Ventricular Septal Defect Enlargement Research Articles

Long-term results after the réparation à l'étage ventriculaire procedure for transposition of the great arteries and double-outlet right ventricle with pulmonary stenosis.

The purpose of this study is to describe the long-term results of the 'réparation à l'étage ventriculaire' (REV) technique for double-outlet right ventricle and transposition of the great arteries (TGA) with pulmonary stenosis (PS). Between 1980 and 2021, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, ventricular septal defect and PS (n = 116, 73.9%). Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 109 patients (69.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89.3%. Thirty-seven patients (23.6%) required 68 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months to 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6%, respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years. The REV procedure is a good alternative for TGA and double-outlet right ventricle with PS patients. Only a quarter of the patients required redo surgery on the RVOT. Reoperations for left ventricular outflow tract obstruction are scarce.

Long-term results after the REV (réparation à l’étage ventriculaire) procedure for malposition of the great arteries

Several surgical techniques can be used to repair anomalies of the ventriculoarterial connection with sub-pulmonary stenosis. Although there is no equivocal evidence that one technique is superior to another, the arterial switch operation (ASO) and Rastelli procedure are the preferred options to correct transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO). Despite innovative features and satisfactory short- and long-term results, the “réparation à l’étage ventriculaire” (REV) procedure has not gained large popularity and still struggles to establish itself as a legitimate alternative to the Rastelli operation. By describing our long-term results, we wish to address the misconceptions about the technical aspects and the long-term results of this technique that have been suggested to explain the reluctance towards it. Between 1980 and 2022, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, VSD and LVOTO ( n = 113, 72.0%). Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) had a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 65 patients (41.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89%. Thirty-seven patients (23.6%) required 67 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months – 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6% respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years ( Fig. 1 ). The REV procedure is a good alternative to the Rastelli intervention when an arterial switch operation is not feasible. A quarter of the patients required redo surgery on the right ventricular outflow tract. Reoperations for left ventricular outflow tract obstruction are scarce.

Long-term results of biventricular correction for patients with double outlet right ventricle.

The purpose of this study was to report outcomes of double outlet right ventricle biventricular repair at a single institution in developing countries and to investigate post-operatively determined risk factors for mortality and late intervention. Patients with double outlet right ventricle who underwent biventricular repair in our centre from January 2009 to December 2021 were included. A total of 96 patients (male/female = 61/35) with biventricular repair were included. According to its specific anatomical type, the appropriate individual surgical plan was selected. Multivariate analysis indicated that prolonged cardiopulmonary bypass time(OR = 1.044; p = 0.012) and pre-operative moderate or above pulmonary hypertension(OR = 24.558; p = 0.023) were independent risk factors for early mortality. Univariate analysis showed that different anatomical types and different surgical methods had similar late intervention and late mortality. Concomitant coarctation of the aorta(OR = 40; p = 0.020) and concomitant ventricular septal defect enlargement(OR = 26.667; p = 0.005) were independent risk factors for late intervention by multivariate analysis. Selection of appropriate surgical techniques based on different anatomical types often results in similar late outcomes. For patients with concomitant ventricular septal defect enlargement during the operation, it is necessary to fully expand and avoid damage to the conduction bundle. We should timely intervention in patients with coarctation of the aorta and pay attention to the occurrence of left ventricular outflow tract obstruction during follow-up.

Results of the arterial switch operation for Taussig-Bing variants in the setting of a lower-middle income country: A single-institution experience.

This study was conducted to evaluate the surgical results of the arterial switch operation for Taussig-Bing variants, at a single institution in a lower-middle income country. Between June 2010 and December 2018, all consecutive patients diagnosed with Taussig-Bing variants who underwent the arterial switch operation and ventricular septal defect closure were included in the study. A total of 72 patients of Taussig-Bing variants underwent arterial switch operation and ventricular septal defect closure. There were 10 early deaths (13.9%) and 2 late deaths (2.8%). Intraoperative ventricular septal defect enlargement (hazard ratio [HR] 7.23, 95% confidence interval [CI] 3.1294-16.7167; p < .001), secondary aortic cross-clamping (HR 28.38, 95% CI 4.8427-166.3484; p < .001), postoperative pneumonia (HR 5.64, 95% CI 1.2724-24.9917; p = .023), and postoperative sepsis (HR 5.28, 95% CI 1.3512-20.6553; p = .017) were risk factors for overall mortality by competing risk analysis. Sixty patients (83.3%) required septoparietal trabeculation division/resection during the arterial switch operation in an attempt to avoid right ventricular outflow tract obstruction. The reoperation rate for right ventricular outflow tract obstruction at last follow up was 6% (three patients). The estimated freedom from reoperation for right ventricular outflow tract obstruction at 1, 5, and 9 years was 98.3%, 91.9%, and 91.9%, respectively. The results of arterial switch operation for Taussig-Bing variants were satisfactory in the operative setting of a lower-middle income country, and performing extensive septoparietal trabeculation division might reduce the reintervention rate for right ventricular outflow tract obstruction in these patients.

Single-center experience with routine clinical use of 3D technologies in surgical planning for pediatric patients with complex congenital heart disease.

This study was planned to assess the application of three-dimensional (3D) cardiac modeling in preoperative evaluation for complex congenital heart surgeries. From July 2015 to September 2019, 18 children diagnosed with complex congenital heart diseases (CHDs) were enrolled in this study (double outlet right ventricle in nine patients, complex types of transposition of the great arteries in six patients, congenitally corrected transposition of the great arteries in two patients, and univentricular heart in one patient). The patients' age ranged from 7 months to 19 years (median age, 14 months). Before the operation, 3D patient-specific cardiac models were created based on computed tomography (CT) data. Using each patient's data, a virtual computer model (3D mesh) and stereolithographic (SLA) file that would be printed as a 3D model were generated. These 3D cardiac models were used to gather additional data about cardiac anatomy for presurgical decision-making. All 18 patients successfully underwent surgeries, and there were no mortalities. The 3D patient-specific cardiac models led to a change from the initial surgical plans in 6 of 18 cases (33%), and biventricular repair was considered feasible. Moreover, the models helped to modify the planned biventricular repair in five cases, for left ventricular outflow tract obstruction removal and ventricular septal defect enlargement. 3D cardiac models enable pediatric cardiologists to better understand the spatial relationships between the ventricular septal defect and great vessels, and they help surgeons identify risk structures more clearly for detailed planning of surgery. There was a strong correlation between the models of the patients and the anatomy encountered during the operation. 3D cardiac models accurately reveal the patient's anatomy in detail and are therefore beneficial for planning surgery in patients with complex intracardiac anatomy.

Comparison of half-turned truncal switch and conventional operations.

To compare conventional procedures with the half-turned truncal switch operation (HTTSO) for the management of complete transposition of the great arteries with left ventricular outflow tract (LVOT) obstruction using time-resolved 3-dimensional magnetic resonance phase-contrast imaging. We identified 2 cases that underwent the Rastelli procedure and one case that underwent the Réparation a l'étage ventriculaire before 2002 [conventional procedures group (group C)], and 16 cases of HTTSO that were performed between 2002 and 2020 [HTTSO group (group H)]. Postoperative haemodynamics were assessed using time-resolved 3-dimensional magnetic resonance phase-contrast imaging in cases in both groups. The median follow-up period was 20.4 years in group C, and 6.1 years in group H. In group C, all 3 patients underwent reoperation because of postoperative right ventricular outflow tract obstruction and/or insufficiency. In addition, permanent pacemaker implantation was needed in 1 patient because of complete atrioventricular block complicated by ventricular septal defect enlargement. In group H, reoperation for LVOT/right ventricular outflow tract obstruction was not needed. A time-resolved 3-dimensional magnetic resonance phase-contrast imaging examination revealed high energy loss and wall shear stress in the winding LVOT in the group C. In contrast, low energy loss and wall shear stress, with straight and smooth LVOT, were identified in group H. HTTSO was shown to be superior to conventional procedures because a straight and wide LVOT could be obtained. Therefore, HTTSO should be the first choice for complete transposition of the great arteries with LVOT obstruction.

Damus-Kay-Stansel procedure with ventricular septal defect enlargement.

A full-term infant who had tricuspid atresia with transposed great arteries, a ventricular septal defect, subpulmonary stenosis with posterior malalignment of the conus septum, bicuspid pulmonary valve, and a high-takeoff left coronary artery was referred to our institution. The subpulmonary stenosis gradually progressed and cyanosis worsened. We successfully performed a Damus-Kay-Stansel procedure and a bidirectional Glenn shunt concomitant with ventricular septal defect enlargement. The conus septum was resected along with thick fibrous tissue through both semilunar valves (without ventriculotomy). Postoperative echocardiography demonstrated that both the ventricular septal defect and the subpulmonary space were enlarged effectively without semilunar valve regurgitation.

Rastelli Operation for D-Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis.

Our preferred approach for the surgical treatment of patients with D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis has been the Rastelli operation. We herein evaluate our 30-year experience with this procedure. Clinical records for patients who underwent the Rastelli operation between 1988 and 2017 at our institution were retrospectively reviewed. Primary outcomes included freedom from death or cardiac transplantation and freedom from conduit reintervention. Forty-seven patients met inclusion criteria. Mean follow-up was 11.7 ± 6.8 years. Forty-three (91.5%) patients received a palliative systemic-to-pulmonary artery shunt and/or atrial septostomy prior to the Rastelli procedure. Five (10.6%) patients required ventricular septal defect enlargement at the time of the Rastelli procedure. The overall mean right ventricle-to-pulmonary artery conduit size was 17.0 mm. Mortalities included one early and three late deaths. Freedom from death or cardiac transplantation was 93% and 84% at 5 and 25 years, respectively. Seven patients required pacemaker placement, two immediately postoperatively and five late. Freedom from conduit replacement was 85% and 25% at 5 and 15 years, respectively. Seven (14.9%) patients required a second conduit intervention. Forty-one (87.2%) patients were New York Heart Association class I or II at the most recent follow-up. The Rastelli operation for D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis offers excellent mid- to long-term survival. The need for conduit replacement remains the most common indication for reintervention, and further study of the optimal choice of conduit will be useful.

Surgical Treatment of Double Outlet Right Ventricle Complicated by Pulmonary Hypertension

Background:Double outlet right ventricle (DORV) is a group of complex congenital heart abnormalities. Preoperative pulmonary hypertension (PH) is considered an important risk factor for early death during the surgical treatment of DORV. The aim of this study was to report our experience on surgical treatment of DORV complicated by PH.Methods:From June 2004 to November 2016, 61 patients (36 males and 25 females) aged 2 weeks to 26 years (median: 0.67 years and interquartile range: 0.42–1.67 years) with DORV (two great arteries overriding at least 50%) complicated by PH underwent surgical treatment in our center. All patients were categorized according to surgical age and lesion type, respectively. Pulmonary artery systolic pressure (PASP), pulmonary artery diastolic pressure (PADP), and mean pulmonary artery pressure (mPAP) were measured directly before cardiopulmonary bypass (CPB) was established and after CPB was removed. An intracardiac channel procedure was performed in 37 patients, arterial switch procedure in 19 patients, Rastelli procedure in three patient, Senning procedure in one patients, and Mustard procedure in one patient. The Student's t-test and Chi-squared test were performed to evaluate clinical outcomes of the surgical timing and operation choice.Results:Fifty-five patients had uneventful recovery. PASP fell from 55.3 ± 11.2 mmHg to 34.7 ± 11.6 mmHg (t = 14.05, P < 0.001), PADP fell from 29.7 ± 12.5 mmHg to 18.6 ± 7.9 mmHg (t = 7.39, P < 0.001), and mPAP fell from 40.3 ± 10.6 mmHg to 25.7 ± 8.3 mmHg (t = 11.85, P < 0.001). Six (9.8%) patients died owing to complications including low cardiac output syndrome in two patients, respiratory failure in two, pulmonary hemorrhage in one, and sudden death in one patient. Pulmonary artery pressure (PAP) dropped significantly in infant and child patients. Mortality of both infants (13.9%) and adults (33.3%) was high.Conclusions:PAP of patients with DORV complicated by PH can be expected to fall significantly after surgery. An arterial switch procedure can achieve excellent results in patients with transposition of the great arteries type. Higher incidence of complications may occur in patients with ventricular septal defect (VSD) type before 1 year of age. For those with remote VSD type, VSD enlargement and right ventricle outflow tract reconstruction are usually required with acceptable results. The degree of aortic overriding does not influence surgical outcome.

Long-term Effect of Enlargement of a Ventricular Septal Defect in the Rastelli Procedure

We investigated the effect of ventricular septal defect (VSD) enlargement on long-term surgical results, late arrhythmia, and left ventricular (LV) function in the Rastelli procedure for D-transposition of the great arteries with LV outflow tract obstruction (LVOTO). From 1979 to 2001, 74 patients (D-transposition of the great arteries, n = 56; double outlet right ventricle, n = 18) underwent the Rastelli procedure. In group A, 46 patients underwent the Rastelli procedure with VSD enlargement, and in group B, 28 patients underwent the Rastelli procedure without enlargement. There were no hospital deaths. Actuarial survival at 20 years was 80% in group A and 91% in group B (P = 0.50). Freedom from reoperations at 20 years was 40.1% in group A and 52.0% in group B. Reoperations for LVOTO were performed in 2 patients in both groups. In postoperative catheterization, LV ejection fraction in group A was 57.1% ± 8.7% vs 57.2% ± 8.1% in group B (P = 0.97); LV end-diastolic volume, 150.0% ± 47.2% vs 142.0% ± 36.9% of the normal volume (P = 0.97). In long-term postoperative echocardiography, the pressure gradient of the LV to the aorta was 12.0 ± 12.8 vs 17.7 ± 26.0 mm Hg in groups A and B (P = 0.31). There were no differences between the groups regarding basal rhythms, anti-arrhythmic agents, and pacemaker implantation rate. VSD enlargement in the Rastelli procedure can be safely performed without early mortality and with long-term low mortality and morbidity regarding arrhythmia, LV function, and reoperation for late LVOTO. VSD enlargement should be considered as an option for avoiding long-term LVOTO.

Anatomic Repair for Corrected Transposition With Left Ventricular Outflow Tract Obstruction

We investigated the long-term outcomes of anatomic repair for congenitally corrected transposition of great arteries (ccTGA) and its variant associated with left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD). From 1987 to 2011, 47 patients (27 with pulmonary atresia and 20 with pulmonary stenosis) with ccTGA and its variant associated with LVOTO and VSD underwent anatomic repair. The mean operative age was 5.5 ± 3.7 years old (range, 1.6 to 21.3). The preoperative right ventricular end-diastolic volume was 133% ± 31% (81 to 222) of their normal size. The atrial switch procedure was Mustard in 31 patients and Senning in 16, with the latter used in all from 2002. The mean follow-up period was 11.6 ± 7.3 years (maximum, 22.7). The VSD was concomitantly enlarged in 4 patients and Damus-Kaye-Stansel (DKS) anastomosis was added in 9 patients with pulmonary stenosis and restrictive VSD. The overall survival rate at 20 years was 70.2% and no mortality has been observed in 21 consecutive patients since 1997. No patient required reoperation for the postoperative systemic ventricular outflow tract obstruction. A surgical heart block developed in 1 patient (2.1%) who underwent concomitant VSD enlargement. None of the patients developed a moderate or greater aortic or neo-aortic regurgitation. Recent outcomes after anatomic repair for congenitally corrected transposition of great arteries associated with LVOTO and VSD were excellent. For patients with pulmonary stenosis and restrictive VSD, additional DKS anastomosis seems to be an effective approach to avoid postoperative systemic ventricular outflow tract obstruction and surgical heart block.

Intermediate-term clinical outcomes of primary biventricular repair for left ventricular outflow tract obstruction and ventricular septal defect

Primary biventricular repair for left ventricular outflow tract obstruction and ventricular septal defect remains challenging. The intermediate-term outcomes and risk factors for mortality remain undefined. All patients undergoing primary biventricular repair of left ventricular outflow tract obstruction and ventricular septal defect from 1995 to 2008 at the C. S. Mott Children's Hospital, University of Michigan Health Systems were analyzed. Thirty-one patients (mean age, 18 days; 20 male) with a median follow-up of 6.7 years (range, 0.3-13.5 years) were identified. The ventricular septal defect was enlarged in 15 patients, and a limited atrial septal defect was constructed in 16 patients. There were 6 hospital and 2 late deaths. Ten-year patient survival was 72.3%. Lower body weight (P = .040), complete atrial septal defect closure (P = .026), and longer cardiopulmonary bypass time (P = .026) were risk factors of hospital mortality. An atrial septal defect was patent in 16 patients at discharge, 2 of whom required later surgical closure. Relief of recurrent left ventricular outflow tract obstruction was performed in 1 patient. No patient required pacemaker implantation. Five-year freedom from right ventricle-to-pulmonary artery conduit replacement was 39.3%. Smaller-sized conduit (P = .020) and use of aortic allograft (P = .048) were risk factors for early failure. Primary biventricular repair for patients with left ventricular outflow tract obstruction and ventricular septal defect provides good early and intermediate-term outcomes. Maintaining a small atrial septal defect may improve hospital mortality. Selective ventricular septal defect enlargement and careful construction of the intraventricular pathway result in a low incidence of recurrent left ventricular outflow tract obstruction, as well as avoidance of heart block. Maximizing valve diameter and avoiding aortic allografts may lengthen conduit longevity.

Double-Root Translocation for Double-Outlet Right Ventricle With Noncommitted Ventricular Septal Defect or Double-Outlet Right Ventricle With Subpulmonary Ventricular Septal Defect Associated With Pulmonary Stenosis: An Optimized Solution

Biventricular repair of double-outlet right ventricle (DORV) with noncommitted ventricular septal defect (VSD) or subpulmonary VSD, associated with pulmonary stenosis, remains controversial. The usual technique, Rastelli or réparation à l'étage ventriculaire (REV) procedure, may not meet a perfect biventricular outflow tract reconstruction in terms of hemodynamic performance and long-term outcome. Here we present an early result of an alternative solution for these anomalies by double-root translocation technique. Between August 2006 and August 2009, a total of 10 consecutive patients underwent a double-root translocation procedure, at a median age of 48 +/- 55 months (range, 1 to 16 years). The VSD was repaired with a Dacron patch, and VSD enlargement was done in 3 patients. The aortic translocation was done with (n = 4) or without (n = 6) coronary reimplantation. The neopulmonary artery was reconstructed with a monocusp bovine jugular vein patch (n = 8) or a homograft patch (n = 2). The mean follow-up interval was 21.9 +/- 11 months (range, 2 to 36). Biventricular outflow tract function was assessed by echocardiography. There were no early or late deaths, and no required reoperations. Two patients required early support by extracorporeal membrane oxygenation. Postoperative echocardiography showed satisfactory hemodynamic effect of the reconstructed biventricular outflow tract and ventricular function. One patient had trivial aortic regurgitation and 4 patients had trivial or mild pulmonary insufficiency in follow-up. The early results showed an optimized solution for DORV with noncommitted VSD or DORV with subpulmonary VSD, associated with pulmonary stenosis. Long-term benefits need to be evaluated with a larger number of patients and longer follow-up.

Early and Intermediate Outcome After Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries

Anatomic repair of congenitally corrected transposition of the great arteries has become a useful surgical strategy with potential advantages over conventional surgical repair. We describe early and intermediate outcomes after anatomic repair and analyze potential risk factors influencing these outcomes. A retrospective review was performed on all patients undergoing anatomic repair between January 1993 and January 2009. The primary outcome was in-hospital mortality. Variables potentially associated with outcome were identified a priori. Bivariate analyses were performed to determine the association between these variables and all outcome measures. In 65 patients who underwent anatomic repair, 35 had Senning/arterial switch and 30 had Senning/Rastelli. Early and intermediate survival rates for Senning/arterial switch operations were 94% and 91%, respectively. Repairs were successful in patients with tricuspid regurgitation, left ventricular outflow obstruction, and left ventricular dysfunction. Predictors of outcome were not identified in this subset. Early and intermediate survival rates for Senning/Rastelli operations were 77% and 60%, respectively. Longer aortic cross-clamp (p = 0.03) and cardiopulmonary bypass times (p = 0.01) were associated with mortality. Ventricular septal defect enlargement was associated with surgical heart block (p < 0.01). Age, prior procedures, atrial-apical discordance, and tricuspid regurgitation were not associated with outcome. Senning/arterial switch operations can be performed with excellent intermediate-term outcomes in patients with lesions previously thought to confer higher risk. Candidates for Senning/Rastelli procedures may be at increased risk for postoperative morbidity and mortality. More data are necessary to determine factors influencing outcome after anatomic repair.

The effect of ventricular septal defect enlargement on the outcome of Rastelli or Rastelli-type repair

Our purpose was to evaluate the effect of ventricular septal defect enlargement on the early and late morbidity and mortality of patients undergoing Rastelli or Rastelli-type operations. A total of 49 patients who underwent Rastelli or Rastelli-type operations between 1991 and 2007 were included in a retrospective follow-up study. Patients were divided into 2 groups: group A had ventricular septal defect enlargement, and group B did not have ventricular septal defect enlargement for comparison. Risk factor analysis for early or late death included patient-related and procedure-related variables, with failure, arrhythmia, and atrioventricular block as outcome parameters. Median age and weight at the time of the operation were 6 years (range, 3 months-22 years) and 17 kg (range, 7-48 kg), respectively. The ventricular septal defect was enlarged in 28 (57%) patients. Ventricular septal defect enlargement showed a significant statistical relation with late ventricular dysfunction, arrhythmia, and residual ventricular septal defect (P = .023, P = .047, and P = .01, respectively, log-rank test). No relation was found between ventricular septal defect enlargement and permanent pacemaker implantation (P = .73, log-rank test). Furthermore, enlargement of the ventricular septal defect did not show any significant effect on the rate of early mortality (P = .69, Cox regression). Kaplan-Meier estimated survival for patients with ventricular septal defect enlargement was 74% at 5 years and 65% at 10 years. Freedom from late death in the group without ventricular septal defect enlargement was 100% at 5 and 10 years and 83% at 15 years. At a median follow-up of 4 years (range, 6 months-16 years), there were 12 late-onset deaths: 11 in group A (n = 28) and 1 in group B (n = 21). Ventricular septal defect enlargement greatly increased the risk of late death (P = .009, Cox regression). Septal resection in patients undergoing Rastelli or Rastelli-type operations has a substantial effect on late morbidity and is a predictive factor for long-term mortality.

Management of Older Single Functioning Ventricles With Outlet Obstruction due to a Restricted “VSD” in Double Inlet Left Ventricle and in Complex Double Outlet Right Ventricle

The occurrence of a restriction of the bulbo-ventricular foramen (BVF) in older patient with double inlet left ventricle (DILV) or tricuspid atresia (TA) with ventriculo-arterial discordance is a well-known condition. Today, the surgical management is to perform a Damus-type operation at the time of the bi-directional Glenn or at the Fontan completion. The ventricular septal defect (VSD) enlargement, associated with muscular resection and a patch enlargement of the subaortic accessory ventricular chamber, is rarely performed but remains indicated in cases with pulmonary valve atresia or regurgitation. This condition is essentially prevented by doing an early Norwood-type operation in the presence of DILV/TA with transposition of the great arteries associated with an aortic arch obstruction. The palliative switch operation is an option that was abandoned because of poor control of the pulmonary blood flow. It is only in cases of large unobstructed BVF that pulmonary artery banding could be undertaken in neonates, followed by close echocardiographic follow-up. The occurrence of a restriction or a closure of the VSD in complex DORV following a Fontan operation is a dramatic event and is quite "new business." It has been recently recognized that the VSD becomes restricted in a number of patients with DORV-nc-VSD treated with a Fontan palliation. This new condition is not surprising knowing that 75% of the VSDs must be enlarged preventively in DORV-nc-VSD repair. In the setting of a Fontan circulation, the supra-systemic left ventricle has severe consequences the right ventricle performance. Attempts at surgical VSD enlargement or catheter-based procedures have resulted in almost constant recurrence. This recently reported complication is in favor of also performing a VSD enlargement at the time of the Fontan completion in complex DORV. It justifies the biventricular repair in complex DORV with two viable ventricles.

Corrected Transposition of the Great Arteries Involving Situs Inversus {I, D, D} and Mild Pulmonary Stenosis: Conduction System Identified During Preoperative Investigations for a Double-Switch Operation

The atrioventricular (AV) conduction system in corrected transposition of the great arteries (cTGA) involving situs inversus remains unclear. A case of cTGA involving situs inversus, mild pulmonary stenosis, and ventricular septal defect (VSD) is reported. An electrophysiologic study using the CARTO system and angiography was performed to identify the AV conduction system before a double-switch operation with VSD enlargement. The His bundle was located at the anteroseptum of the left-sided RA. It descended along the posteroinferior margin of the VSD with cTGA involving situs inversus and a normal-sized pulmonary trunk. No AV block occurred after surgery. The combination of the CARTO system and angiography could be useful for identifying the conduction system.

Long-Term Results After the Rastelli Repair for Transposition of the Great Arteries

This study sought to assess risk factors for late mortality after the Rastelli operation for patients with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction. Records of 39 patients who underwent the Rastelli operation between 1977 and 2004 were reviewed. Median age at the time of operation was 5.1 years (2.2 years within the last 5 years). There were no early deaths. During a median follow-up of 8.9 years (range, 0 to 25 years), 2 patients died of sudden death, 1 of pneumonia, 1 during reoperation, and 2 received heart transplantation. Freedom from death or transplantation was 93.8% +/- 4.3% and 57.5% +/- 15.1% at 10 and 20 years, respectively. Freedom from conduit replacement was 48.8% +/- 10.3% and 32.5% +/- 10.3% at 10 and 20 years, respectively. Subvalvular and valvular left ventricular outflow tract obstruction (p = 0.012), stenosis of the peripheral pulmonary arteries (p < 0.001), enlargement of the ventricular septal defect (p = 0.030), and longer ischemic time (p = 0.015) were predictive for death or transplantation. Patients younger than 4 years at the time of the Rastelli operation showed a trend toward a better freedom from death or transplantation (p = 0.068), but needed significantly more conduit replacements (p = 0.038) compared with patients 4 years or older. The Rastelli operation is a low-risk procedure with regard to early mortality. The status of the pulmonary arteries and ventricular septal defect enlargement are predictive for long-term survival. Patients 4 years of age or older at the time of the Rastelli operation require fewer reoperations for conduit exchange. Nevertheless, early Rastelli repair is recommended because patients 4 years or older are at risk for a higher long-term mortality.

Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections: Risk analyses in 189 patients

This study was undertaken to compare long-term results of various types of surgical repairs for either congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections, and to analyze the risk factors that affect early and late mortality and reintervention. Between January 1972 and September 2005, a total of 189 patients (median age 8.3 years, range 2 months to 47 years old) with congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections underwent definitive repairs. The definitive repairs comprised a conventional repair (atrial septal defect, or ventricular septal defect closure with or without pulmonary stenosis release, or isolated tricuspid valve surgery) in 36 patients (group I), conventional Rastelli in 31 patients (group II), double-switch operation (atrial switch plus arterial switch) in 15 patients (group III), atrial switch plus intraventricular rerouting (with or without extracardiac conduits) in 69 patients (group IV), and a Fontan-type repair in 38 patients (group V). The mean follow-up period was 10.1 years. Hospitalization and late mortality and reoperation were indicated as events. Risk factors for these events were analyzed by logistic regression for hospital death and a Cox proportional hazards model for late events. The Kaplan-Meier survival including hospital and late mortality was 62.4% at 32 years in group I, 78.5% at 27 years in group II, 74.5% at 15 years in group III, 80% at 16 years in group IV, and 79.3% at 22 years in group V. The reoperation-free ratio was 64.2% in group I, 76.6% in group II, 84.4% in group III, 89.6% in group IV, and 91.3% in group V. Risk analyses showed that the risk for hospital death was preoperative in patients with more than moderate tricuspid regurgitation and a cardiopulmonary bypass time of more than 240 minutes. A risk for late mortality was the presence of tricuspid regurgitation. Risks for reoperation were preoperative cardiomegaly, preoperative tricuspid regurgitation of more than grade II, ventricular septal defect enlargement, and body weight less than 10 kg. Risks for pacemaker implantation, as indicated by multivariate analysis, were ventricular septal defect enlargement during operation and age less than 3 years. There were no statistical differences between long-term survival rates of patients who underwent conventional surgical repair versus those of patients who underwent anatomic surgical repair. Results of conventional repair were satisfactory except in patients with significant tricuspid regurgitation. Results of anatomic repair were also satisfactory even for patients with significant tricuspid regurgitation, and therefore, anatomic repair should be the procedure of choice for those patients.

Transcatheter creation and enlargement of ventricular septal defects for relief of ventricular hypertension

Creation or enlargement of a ventricular septal defect is indicated in rare clinical situations. In the setting of double-outlet right ventricle requiring single-ventricle palliation, left ventricular outflow tract obstruction caused by progressive restriction at the ventricular septal defect poses an uncommon but recognized dilemma. In this situation surgical ventricular septal defect enlargement may be desirable but risks damage to the atrioventricular valve or conduction system. We report the results of a novel technique for transcatheter creation or enlargement of ventricular septal defects as an alternative to reoperation when decompression of an isolated ventricle is indicated. Eight patients had undergone transcatheter ventricular septal defect enlargement or creation, and 3 of these patients had undergone 4 prior surgical attempts at left ventricular decompression. Ventricular aneurysms had developed in 3 patients before intervention. Five patients underwent ventricular septal defect creation, and 3 patients underwent enlargement of existing ventricular septal defects. Initial intervention resulted in a decreased ventricular septal defect pressure gradient from 76.9 mm Hg to 20.3 mm Hg (P = .004). There was no procedural mortality or sustained heart block. Two patients had moderate-to-severe atrioventricular valve regurgitation, and 1 required surgical repair. At last follow-up, all ventricular septal defects remained patent, with recurrent obstruction in the majority of cases caused by muscular hypertrophy beyond the stent margins. In 1 patient a ventricular aneurysm has regressed in size. Repeat intervention reduced recurrent obstruction, but recurrence was the rule. When reoperation is considered high risk, transcatheter creation and enlargement of ventricular septal defects is possible with excellent short-term results. Recurrent obstruction is common but responds to repeated intervention. Further studies are required to establish clinical benefit.