary definitive repair of interrupted aortic arch with ventricular septal defect

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.     

Successful biventricular repair after initial Norwood operation for interrupted aortic arch with severe left ventricular outflow tract obstruction

Background. Management of newborns with interrupted aortic arch (IAA) remains challenging. Associated severe left ventricular outflow tract obstruction (LVOTO) have often led to increased mortality with neonatal biventricular repair. We review our experience with an alternative approach for this complex surgical problem.

Methods. From May 1991 to June 1999, 28 neonates were treated for IAA. Thirteen of 28 neonates (46%) had type B IAA, ventricular septal defect (VSD) and severe LVOTO (Z value –2 to –7; mean –5 ± 1.7). Mean age was 8 days (3 to 23 days old) with average weight of 3.3 kg (2.4 to 4.2 kg). Eight of 13 (62%) had anomalous right subclavian artery. Ten of 13 (77%) had thymic aplasia and chromosome 22 region q11 deletion. All 13 patients were treated initially with a modified Norwood procedure.

Results. There were no perioperative deaths. Complications included 2 patients with recurrent arch stenosis treated with balloon dilatation. Two patients had systemic arterial shunt revision. Follow-up ranged from 2 to 99 months old (mean 39 months). There were 2 late deaths unrelated to any operation. Nine of 12 patients had a second stage palliation consisting of a bidirectional Glenn shunt. Six patients went on to have biventricular repairs (3 Ross-Konno, 2 Rastelli, 1 VSD closure with LVOT resection). One patient had a modified Fontan operation and 5 patients are awaiting potential biventricular repair.

Conclusions. Children with IAA and severe LVOTO may be managed by initial Norwood palliation with an excellent outcome likely. This initial “univentricular” approach has enabled eventual successful biventricular repair despite severe LVOTO.     

Neonatal Ross Konno procedure with aortic arch repair—a case report

A 14-day-old neonate presented with severe left ventricular outflow tract obstruction (LVOTO) and type B interrupted aortic arch. The child underwent a neonatal Ross Konno with interposition graft for arch reconstruction.     

Two cases of Konno's operation for aortic stenosis developing after repair of interruption of the aortic arch with ventricular septal defect

Two patients, 8-year-old and 7-year-old boys, underwent Konno's operation for left ventricular outflow tract obstruction, which had developed after the repair of interrupted aortic arch and ventricular septal defect. Both cases had bicuspid aortic valve and severe subaortic stenosis. The annular diameters were 15 mm and 16 mm, and pressure gradients between the left ventricle and the ascending aorta were 110 mmHg and 105 mmHg, respectively. A SJM prosthetic valve (23 mm, 25 mm) was used. Postoperative courses were good. Left ventricular outflow anomalies are frequently found in cases of interrupted aortic arch complicated with ventricular septal defect. The stenosis not infrequently develops and becomes apparent several years after primary or staged repair for interrupted arch with VSD as in the present cases. Therefore, careful observation should be carried out following the repair. Konno's operation can be safely performed for this lesion with low mortality and morbidity for valvular and subvalvular aortic stenosis even with small aortic ring.     

Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: a Congenital Heart Surgeons Society study

OBJECTIVE: We sought to determine the prevalence of outcomes and associated patient and management factors for neonates with interrupted aortic arch. METHODS: From 1987 to 1997, a total of 472 neonates were enrolled prospectively from 33 institutions. Competing risks methodology was used to determine simultaneous risk and associated incremental risk factors for death, initial and subsequent left ventricular outflow tract procedures, and arch reinterventions. RESULTS: Overall survival was 59% at 16 years after study entry but improved with successive birth cohort. In general, risk factors for death in each of the competing risks analyses included lower birth weight, younger age at study entry, type B interrupted aortic arch, and major associated cardiac anomalies. Of 453 patients who had interrupted aortic arch repair, after 16 years 33% had died and 28% had undergone an arch reintervention. Reintervention was more likely for those who had truncus arteriosus repair, interrupted aortic arch repair by a method other than direct anastomosis with patch augmentation, and the use of polytetrafluoroethylene as either an interposition graft or a patch. From study entry, competing risks after 16 years showed that 28% had died and 34% had undergone an initial left ventricular outflow tract procedure. Initial left ventricular outflow tract procedure was more likely for those with single ventricle, type B interrupted aortic arch, bicuspid aortic valve, or anomalous right subclavian artery. Among those who had undergone an initial left ventricular outflow tract procedure, after 16 years 37% had died and 28% had undergone a second procedure. CONCLUSION: Anatomic features affect mortality and initial left ventricular outflow tract procedures, whereas characteristics of the arch repair affect arch reintervention.     

Primary biventricular repair for interrupted aortic arch with left ventricular outflow tract obstruction and tricuspid valve regurgitation; report of a case

An interrupted aortic arch was diagnosed in a 10-day-old girl weighing 3.3 kg, as was perimembranous ventricular septal defect (VSD) and severe tricuspid valve regurgitation (TR). The subaortic diameter was 3.6 mm and the aortic valve (3.7 mm in diameter) was bicuspid. We chose definitive repair, modified Yasui procedure, because of severe TR and no straddling of mitral valve. In primary biventricular repair, we undertook extended aortic arch anastomosis. Left ventricular outflow tract reconstruction consisted of intracardiac rerouting from the VSD to the pulmonary artery by using expanded-polytetrafluoroethylene (ePTFE) and Damus-Kaye-Stansel (DKS) anastomosis. Right ventricular outflow tract reconstruction was performed by the Rastelli procedure with an ePTFE valved conduit. Moreover, we carried out semicircular annuloplasty for severe TR.     

One stage repair of aortic arch anomalies and intracardiac defects

Between August 1985 and May 1990, 27 neonates and infants underwent combined correction of intracardiac and aortic arch anomalies through a median sternotomy. Coarctation (CoA) was combined with VSD (6), AVSD (2), Taussig-Bing (TB) heart (5), transposition of the great arteries (TGA) (1), TGA + VSD (2), congenitally corrected TGA + VSD (1) and VSD + myxoid stenotic outlet valves (1). Interrupted aortic arch (IAA) was combined with VSD (10) and TB heart (1). Two patients had IAA type B as well as CoA. Age at operation varied from 2 to 243 days with a mean age of 51 days. Twenty patients (70%) were younger than 30 days. One TGA + VSD and all TB hearts had maligned outlet septum and right ventricular outflow tract obstruction (RVOTO). Posterior outlet septum deviation and left ventricular outflow tract obstruction (LVOTO) occurred in 8 patients with malalignment VSD and IAA (7) or CoA (1). Aortic arch reconstruction was performed using extended end-to-end anastomoses. In 3 patients, arch hypoplasia necessitated patch implantation. VSDs were closed through the right atrium unless the anatomy dictated otherwise. One TB heart was reconstructed with a Kawashima procedure. All other TB hearts and all TGAs were corrected with arterial switch operation. Obstructing outlet septum was resected whenever necessary. Follow-up was complete and included echo-Doppler control. Eleven patients had postoperative heart catheterisation. Early mortality was 18.5% (5 patients). Persisting LVOTO or RVOTO was responsible. There was no late mortality. Five patients were reoperated upon: 3 for stenotic anastomoses and 2 for a subaortic membranous stenosis. Successful balloon dilatation of recoarctation was performed four times.(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical treatment of interrupted aortic arch in infants under three months of age

From March, 1982, through March, 1988, 8 infants less than 3 months of age underwent surgical treatment of interrupted aortic arch. Five patients had IAA type A and 3 patients had type B. Seven patients with associated VSD underwent staged operations and 1 infant with aortopulmonary window underwent primary repair off cardiopulmonary bypass. Aortic arch reconstruction was by subclavian aortic anastomosis (6) or large tube graft (8 mm) interposition (2) combined with pulmonary artery banding (5). All seven patients with associated VSD survived the first-stage operation. One patient subsequently underwent pulmonary artery banding, two underwent patch aortoplasty and four patients underwent intracardiac repair and removal of a pulmonary artery band, with six survivors (86%). The operative death occurred in an infant in whom modified Damus-Kaye-Stansel operation was carried out for severe subaortic stenosis, which rerouting all left ventricular blood through VSD and the main pulmonary artery into the ascending aorta and reconstructing right ventricular-distal pulmonary artery connection by a valved external conduit. All six surviving patients are doing well at present (mean follow up of 3 years) without any significant pressure gradient between the ascending and thoracic aorta. Based on these data we conclude: (1) Aortic arch reconstruction and pulmonary artery banding can be reliably performed even in critically ill infants with IAA and isolated VSD. (2) The primary repair will provide better chance of survival in infants with IAA associated with significant LVOTO, truncus arteriosus or aortopulmonary window that do not readily lend themselves to pulmonary artery banding.     

Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair

There is uncertainty regarding the best method of repair of interrupted aortic arch. The question is whether to perform primary definitive repair of this anomaly plus the associated defects versus arch repair only and palliation of the intracardiac defects, usually by pulmonary artery banding. Since 1976, 16 infants with interrupted aortic arch have been treated surgically. They were seen at 5.2 +/- 3 days of age and weighed 3.2 +/- 0.7 kg. The interruption occurred between the left carotid and left subclavian arteries (type B) in 9 and between the left subclavian artery and the descending aorta in 7 (type A). Isolated ventricular septal defect (VSD) was the only associated anomaly in 7 and aortopulmonary window, in 4. Two patients had truncus arteriosus type 1. Three had transposition of the great arteries: 1 with VSD and 2 with single ventricle. Prior to 1980, our policy was to palliate all patients. Between 1976 and 1980, 4 infants underwent left thoracotomy with arch repair plus pulmonary artery banding (3, VSD; 7, transposition of the great vessels and single ventricle) with only 1 (25%) survivor. Because of this high mortality, 8 patients with interrupted aortic arch and VSD or aortopulmonary window, seen since 1980, received complete repair with median sternotomy, end-to-end arch anastomosis, and closure of the VSD or aortopulmonary window utilizing profound hypothermia and circulatory arrest. All 8 survived.(ABSTRACT TRUNCATED AT 250 WORDS)     

Neonatal repair of right interrupted aortic arch with cerebro-myocardial perfusion technique

Right interrupted aortic arch and descending aorta is exceedingly rare and most likely cause respiratory presentation, since patent ductus arteriosus (PDA) courses over the right mainstem bronchus. We report a case of successful neonatal biventricular repair of a right interrupted aortic arch (type B), with an aberrant right subclavian artery ventricular septal defect (VSD) in a 2.7?kg term neonate with DiGeorge syndrome. Patient presented in severe respiratory distress and acidosis at one day old. Two-dimensional (2D) echocardiography revealed aortic arch interruption beyond the common carotid arteries with large perimembranous outlet VSD. Aortic annulus diameter was 4.8?mm and there was no left ventricle (LV) outflow tract obstruction. Three-dimensional (3D) CT-scan confirmed these findings and identified a right-sided ductal arch that continued over the right mainstem bronchus into a right-sided descending aorta and aberrant right subclavian artery. Brachiocephalic perfusion and ductal perfusion was employed for cooling during cardiopulmonary bypass. Under deep hypothermia (27 °C rectal temperature), selective cerebro-myocardial perfusion was used for successful aortic arch repair without sacrificing the aberrant right subclavian artery. A direct tension-free anastomosis was attained. Her postoperative course was uneventful and her respiratory symptoms disappeared postoperatively. Early surgical correction is mandatory for these patients with unique anatomy and presentation.     

A case of tracheo-bronchial stenosis after extended end-to-end aortic arch anastomosis for interrupted aortic arch treated with suspension of the ascending artery and pulmonary artery

A 9-day-old boy had pulmonary artery banding and extended end-to-end aortic arch anastomosis for ventricular septal defect (VSD) and type A interrupted aortic arch. Severe dyspnea gradually developed. At 3 months of age, intracardiac repair of VSD was performed. Weaning from the ventilator was difficult. Endoscopic examination and chest CT revealed stenosis of the right and left main bronchi and compression of tracheal bifurcation and the right and left main bronchi by the ascending aorta and pulmonary artery. Suspension of the ascending aorta and pulmonary artery was performed 15 days after VSD closure. Nine days after this procedure, the patient was weaned from respirator. Postoperative course was uneventful. Bronchial stenosis may be caused from extended end-to-end aortic arch anastomosis.     

Primary repair for aortic arch obstruction associated with ventricular septal defect

Background

The aim of the present study was to evaluate the current outcome and reoperation rate after applying a one-stage correction strategy for interrupted aortic arch (IAA) with ventricular septal defect (VSD) and also for aortic coarctation and hypoplastic aortic arch (CoA-HyAA) with VSD beginning September 1999.

Methods

Twenty-four consecutive patients with IAA (n = 12) or CoA-HyAA (n = 12) with VSD underwent early one-stage correction. Patients' mean age was 12 days (range, 2 to 188); mean weight was 3.6 kg (range, 2.1 to 7.3), 6 patients were less than 2.5 kg. Three IAA were type A, 5 type B1, 3 type B2, and 1 type C. Associated anomalies included a large VSD in all, left ventricular outlet tract obstruction in 5, transposition of the great arteries, aortopulmonary window, persistent truncus arteriosus, and double-outlet right ventricle in 1 patient. Selective brain perfusion through innominate artery and selective coronary perfusion through aortic root during aortic arch reconstruction was used in all patients. Mean follow-up was 2.2 ± 0.9 years.

Results

There was no early, no late mortality, and no postoperative neurologic complications. Mean crossclamp duration was 72 ± 32 minutes, lowest temperature 22.8 ± 4°C and selective brain and coronary perfusion duration was 34 ± 13 minutes. Eighteen patients required delayed sternal closure at 1.7 days postoperatively. New perioperative management reduced the overall morbidity. Four patients after IAA plus VSD repair developed aortic arch restenosis and were successfully treated by balloon dilatation. One patient with d-TGA underwent right ventricular outflow tract reconstruction of right ventricular outlet tract obstruction 7 months after the initial repair. Pressure gradients across the anastomosis at most recent follow up were less than 10 mm Hg. All patients are asymptomatic and are developing normally.

Conclusions

One-stage complete correction is feasible in newborns with aortic arch obstruction with VSD. Complex cardiac anatomy presents no additional risk for the procedure. The early one-stage correction yields excellent surgical results and good functional outcome.     

Simplified method for total correction of interrupted aortic arch with ventricular septal defect in infancy.

Type A interrupted aortic arch with a ventricular septal defect (VSD) in a 3-month-old infant was successfully corrected with the aid of profound hypothermia, limited cardiopulmonary bypass, and total circulatory arrest. The aortic arch was reconstructed by side-to-side anastomosis of the ascending aorta and the main pulmonary artery and then creation of a tube from the anastomotic orifice to the patent ductus arteriosus (PDA) by using a superabundant flap of the anterior wall of the main pulmonary artery. The pulmonary arteriotomy and VSD then were closed. The operative field can be approached easily through a median sternotomy with minimum dissection. Cardiac catheterization and angiocardiography 2 months later demonstrated a satisfactory reconstruction of both the aortic arch and the pulmonary artery.     

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.     

Modified Rastelli using an autograft: A new concept for correction of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction (with an extension to other congenital heart defects)

We describe in this article a new approach in transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO). It consists of an operation combining the intracardiac infundibular resection of the Lecompte procedure to allow a direct VSD-aorta connection and the right ventricle-pulmonary artery (RV-PA) connection with a segment of tubular aortic autograft, (with or without an additional monocusp patch). Thus, a viable conduit, capable of growth, is interposed between RV and PA. We have performed this procedure in 19 patients (age, 2 months to 11 years), 6 being under 1 year. All patients are doing well after a maximum follow-up of 72 months, exhibiting growth of the conduit and satisfactory RV pressures. This new approach allows primary correction even in the young and can be extended, we think, for other defects with pulmonary stenosis or atresia. It shares, however, with many other procedures, the valveless nature of the repair with the potential late need for valve replacement surgery. Copyright © 2000 by W.B. Saunders Company     

Staged repair of interrupted aortic arch and ventricular septal defect in infancy

Staged repair of interrupted aortic arch and ventricular septal defect was carried out in 20 infants from 1979 through 1990. Among the important associated cardiac defects were transposition of the great arteries, truncus arteriosus, and anomalous origin of the right pulmonary artery. The first stage, usually consisting of the placement of an 8- or 10-mm polytetrafluoroethylene graft, pulmonary artery banding, and ligation of the patent ductus arteriosus, resulted in 20 survivors (100%) There were two interim deaths (10%) before the second stage of ventricular septal defect closure and pulmonary artery band removal, which had 15 survivors (83%, 15/18). Because the major morbidity and mortality early in this experience could be traced to leaving the pulmonary artery band on too long, early removal (within 2 to 3 months) was begun. Since 1985, 8 (100%) of 8 infants have survived both stages and are now doing well. Because of the relatively large polytetrafluoroethylene graft, only 1 child (aged 9 years) has experienced substantial late aortic arch obstruction and undergone placement of an 18-mm Dacron graft without difficulty. Of interest is the finding that in only 1 (5%) of the 20 patients has major (greater than or equal to 40-mm Hg gradient) left ventricular outflow tract obstruction developed. In summary, the staged repair of interrupted aortic arch with ventricular septal defect has become very reliable despite the condition of the infant or major associated cardiac anomalies and can be recommended for infants at high risk for primary repair. More long-term information will be needed to determine which approach will be the best choice for the majority of infants.     

Surgical management of tunnel-like subaortic stenosis via ventricular septal defect in a patient with the interrupted aortic arch

A 24-day-old male with interrupted aortic arch (type B), ventricular septal defect, and tunnel-like subaortic stenosis underwent a one-stage surgical treatment The operative procedure comprised reconstruction of the aortic arch, transatrial excision of the subaortic fibromuscular tissue via the ventricular sepatal defect, and patch closure of the defect. The patient tolerated the procedure well and the postoperative echocardiography demonstrated a residual pressure gradient across the left ventricular outflow tract of 20 mmHg. Our result suggests that the transatrial surgical management of subaortic stenosis via the ventricular sepatal defect produces a safe and promising surgical option.     

Repair of interrupted aortic arch: results after more than 20 years

Background. This study focused on the influence of concomitant anomalies, the individual surgical approach, and the probability for reinterventions.

Methods. Between 1975 and 1999, 94 patients with interrupted aortic arch were evaluated for short- and long-term results after surgical treatment.

Results. Interrupted aortic arch was associated mainly with a ventricular septal defect (85%) and left ventricular outflow tract obstruction (LVOTO, 13%). Mean follow-up was 6.7 years (median 6.9 years, 628.4 patient years). A single-stage operation was performed in 76 cases. Early mortality for two-stage procedures was 37% and late mortality was 26%, compared with single-stage procedures, with an early mortality of 12% and a late mortality of 20%, respectively. Early mortality in patients with additional LVOTO was 42% and late mortality was 50%. Freedom from reoperation at 5 years was 62%, and at 10 years was 49%. Reinterventions were performed mainly for residual arch stenosis, also with bronchus or tracheal compression, or LVOTO.

Conclusions. Arch continuity and repair of associated anomalies can be achieved with an acceptable overall risk in this often complex entity. Associated anomalies play an important role in the outcome. Single-stage repair with primary anastomosis of the arch should be the surgical goal. The long-term probability for reoperation is high.     

Early and long term outcome of the arterial switch operation for transposition of the great arteries: predictors and functional evaluation

Objectives: The aims of this report were to study the early and late outcome in terms of mortality, freedom from reoperation, predictors for late pulmonary stenosis (PAS) and insufficiency of the neo-aortic valve (AVI) in patients with transposition of the great arteries (TGA) undergoing arterial switch operation (ASO). Materials and methods: Between January 1990 and December 2001, 134 patients with TGA underwent ASO. The patients were divided in Group I (n=88)-TGA with intact ventricular septum and Group II (n=46)-TGA with ventricular septal defect (VSD). The pulmonary artery was reconstructed employing the direct anastomosis technique (PT-I) in 21 (15.7%) patients, the double-patch technique (PT-II) in 41 (30.6%), single pantaloon patch (partial circumference) (PT-III) in 46 (34%) and single pantaloon patch (total circumference) (PT-IV) in 35 (26%) patients. The mean follow-up was 3.4±1.3 years. Results: The hospital mortality was 17 (12.7%) patients. The mortality in Group I was significantly lower than Group II (P=0.002). The overall actuarial survival at 1, 3 and 5 years follow-up resulted to be 98, 93, and 91.5%, resulting to be significantly higher in Group I (P=0.032). The multivariate analysis revealed the complex TGA (P=0.007), VSD (P=0.032), coronary anomalies (P=0.004), aortic coarctation or hypoplastic aortic arch (P=0.021), left ventricular outflow tract obstruction (LVOTO) or moderate PAS (P=0.041) as strong predictors for poor free-reoperation cumulative survival. A strong inverse correlation was found between the mean trans-pulmonary gradient at follow-up and the age at the operation (r=−0.41, P<0.0001). The univariate analysis revealed the PT-I technique (P=0.002), prior moderate PAS (P=0.0001), and age <1 month (P=0.018) as strong predictors for moderate-to-severe PAS. The neo-AVI incidence was significantly higher in Group II (P=0.011). Predictors for neo-AVI were male sex (P=0.003), preoperative neo-AV Z-score >1 (P<0.001), prior or concomitant operation for aortic coarctation or hypoplastic aortic arch (P=0.001), LV retraining (P=0.003). Conclusion: ASO remains the procedure of choice for the treatment of various forms of TGA with acceptable early and later outcome in terms of overall survival and free reoperation. Strong predictors for poor overall free-reoperation survival are complex TGA, VSD, coronary anomalies, aortic coarctation and LVOTO or moderate PAS. The pulmonary artery reconstruction using a single ‘pantaloon patch’ seems to offer less residual stenosis. Patients with a VSD and a significant mismatch between the neo-aortic root and distal aorta are at a higher risk for developing postoperative neo-AVI.     

Neonatal repair of right interrupted aortic arch, aberrant left subclavian artery, ventricular septal defect and retroaortic innominate vein.

We report a rare case of neonatal biventricular repair of a right interrupted aortic arch (type B), with an aberrant left subclavian artery, ventricular septal defect and retroaortic innominate vein in a 4-week-old, 2.7 kg neonate with DiGeorge syndrome. The patient had an unremarkable postoperative recovery. We discuss the anatomy of this rare congenital anomaly, its surgical implications and issues surrounding the adequacy of the left ventricular outflow tract.