The Norwood procedure - does the type of shunt determine outcome?

BACKGROUND: Stage I palliation of hypoplastic left heart syndrome (HLHS) and its variants is usually performed by a Norwood operation. The management of pulmonary blood flow during this procedure remains controversial. The RV-to-PA conduit (RVPAC) has been proposed as the better alternative compared to a systemic-to-pulmonary shunt (SPS). METHODS: A retrospective single center chart review of consecutive patients who underwent a Norwood I procedure between 01/1997 and 09/2006 was performed. All patients were operated in deep hypothermia, with or without circulatory arrest, using different shunt modifications according to surgeon's preference. Patients were divided into two groups depending on surgical management for pulmonary blood flow (modified BT shunt [BT] and non-valved RVPAC [Sano]). RESULTS: Fifty-four patients were included in the study (BT: 31 patients vs. Sano: 23 patients). Diastolic blood pressure during the first 24 hours postoperatively was significantly lower in the BT group (BT: 38.6 +/- 6.9 mmHg vs. Sano: 42.4 +/- 7.2 mmHg; P < 0.01) with a trend towards a higher systolic blood pressure (BT: 74.1 +/- 13.5 mmHg vs. Sano: 69.8 +/- 12.1 mmHg; P = 0.08). Mean circulatory arrest time in the BT group was significantly longer compared to the Sano patients (BT: 41 +/- 21 min vs. Sano: 25 +/- 23 min; P < 0.01). The mean hospital stay was 18.5 days for BT patients and 20 days for Sano patients ( P = 0.45). Early mortality for the total cohort was 14.8 % (n = 8) (BT 19.4 % [n = 6] vs. Sano 8.7 % [n = 2]; P = 0.12). There was no significant difference in inter-stage mortality between the two groups (BT: 18.2 % vs. Sano: 21.1 %; P = 0.47). CONCLUSION: The results for both established surgical methods (BT and Sano) for the palliation of HLHS and its variants have improved over time and are reaching acceptable early mortality rates. There was a trend towards a favorable early outcome for Sano patients, which did not reach statistical significance in this study due to the low patient numbers.

Intracardiac repair of double outlet right ventricle.

Intracardiac repair of double outlet right ventricle (DORV) remains controversial. DORV is a particular mode of ventriculo-arterial connection and not a specific congenital heart disease. It can exhibit a wide spectrum of anatomic and physiologic variations. This heterogeneity has naturally led to controversies over the anatomical definition, classification schemes, and the techniques for surgical repair. From a surgical standpoint, the functional classification that was adopted together by the Society of Thoracic Surgeons (STS), The European Association of Cardio-Thoracic Surgery (EACTS) and the Association of the European Pediatric Cardiologists (AEPC), provides useful information to understand the anatomical variations and the choice of the surgical technique. The lesions that remain a surgical challenge are those where "200% of the great vessels" arise from the right ventricle. Namely: DORV-nc-VSD and DORV-AVSD-heterotaxy. Both of these lesions have traditionally been indications for single-ventricle palliation. In our series, there was one death in 15 of these challenging patients (6.7% mortality) following bi-ventricular repair. Two patients required a one and a half repair. Long-term results of biventricular repair of complex DORV are not yet available. These data would be crucial to validate the intracardiac repair technique."

[Surgical repair of atrial septal defect in 1011 consecutive patients]. / Traitement chirurgical des communications interauriculaires chez 1011 patients consécutifs. Incidence des complications postopératoires précoces.

The immediate postoperative complications of 1011 consecutive patients undergoing surgical repair of atrial septal defects between 1980 and 1998 at Marie Lannelongue Hospital were analysedwith the ultimate objective of comparing their incidence with that of percutaneous closure. Five patients died (0.49%) of low cardiac output (N=3), pulmonary oedema (N=1) or pulmonary hypertension (N=1). A total of 356 patients (35.2%) had 448 postoperative complications. There were 77% minor and 23% major complications. The minor complications included arrhythmias and conduction defects (N=130), respiratory complications (N=90) and pericardial effusions (N=64). The main major complications were cardiac failure (N=27), cardiac tamponade (N=13), neurological complications (N=8) and reoperation (N=28). Of the survivors, 95.6% of patients were discharged from hospital with no residual problem and 41 (4.4%) had sequellae: arrhythmias (N=29, including one pacemaker implantation), neurological complications (N=4), acquired mitral regurgitation (N=2), phrenic nerve paralysis (N=1) and minimal residual shunt (N=10). Factors correlated with surgical morbidity were age, the severity of pulmonary hypertension, the type of atrial septal defect (less morbidity with ostium secundum defects), the presence of associated malformations, the surgical approach (less morbidity with the right postero-lateral thoracic approach). This study demonstrated the incompressible risk of open heart cardiac surgery. It will form a basis for a comparative study of the two modern methods of treating atrial septal defect: surgical and percutaneous closure.

Additional pulmonary blood flow has no adverse effect on outcome after TCPC.

BACKGROUND: Use of additional sources of pulmonary blood flow (APBF) with bidirectional cavopulmonary anastomosis (BDG) and total cavopulmonary connection (TCPC) remains controversial. We have therefore assessed the effects of APBF on BDG and TCPC outcome. METHODS: From 1996 to 2000, 106 patients underwent BDG, either isolated (group 1, n = 54), or with APBF via pulmonary artery (PA) (group 2, n = 30), or Blalock-Taussig shunt (BTS) (group 3, n = 22) with 28 patients completed by TCPC. RESULTS: After BDG low output syndrome was more frequent in group 2 and less in 3 ( p = 0.01), whereas superior cava syndrome was again more common in group 2 and less in groups 1 and 3 ( p < 0.05) than expected. Mortality and complications were similar after TCPC in all groups. Oxygen saturation (SaO (2)) was lower without than with APBF ( p < 0.002) after BDG and in group 3 than in group 2 after TCPC ( p < 0.05). Repeated measures ANOVA showed no effect of APBF on SaO (2), PA pressures, fractional shortening, end-diastolic pressure and AV-valve function. CONCLUSIONS: Early after BDG, APBF via PA presents different difficulties than APBF via BTS. However, BDG and TCPC outcomes are not affected by the presence or absence of APBF.

The Aristotle score: a complexity-adjusted method to evaluate surgical results.

OBJECTIVES: Quality control is difficult to achieve in Congenital Heart Surgery (CHS) because of the diversity of the procedures. It is particularly needed, considering the potential adverse outcomes associated with complex cases. The aim of this project was to develop a new method based on the complexity of the procedures. METHODS: The Aristotle project, involving a panel of expert surgeons, started in 1999 and included 50 pediatric surgeons from 23 countries, representing the EACTS, STS, ECHSA and CHSS. The complexity was based on the procedures as defined by the STS/EACTS International Nomenclature and was undertaken in two steps: the first step was establishing the Basic Score, which adjusts only the complexity of the procedures. It is based on three factors: the potential for mortality, the potential for morbidity and the anticipated technical difficulty. A questionnaire was completed by the 50 centers. The second step was the development of the Comprehensive Aristotle Score, which further adjusts the complexity according to the specific patient characteristics. It includes two categories of complexity factors, the procedure dependent and independent factors. After considering the relationship between complexity and performance, the Aristotle Committee is proposing that: Performance = Complexity x Outcome. RESULTS: The Aristotle score, allows precise scoring of the complexity for 145 CHS procedures. One interesting notion coming out of this study is that complexity is a constant value for a given patient regardless of the center where he is operated. The Aristotle complexity score was further applied to 26 centers reporting to the EACTS congenital database. A new display of centers is presented based on the comparison of hospital survival to complexity and to our proposed definition of performance. CONCLUSION: A complexity-adjusted method named the Aristotle Score, based on the complexity of the surgical procedures has been developed by an international group of experts. The Aristotle score, electronically available, was introduced in the EACTS and STS databases. A validation process evaluating its predictive value is being developed.

Ross and Ross-Konno procedure in children and adolescents: mid-term results.

OBJECTIVES: The aim of the study was to analyze mid-term results of aortic root replacement with pulmonary autograft in children and adolescents in two centers. METHODS: From December 1997 through August 2003, a total of 66 patients underwent the Ross procedure in two centers. Indication for Ross procedure was predominantly aortic stenosis in 24 patients and predominantly aortic regurgitation (AR) in 22 patients. Twenty patients with severe left ventricular outflow tract obstruction (LVOTO) underwent Ross-Konno procedure. No patient had a geometric mismatch of more than 5 mm in favor of the aortic annulus. RESULTS: There was no early death. One patient died 3 months after surgery due to bacterial endocarditis. Survival on median follow-up period of 2.4 years was 98.5%. Neo-aortic regurgitation was none in 29 (44%) patients, trivial in 35 (53%) patients and mild in 2 (3%) patients. One patient (1.5%) needed aortic valve replacement because of autograft failure. Actuarial freedom from more than trivial neo-aortic regurgitation, or aortic valve replacement was 95% at 5 years follow-up. There was no patient either with recurrent LVOTO or significant aortic root dilatation. Freedom from redo was 93% at 5 years of follow-up. There had been a significant reduction (P = 0.001) and normalization in the left ventricle diastolic diameter index and left ventricle mass index, respectively, within 3-12 months after operation. Sixty-three percent of all operated patients are without medication; no one is on anticoagulation therapy. CONCLUSIONS: Our 7 years experience with the Ross and Ross-Konno operation has shown excellent mid-term results, with mortality rate approaching zero in both simple and complex left heart lesions, even in the neonates and infants. It is a procedure of choice in children with severe anomaly of the aortic valve and/or left ventricular outflow tract obstruction. The main concern is dilatation of the neo-aortic root leading to progression of AR, especially in the settings of geometric mismatch of aortic and pulmonary roots and bicuspid, regurgitant aortic valve. The risk of autograft failure in these specific subsets of patients remains to be determined.

Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch.

OBJECTIVES: Biventricular repair of double outlet right ventricle non-committed ventricular septal defect (DORVncVSD) is usually achieved by a VSD rerouting to the aorta. This technique can be limited by the presence of tricuspid chordae and by the pulmonary artery to tricuspid valve distance. Furthermore, there is an important risk of late subaortic obstruction related to the long patch required that creates a potential akinetic septal area. Presented here is another technique; by VSD rerouting to the pulmonary infundibulum and arterial switch. METHODS: Ten patients, with DORVncVSD, underwent a VSD rerouting to the pulmonary infundibulum followed by arterial switch. Seven had a previous pulmonary artery banding and one a moderate infundibular stenosis. The median age at surgery was 16 months (range 3 weeks to 4.5 years). All patients had a bilateral infundibulum, with a large persistent subaortic conus, D malposition of the aorta, side-by-side vessels and double loop coronary patterns. The VSD was perimembranous with inlet or trabecular extension. Subaortic obstruction was constant. The VSD was severely distant from both the aortic and the pulmonary annulus. The operation was conducted through a combined approach. The VSD was constantly enlarged superiorly. The almost permanent subaortic obstruction was released. The VSD was always found quite close to the pulmonary infundibular ostium. The arterial switch technique was adapted to the complex coronary anatomy. RESULTS: There was one non-cardiac death. At a mean follow-up of 20 months, all nine survivors are in NYHA class I, in sinus rhythm, and have no subaortic gradient greater than 15 mm. CONCLUSION: This technique of VSD rerouting to the pulmonary artery and arterial switch limits greatly the size of the rerouting patch, respects the tricuspid chordae and is independent of the pulmonary artery-tricuspid valve distance. In this early series of biventricular repair of DORVncVSD, the VSDs were always found close to the pulmonary artery, allowing this new type of repair.

[Right atrial compressive aneurysm with favorable outcome after surgery at the age of 1 month]. / Anévrisme compressif de l'oreillette droite d'évolution favorable après traitement chirurgical à 1 mois de vie.

Right atrial aneurysms are uncommon and often revealed by arrhythmia. We report a case of right atrial aneurysm which required an early management and its follow up during one year. The abnormality was detected at 32 weeks gravida during a systematic echography. At birth the newborn was asymptomatic but with the progressive increase of the aneurysm size shifting the heart and vessels and the occurrence of an intra-cardiac thrombus, a surgical management was decided at the age of one month. The resection of the aneurysm was therefore performed and no complication occurred during recovery. After one year the child remains healthy. The surgical indications are difficult to precise as the majority of these aneurysms are detected at an adult age. Surgical management appears to be essential in case of poor tolerance. The prognosis after surgery seems favorable.

Biventricular repair of transposition of the great arteries and unbalanced ventricles.

BACKGROUND: It is well established that the arterial switch operation is the surgical procedure of choice in patients with transposition of the great arteries and balanced ventricular anatomy. The surgical approach of choice in patients with transposition but unbalanced ventricular size is unknown. OBJECTIVES: Since the beginning of the arterial switch operation program, patients with transposition of the great arteries and unbalanced ventricles underwent biventricular repair by means of the arterial switch operation and repair of any associated lesions, either through a single or staged surgical procedure. The aim of this retrospective study is to analyze whether this approach can be proposed to such patients. METHODS: Forty-four patients with transposition of the great arteries and unbalanced ventricles underwent this surgical approach since 1984. Two groups were defined: group I had transposition with a dominant right ventricle (n = 28), and group II had transposition with a dominant left ventricle (n = 16). In group I the median age and weight at the arterial switch operation were 8.5 days (range, 5-70 days) and 3.1 kg (range, 1.5-3.7 kg), respectively. The median end-diastolic left ventricular volume, mass, and long-axis ratio were 15 mL/m2 (range, 11-16 mL/m2), 31.5 g/m2 (range, 20-66 g/m2), and 0.85 (range, 0.9-0.7), respectively. The mitral valve diameter was slightly hypoplastic, with a median z value of -1.22 (range, -0.3 to 3.7). In group 2 the median age and weight at the arterial switch operation were 42 days (range, 8 days-15 years) and 3.5 kg (range, 2.8-35 kg), respectively. Associated lesions in this group were coarctation in 9 and single (n = 12) or multiple (n = 4) ventricular septal defects. The median long-axis ratio and tricuspid z value were 0.6 (range, 0.3-0.8) and -0.9 (range, -0.5 to 3.3), respectively. In this group 9 patients had a single-stage procedure with fenestrated ventricular defect patches, atrial septal defect patches, or both; 7 patients underwent the staged approach. RESULTS: In group I there was 1 early death from sepsis after weaning from postoperative extracorporeal membrane oxygenation. Three patients had severe pulmonary hypertension, one of whom died 1 year later. All survivors demonstrated, at discharge from the hospital, equilibrated ventricular size, with a median left ventricular end-diastolic volume of 25 mL/m2 (range, 21-30 mL/m2). In group II there were 2 early and 1 late deaths. All early deaths occurred in patients without voluntary residual intracardiac shunts. Median early postoperative long-axis ratio and tricuspid z value were 0.8 (range, 0.7-1) and -0.2 (range, 0.74 to 1.2), respectively. CONCLUSION: This study demonstrates that the arterial switch operation in patients with transposition of the great arteries and unbalanced ventricles remains a good surgical option.

Young Investigator's Prizewinner 2001. Direct visualization of the influence of normothermic as opposed to hypothermic cardiopulmonary bypass on the systemic microcirculation in neonatal piglets.

The direct visualization of systemic microcirculation using intravitalmicroscopy permits the classification of proinflammatory and ischemic microvascular alterations during normothermic and hypothermic cardiopulmonary bypass in neonates. We used seven newborn piglets, on average aged 9 days, and weighing 3200g, as a control group. In addition, we studied nine piglets subjected to 60 minutes of constant nonpulsatile flow using hypothermic extracorporeal circulation at 28 degrees C, and five piglets using normothermic conditions at 37 degrees C. The microvascular network of the greater omentum and the subcutaneous tissue was directly visualized using intravitalmicroscopy. We analysed interactions between leukocytes and endothelial cells, microvascular morphology, and microrheological conditions, focussing on signs of ischemic and proinflammatory alterations. During normothermic cardiopulmonary bypass, the numbers of activated leukocytes were elevated compared to hypothermic cardiopulmonary bypass (p > 0.05). Arteriolar diameter decreased during hypothermia. Capillaries were markedly dilated during normothermia. Patterns of microvascular perfusion, for both types of cardiopulmonary bypass, showed signs of ischemic damage, revealed by a reduced functional capillary density. Perfusion dependent levels of lactate were higher during normothermic cardiopulmonary bypass (p > 0.05). This new experimental approach revealed that non-pulsatile cardiopulmonary bypass, independent of temperature, induces a proinflammatory and ischemic response compared to an unaltered control group. The markedly elevated numbers of activated adherent leukocytes, the reduced capillary density, and the high lactate levels in those undergoing bypass in normothermic conditions indicate a more pronounced inflammatory stimulus and tissue hypoperfusion compared to the possible protective effect of hypothermia for children undergoing cardiopulmonary bypass.

Late outcome after arterial switch operation for transposition of the great arteries.

BACKGROUND: Early and midterm results of the arterial switch operation (ASO) in transposition of the great arteries (TGA) are good, but late outcome data in large populations are still few. METHODS AND RESULTS: Twelve hundred patients had an ASO for TGA between 1982 and 1999, with prospective follow-up of 1095 survivors. Outcome measures included late death, reoperation, aortic insufficiency (AI), pulmonary stenosis (PS), and coronary anomaly. Median follow-up was 4.9 years (range 0.5 to 17 years). Late death occurred in 32 patients; survival was 88% at both 10 and 15 years. The hazard function for death declined rapidly, with no deaths after 5 years. Late mortality was correlated with reintervention and major events in the intensive care unit. Reoperation was performed in 103 patients, more often in complex TGA; the cause was mainly PS. Freedom from reintervention was 82% at 10 and 15 years, with a hazard function that declined rapidly but slowly increased after 3 years. At the last follow-up, PS was present in 3.9% of patients, and grade II or more AI was present in 3.2%, with a cumulative incidence of 9% at 15 years. Among the 278 patients who had a coronary arteriography, 8% had coronary lesions. Normal left ventricle and sinus rhythm were seen in 96.4% and 98.1%, respectively. CONCLUSIONS: Fifteen years after ASO, late mortality was low, with no deaths after 5 years; reoperation, mainly owing to PS, occurred throughout the follow-up. AI and coronary obstruction are rare but warrant further follow-up. Good left ventricular function and sinus rhythm are maintained.

Surgical management and indication of left ventricular retraining in arterial switch for transposition of the great arteries with intact ventricular septum.

OBJECTIVE: Arterial switch is the operation of reference for the surgical treatment of transposition of the great arteries. In cases of late referral, perinatal complications or early left ventricular (LV) dysfunction, the one stage arterial switch is contra indicated. Anatomical repair remains possible in these patients following a LV retraining. METHODS: From January 1992 to January 2000, a LV retraining was attempted in 22 patients with transposition of the great arteries with intact ventricular septum (TGA IVS), whereas 470 direct arterial switch and 2 Senning were performed. Indication for LV retraining was based on a combination of factors including: an age older than 3 weeks, a "banana shape" aspect of the inter-ventricular septum and mainly a LV mass <35G/m(2). RESULTS: The mean age at LV retraining was 3.2 months ranging from 9 days to 8 months. Usually conducted by sterntomy, it associated a loose PA banding with a LV/RV at 65% with a systemico-pulmonary shunt. The first stage was associated with frequent LV dysfunction and the LV retraining was discontinued in two patients in favor of one Senning and one early switch followed by ECMO. One patient died at first stage from a mediastinitis. Nineteen patients underwent a second stage arterial switch that was performed when the LV mass had reached 50 G/m(2) after a mean delay of 10 days, ranging from 5 days to 6 weeks. After a mean follow up of 25 months, there was one non-cardiac late death. The 17 patients followed and leaving with an arterial switch are in NYHA class I, with a mean LV shortening fraction of 39%. CONCLUSIONS: Arterial switch following LV retraining in TGA IVS is a satisfactory option. The inferior limit of 35 G/m(2) adopted, to indicate LV retraining, seems a safe landmark. The quality of the myocardium generated and the respective roles played by the LV afterload, LV wall shear stress, LV inflow and outflow to induce the LV remodeling remain under debate.

Long-term results after valvotomy for congenital aortic valvar stenosis in children.

As interest increase in the Ross procedure performed as a therapeutic option for children with congenital aortic valvar stenosis, it becomes increasinly important to know the late results of aortic valvotomy in this population. We have therefore examined retrospectively the medical records of 121 consecutives survivors undergoing aortic valvotomy before 10 years of age between 1974 and 1992. The mean age at the first valvotomy was 29 months, with a range from 3 days to 10 years. The mean duration of follow up was 9.4 years, with a range from 1.6 to 22 years. Fifteen patients (12.3%; 70% CL: 10-16) died: 9 following reoperation, and 6 late after surgery. Death was related to the hearts in 86% of cases. The actuarial survival rate was 79% (70% CL: 72/84) at 10 years. Young age at the first valvotomy, and the number of procedures, emerged as risk factors of secondary mortality. Reoperations on the aortic valve, 73 in all, were required in 56 patients. The second procedure was done after a mean interval of 6 years, with a range from 1 day to 18 years. This was for restenosis in three-quarters of the cases. The aortic valve was replaced in 30 patients, at a mean of 9 years, and with a range from 9 months to 18 years, after the first procedure. The survival without replacement at 20 years was 29% (70% CL: 15-49). No factor was identified with a relationship either to reoperation or valvar replacement. Long term results after aortic valvotomy, therefore, show a high late mortality, frequent reinterventions, and an almost inescapable eventual need for valvar replacement. The ongoing use of the Ross operation is justified, even if longterm studies in children are still needed to validate its use.

Congenital mitral stenosis with or without associated defects: An evolving surgical strategy.

BACKGROUND: Congenital mitral stenosis (CMS) remains a surgical challenge, particularly when it is associated with other heart defects. As in other groups of heart defects, there is a trend toward early single-stage complete repair, but the optimal surgical approach remains unanswered. METHODS AND RESULTS: This study was designed to analyze the evolution of surgical strategies in patients with CMS and associated defects through single-stage and staged repair. Between 1980 and 1999, 72 children were operated on for congenital heart defects, including CMS. Preoperative transmitral gradient was 12.6+/-7 mm Hg. Preoperatively, all the patients were NYHA class III to IV. Thirteen patients had an isolated CMS; in 59, it was associated with other heart defects, mainly ventricular septal defect (n=28) or multilevel left ventricular obstruction (n=41). In this group of patients, 33 had a staged approach, and 26 had a single-stage approach. Early mortality was 12.5% (9 patients). There were no deaths in the isolated CMS and single-stage repair groups. Logistic regression revealed that early mortality was influenced by association with left ventricular outflow tract obstruction (P:<0.001) and by use of a staged approach (P:<0.01). There was no late mortality in isolated CMS; there were 2 late deaths in the group of single-stage repair and 6 late deaths in the staged approach group (P:<0.01). Reoperation was required in 24 patients, mainly for residual mitral valve dysfunction or residual left ventricular outflow tract obstruction. Including the reoperations, 10 patients received a prosthetic mitral valve. At 15 years after surgery, survival was 69.6+/-7.5%, freedom from reoperation was 70.8+/-6.3%, and freedom from mitral valve replacement was 69+/-6%. CONCLUSIONS: Surgery for isolated CMS gives excellent early and long-term results. In patients with associated heart defects, a single-stage operation seems superior to a staged approach. Mitral valve replacement in this category of patients should be reserved as a salvage procedure.

Presentation of the International Nomenclature for Congenital Heart Surgery. The long way from nomenclature to collection of validated data at the EACTS.

An International Nomenclature for Congenital Heart Surgery was officially adopted at the Annual Meeting of the EACTS in Glasgow, UK on September 6, 1999. This nomenclature was achieved following 1 year's work of the International Nomenclature and Data Base Committee for Congenital Heart Surgery of the Society of Thoracic Surgeons. This international group included members from the STS, AATS, AHA and EACTS and associated surgeons and cardiologists from United States, Canada, Australia and Europe. The Nomenclature includes a minimal data set of 21 items and lists of 150 diagnoses, 200 procedures, 32 complications and 28 extra cardiac anomalies and preoperative risk factors. It will serve as a basis for the Pediatric European Cardiac Surgical Registry (http://www.pediatric. ecsur.org). The outcome of such an International Nomenclature represents an important event for the medical community in charge of treating patients with congenital heart diseases. It will allow scientific exchanges on an international scale and promote multicenter evaluation of congenital heart surgery. Nevertheless, this Nomenclature is only the first step. Further collection of validated data at the Pediatric ECSUR Data Base requires ethical belief, time consumption and financial resources. Comparison of results, according to pathologies, across centers and countries will help define, in the future, official European standards of Quality of Care available for health care organizations, public scrutiny and governmental agencies.

Transaortic closure of residual intramural ventricular septal defect.

BACKGROUND: Residual intramural ventricular septal defect is an unusual cause of left-to-right shunt after biventricular repair of conotruncal anomalies. It results from the insertion of the patch within the trabeculated right ventricular free wall related to the ventriculoinfundibular fold creating a communication through the intertrabeculated spaces to the right ventricular cavity. This complication often leads to unsuccessful reoperations unless the exact mechanism of the shunt has been identified. METHODS AND RESULTS: Five patients presented with residual intramural ventricular septal defects. Three had double outlet right ventricle, one pulmonary atresia with ventricular septal defect, and one tetralogy of Fallot. One patient was unsuccessfully reoperated on for closure of the residual ventricular septal defect through the right ventricular approach. The surgical treatment, which consisted of patch closure of the residual intramural ventricular septal defect through aortotomy, was successful in 3 patients. In the 2 remaining patients the hemodynamically insignificant residual intramural ventricular septal defect remained untouched. No mortality or morbidity occurred. CONCLUSIONS: Residual intramural ventricular septal defect should be suspected in presence of a residual ventricular septal defect after biventricular repair of conotruncal anomalies. It is not accessible through either atriotomy or right ventriculotomy. The transaortic approach allows an easy treatment of this rare complication.