Repair Of Atrioventricular Septal Defect Research Articles

Results of one-and-a-half-patch technique for repair of complete atrioventricular septal defect with a large ventricular component.

Management of complete atrioventricular (AV) septal defect (CAVSD) with a large ventricular component (>1 cm) remains controversial. The purpose of this study was to assess the feasibility of the one-and-a-half patch technique in repairing this lesion. This was a retrospective review of patients undergoing surgical repair of CAVSD with a large ventricular component (>1 cm). Of the 51 patients who were identified in our database (2005-13), 18 underwent the two-patch repair, 12 underwent the modified single-patch repair and 21 underwent the one-and-a-half-patch repair. The CAVSD was combined with tetralogy of Fallot (TOF) in 3 patients and with double-outlet right ventricle (DORV) and pulmonary stenosis (PS) in two individuals in the one-and-a-half-patch group. There were two hospital deaths in the two-patch group, but no deaths in the other two groups. The modified single-patch procedure was associated with the shortest myocardial ischaemic and cardiopulmonary bypass (CPB) times, the two-patch procedure was associated with the longest times and the 1.5-patch procedure times were intermediate. Median follow-up was 35 months (41.6 ± 27.2 months). There were no reoperations in the 1.5-patch group while reintervention was required for 1 patient in the two-patch group (P = 0.252) and 3 patients in the modified single-patch group (P = 0.017). The function of the reconstituted AV valves improved after operation in the 1.5-patch group. No patient in these three groups developed subsequent left ventricular outflow tract obstruction. The 1.5-patch technique is an attractive clinical option. It produces acceptable mid-term results comparable with two conventional techniques in patients with similarly sized ventricular component.

Should Repair of Partial Atrioventricular Septal Defect Be Delayed Until Later in Childhood?

Surgical repair of partial atrioventricular septal defects (AVSD) has been successful for more than 60years. However, recent data from the Pediatric Heart Network show that 31% of patients have moderate or severe left atrioventricular valve regurgitation (LAVVR) at follow-up. Previously, our institution found that only 9% of patients had more than moderate LAVVR at the last follow-up. Our objective was to determine the long-term outcomes after repair of partial AVSD in the current era. We reviewed all patients with partial AVSD who had primary biventricular repair from January 1995 to June 2011 at our institution. The Kaplan-Meier method was used to estimate the survival free of an event, and factors were evaluated for an association with each outcome using the log-rank test. All 105 patients with partial AVSD who had surgery during this period were evaluated. The median age at surgery was 7.9years. The overall survival rate at 1 year was 97%. Median follow-up was 5.3years (interquartile range 1.7 to 11.1). At 3years, the survival rate free from reoperation was 89%. Thirteen patients required reoperations with the most common reason being LAVVR. A total of 10 patients developed more than moderate LAVVR with a cumulative incidence of 8% by 2years. The discrepancy with the Pediatric Heart Network data may be due to the later age of operation for patients in our cohort suggesting that elective repair of partial AVSD should be deferred until children are somewhat older (ages 5 to 8years). Neither patient age (p=0.11) nor severity of preoperative LAVVR (p= 0.16) were identified as statistically significant risk factors. In conclusion, there is less morbidity and mortality after surgical repair for partial AVSD.

Post-operative left atrioventricular valve function after the staged repair of complete atrioventricular septal defect with tetralogy of Fallot.

This study reviewed surgical outcomes of staged repair for complete atrioventricular septal defect with tetralogy of Fallot, especially with focusing on the post-operative left-sided atrioventricular valve function. Between 1992 and 2013, 10 patients with complete atrioventricular septal defect with tetralogy of Fallot underwent total correction by the following surgical strategy. Systemic-to-pulmonary shunt was placed at first at the mean age of 1.5 ± 1.3 months. Then confirming sufficient development of the left heart structures, the total correction was performed at the mean age of 1.4 ± 0.6 years. Second shunt was required in 4 (40 %) patients to develop the left heart structures. The left ventricular end-diastolic volume before total correction was 127 ± 30 % of normal size. The two-patch repair was applied in 8 (80 %) patients. There was no mortality, and 1 reoperation case for left-sided atrioventricular valve regurgitation. The follow-up was completed on all patients and the mean follow-up period was 7.4 ± 7.0 years. The post-operative left-sided atrioventricular valve regurgitation kept less than moderate for 10-year follow-up in all patients except one patient who required the repair of left-sided atrioventricular valve 1 year after the total correction. The post-operative left-sided atrioventricular valve function after the repair of complete atrioventricular septal defect with tetralogy of Fallot maintained with the application of the two-patch repair, early and repeated palliative systemic-to-pulmonary shunt, and the early definitive surgery.

Successful slide tracheoplasty and partial atrioventricular septal defect repair following extracorporeal membrane oxygenation support

A two-year-old boy with a background history of Down syndrome and partial atrioventricular septal defect presented with acute respiratory distress requiring intubation and mechanical ventilation. He continued to deteriorate, despite ventilation; direct laryngoscopy, bronchoscopy, and computed tomography demonstrated severe long segment tracheal stenosis. He was placed on extracorporeal membrane oxygenation to stabilise his condition. A slide tracheoplasty and complete repair of the partial atrioventricular septal defect was successfully undertaken. His post-operative recovery was complicated by myocardial infarction and stroke but he made a full recovery. This represents the first report of slide tracheoplasty and partial atrioventricular septal defect repair in a child following extracorporeal membrane oxygenation support.

EARLY OUTCOME AFTER AORTIC VALVE REPLACEMENT ON PATIENTS WITH REDUCED LEFT VENTRICULAR EJECTION FRACTION

Background: To evaluate the early results of operation for partial atrioventricular septal defect. Methods: Twenty-sevent patients underwent surgical correction of partial atrioventricular septal defect from 1/2011 to 12/2013 at Cardiovascular Centre of Hue Central Hospital. There were 7 (25.9%) female patients and 20 (74.1%) male patients, 18.5% of patients aged < 1 age, 55.6% of patients aged ≥ 1 to 15 years, and 25.9% of patients aged ≥ 16 to 60 years. Sevent (25.9%) had congestive heart failure. There was a primum atrial septal defect in 100% of patients. A cleft of the anterior mitral leaflet was diagnosed in 100% of patients. 92.6% of patients had either moderate or severe mitral incompetence prior to operation. The pulmonary artery systolic pressure exceeded 40 mmHg in 85,.2% of patients. Results: Atrial septal defects were closed with a pericardial patch in 100% of patients. The cleft in its anterior leaflet was closed in 100% of patients. Postoperatively, moderate mitral insufficiency developed in 14.8% of patients. 85.2% of patients have mild mitral incompetence. One patients (3.7%) needed a permanent pacemaker. There was no intraoperative mortality. At 6-9 months postoperatively, left atrioventricular valve insufficiency was moderate in 2 (7.4%) patients and mild in 25 (92.6%) patients who had had cleft closure alone. Conclusions: Repair of partial atrioventricular septal defect is safe and good. It is important to close the cleft in the left atrioventricular valve. The mitral valve should be repaired in a conservative manner. Intraoperative complications occur but are uncommon, suggesting that short-term follow is excellent.

Experience with the surgical treatment of atrioventricular septal defect with left ventricular outflow tract obstruction

We sought to determine the prevalence, morphology, surgical methods and results of surgery for left ventricular outflow tract obstruction (LVOTO) associated with atrioventricular septal defect (AVSD). Correction of AVSD was performed in 615 patients. Twenty-three (3.7%) patients with LVOTO were identified. Sixteen (70%) of them had partial and 7 (30%) had complete AVSD. Surgery for AVSD was performed at a median of 0.6 years (mean 2.1 ± 3.0 years), and surgery for LVOTO at a median of 3.4 years (mean 4.7 ± 3.5 years). The point and period prevalence of LVOTO in AVSD were determined. Detailed morphological study, individualized repair of AVSD with LVOTO and long-term follow-up were performed. Early and long-term results were analysed. The point prevalence of LVOTO at the time of AVSD repair was 1.3%. The period prevalence of LVOTO was 3.7% in course of 8.3 ± 6.0 (0-18.4) years and 191.4 patient-years following AVSD repair. Causes of LVOTO were fibromuscular membrane (n = 17), septal hypertrophy (n = 17), abnormal atrioventricular (AV) valve (n = 9), muscular bands (n = 3), fibrous strands (n = 4) and stenotic aortic valve (n = 2). Usually, a combination of several obstructive lesions was present. LVOTO was present at the time of AVSD repair in 8 patients (35%) and developed after repair in 15 (65%) patients. Membrane excision (n = 17), myectomy (n = 17), excision of abnormal AV valvar tissue (n = 8), excision of muscular bands and fibrous strands (n = 6), AV valve replacement (n = 2) and aortic valvotomy (n = 2) were required. There was 1 (4%) early and 1 (4%) late death. Six (29%) survivors required reoperation for recurrence of LVOTO at an average interval of 6.3 ± 3.2 years after surgery. The actuarial survival at 1 and 10 years was 96 and 88%, respectively. The actuarial freedom from reoperation for LVOTO was 80, 40 and 20% at 6, 10 and 15 years after surgery, respectively. Eighteen (78%) patients remain in good condition at mean 6.0 ± 5.5 years after surgery. The point prevalence of LVOTO at the time of AVSD repair was 1.3%, and period prevalence 3.7%. Fibromuscular membrane, septal hypertrophy and valvar attachments represent the most common causes of LVOTO. Usually, more structures are involved. The repair must be individualized. The presence of LVOTO increases the need for reoperation.

Subaortic stenosis after repair of complete atrioventricular septal defect: Anatomic and technical issues

Subaortic stenosis after repair of complete atrioventricular septal defect: Anatomic and technical issues

Reoperation for left ventricular outflow tract obstruction after repair of atrioventricular septal.

Left ventricular outflow tract obstruction (LVOTO) is an important source of morbidity and mortality after repair of atrioventricular septal defect (AVSD). The intrinsic anatomy of the left ventricular outflow tract in AVSD is complex and predisposes to the development of LVOTO. LVOTO after repair of AVSD usually involves multiple levels and sources of obstruction, and surgical intervention must address each component of the obstruction. This includes fibromuscular obstruction, septal hypertrophy, and valve related sources of obstruction. Special attention is also directed to the anterolateral muscle bundle of the left ventricle, a well defined but under recognized feature of the left ventricular outflow tract in AVSD. It is present in all patients with AVSD, and resection of a hypertrophic anterolateral muscle bundle of the left ventricle should be incorporated in all operations for LVOTO after repair of AVSD. LVOTO after repair of AVSD has several unique features that must be taken into consideration to maximize outcome after surgical intervention. These include anatomic factors, technical aspects of surgical intervention, and proper selection of the operation used for relief of LVOTO.

Early Postoperative Outcomes Following Surgical Repair of Complete Atrioventricular Septal Defects

To evaluate the impact of Down syndrome on the early postoperative outcomes of children undergoing complete atrioventricular septal defect repair. Retrospective cohort study. Single tertiary pediatric cardiac center. All children admitted to PICU following biventricular surgical repair of complete atrioventricular septal defect from January 2004 to December 2009. None. A total of 107 children, 67 with Down syndrome, were included. Children with Down syndrome were operated earlier: 4 months (interquartile range, 3.5-6.6) versus 5.7 months (3-8.4) for Down syndrome and non-Down syndrome groups, respectively (p < 0.01). There was no early postoperative mortality. There was no significant difference in the prevalence of dysplastic atrioventricular valve between the two groups. Two children (2.9%) from Down syndrome and three children (7.5%) from non-Down syndrome group required early reoperation (p = 0.3). Junctional ectopic tachycardia was the most common arrhythmia, and the prevalence of junctional ectopic tachycardia was similar between the two groups (9% and 10% in Down syndrome and non-Down syndrome, respectively, p = 1). One patient from each group required insertion of permanent pacemaker for complete heart block. Children with Down syndrome had significantly higher prevalence of noncardiac complications, that is, pneumothorax, pleural effusions, and infections (p < 0.01), than children without Down syndrome. There was a trend for longer duration of mechanical ventilation in children with Down syndrome (41 hr [20-61 hr] vs 27.5 hr [15-62 hr], p = 0.2). However, there was no difference in duration of PICU stay between the two groups (2 d [1.3-3 d] vs 2 d [1-3 d], p = 0.9, respectively). In our study, we found no difference in the prevalence of atrioventricular valve dysplasia between children with and without Down syndrome undergoing complete atrioventricular septal defect repair. This finding contrasts with previously published data, and further confirmatory studies are required. Although clinical outcomes were similar, children with Down syndrome had a significantly higher prevalence of noncardiac complications in the early postoperative period than children without Down syndrome.

Early and midterm outcome of complete Atrioventricular Septal Defect (AVSD) in a single institution

Background Surgical repair of complete atrioventricular septal defect is a well established procedure performed in young children. We sought to determine the rate of survival, reoperation and occurrence of Left Atrioventricular Valve Regurgitation (LAVVR).

Abstract 18101: Three-Dimensional Echocardiography Identify Mechanisms of Early Post Operative Valve Failure in Pediatric and Congenital Heart Disease

Background: Three-dimensional echocardiography (3DE) is an accurate and informative imaging modality routinely used in pre-operative assessment for surgical planning. There is however little information on 3DE role in early post-operative valve failure assessment. This is a report on our 5 years experience of 3DE in early post-operative mitral/left atrioventricular valve (MV/LAVV) failure assessment, its impact on our understanding of its mechanism and the clinical decision for returning to the operating theatre for re-repair. Methods and results: We retrospectively reviewed all patients chart and echo with MV/LAVV surgery between 2008 and 2012, excluding primary repair of atrioventricular septal defect (AVSD) and primary valve replacement. Early valve failure (EVF) is defined as moderate or greater MV/LAVV regurgitation within 30 days post repair. There were 132 MV/LAVV surgeries consisting of repaired complete AVSD (n=85), congenital MV abnormality (n= 17) and primum AVSD (n= 16). Pre operative moderate or greater regurgitation was present in 55 (42%) and post op EVF in 33 patients (25%). 3DE were performed in 22 (67%) of 33 EVF with 8 patients returning to the operating room for re-repair due to poor clinical progress in intensive care unit. Six of 8 re-repair were infants. 3DE accurately identified the mechanism of regurgitation such as suture dehiscence at base of cleft, leaflet tears and patch failure (see Figure). Four of 8 patients MV/LAVV regurgitation improved to mild or less regurgitation at follow-up (median 2 months), 3 remained unchanged and 1 subsequently died from intractable heart failure despite MV/LAVV regurgitation improving from severe to moderate. Conclusion: Although patient returning for re-repair of EVF were mainly driven by poor clinical progress in intensive care. 3DE accurately identified the mechanism of early post-operative valve failure thus facilitating surgical planning and decision for re-repair.

Repair of complete atrioventricular septal defect: close to the moon? From giant leap for the medical community to small steps for our patients

Outstanding low mortality has been achieved in the last 10 years for almost all types of congenital cardiac repair. If the 60’s, 70’s and 80’s represented times of exceptional innovation, with a giant leap from palliative surgery to the attainment of successful complex repairs, then the current era is a time of perfectionism, with goals of achieving very low morbidity and mortality, in incremental small steps, through technically ideal surgery and advances in overall intensive care and patient management.

Modified Single-Patch Compared With Two-Patch Repair of Complete Atrioventricular Septal Defect

We compared the outcomes of modified single-patch and two-patch surgical repair of complete atrioventricular septal defect (CAVSD) on left ventricular outflow tract (LVOT) diameter and on left atrioventricular valve (LAVV) coaptation. We reviewed retrospectively postoperative 2-dimensional echocardiograms of all CAVSD patients who underwent modified single-patch or two-patch repair between 2005 and 2011. We measured the leaflet coaptation length of the LAVV in the apical four-chamber view. The LVOT was measured in the long axis view. Fifty-one patients underwent CAVSD repair at a median age of 4 months (range, 1 to 9 months) (single-patch, n=29; two-patch, n=22). The images from 46 echocardiograms were adequate for analysis. Modified single-patch repair required significantly shorter bypass time (102.0±33.6 vs 152.9±39.5 minutes, p<0.001) and ischemic time (69.0±21.7 vs 106.9±29.7 minutes, p<0.001) than did two-patch repair. The indexed coaptation length of the septal and lateral leaflets was not different between single-patch and two-patch (3.1±2.3 vs 4.1±3.1 mm/m2, p=0.25; 2.3±2.3 vs 3.3±3.0 mm/m2, p=0.21). Indexed LVOT diameter was not different in the two groups (26.1±5.2 vs 28.5±7.1 mm/m2, p=0.22). There was no hospital or late death during the median follow-up time of 35 months (range, 1 to 69 months). Five patients underwent reoperation after single-patch repair (3 with residual ventricular septal defect [VSD] and LAVV regurgitation, 1 with residual VSD, 1 with pacemaker implantation). After the two-patch repair, 1 patient required reoperation for a residual VSD and right atrioventricular valve regurgitation (p=0.22). The modified single-patch repair was performed with significantly shorter bypass time and myocardial ischemic time. The postoperative LVOT diameter and LAVV leaflet coaptation length were not significantly different between techniques.

Surgical Outcome of Reoperation Due to Left Atrioventricular Valve Regurgitation After Previous Correction of Complete Atrioventricular Septal Defect

The aims of this study were to evaluate the early and late outcomes in patients undergoing reoperation due to left atrioventricular valve regurgitation (LAVVR) after initial complete repair (ICR) of complete atrioventricular septal defect (CAVSD). Between January 1990 and April 2013, 45 consecutive patients underwent reoperation due to severe LAVVR. The mean age was 7.5 ± 6.2 years. Associated LAVV malformations were found in 22 (49%) patients and associated cardiac malformations in 18 (40%). The mean follow-up was 6.8 ± 2.6 years. LAVV repair was possible in all patients. There were two hospital deaths (4.5%). Ten patients (22%) required a second reoperation due to severe LAVVR at mean 7.5 ± 8.4 months after the first reoperation. The actuarial overall survival and free-reoperation survival rates at one, three, and five years were 95.4%, 92.8%, and 92.8% and 89%, 80.5%, and 72%, respectively. Multivariate analysis revealed that the associated cardiac malformations, LAVV leaflet prolapse or detachment from the septal patch, associated LAVV malformations, and post-first correction LAVVR grade ≥ 2 were strong predictors for poor overall free-reoperation survival in patients undergoing reoperation due to LAVVR after ICR of various forms of ACVSD. Patients with severe LAVVR post-ICR of CAVSD may undergo reoperation with acceptable postoperative mortality and morbidity; however, they are at an increased risk for developing postoperative LAVVR and subsequent reoperation.

Subaortic Stenosis after Repair of Complete Atrioventricular Septal Defect: Anatomic and Technical Issues

Objective: This report is a review of our institutional experience with subaortic stenosis developing after repair of complete atrioventricular septal defects (CAVSD).

Surgical Management of Complete Atrioventricular Septal Defect with Tetralogy of Fallot

The objective of this report is to evaluate our surgical approach to complete atrioventricular septal defect with tetralogy of Fallot (TOF) and to determine the best treatment for this malformation. From 2007 to 2012, 17 children underwent surgical correction of complete atrioventricular septal defect with TOF. Nine of these patients underwent complete correction, two underwent one and a half ventricle repair, and six underwent univentricular repair. There was 1 in-hospital death and 1 late death. Patients with lower weight required longer intensive care unit stay (P <0.05; r = -0.834). Follow-up data were available for all patients who survived the operation (range, 3-60 months). The Kaplan-Meier curve indicated that 5-year survival was 88.2%. All survivors remained in good condition in New York Heart Association (NYHA) class I or II. Complete repair of atrioventricular septal defect with TOF has got favorable outcomes in terms of mortality and reoperation rate. Unbalanced atrioventricular septal defect with TOF can be managed well with one and a half ventricle repair. Two-patch technique through a transatrial approach alone is a good treatment choice for patients. Management of right ventricular outflow tract (RVOT) is similar to that of isolated TOF.

Outcomes of repair of complete atrioventricular septal defect in the current era

We sought to evaluate the surgical outcomes of the repair of complete atrioventricular septal defects (cAVSDs) in our institution in the current era. From 2000 to 2011, 138 patients underwent definitive repair of cAVSD. Repair was performed using a two-patch technique in 92.0% of patients and one-patch technique in 2.2%, and the ventricular septal component was closed directly in 5.8% of patients. Operative mortality was 1.4% (2 of 138). Overall mortality was 5.8% (8 of 138). Follow-up was 96% complete. Freedom from reoperation was 84.3% (95% CI 77.1-91.5%) at 8 years. Age >6 months at repair was associated with higher rates of reoperation (P = 0.001; HR 6.85; 95% CI 2.30-20.44). However, operating at <6 months of age was associated with longer intensive care unit stay (P = 0.019; median 2.7 vs 1.4 days), mechanical ventilation (P = 0.001; median 1.7 vs 0.9 days) and postoperative hospital stay (P = 0.016; median 8 vs 5 days). Moderate or greater left atrioventricular valvular regurgitation (LAVVR) at discharge was a risk factor for reoperation (P < 0.001; HR 10.85; 95% CI 3.75-31.40). Repair of cAVSD carries low mortality, but a moderate reoperation rate. An optimal time for repair of the cAVSD is between 3 and 6 months of age. Repair prior to 3 months of age and the need for cleft closure were associated with a higher degree of LAVVR at discharge. Greater LAVVR at discharge is a risk factor for reoperation regardless of age at initial repair. In the current era, Down's syndrome is not a risk factor for reoperation.

Comparison of Two Surgical Techniques for Complete Atrioventricular Septal Defect Repair Using Two- and Three-Dimensional Echocardiography

Different surgical techniques for complete atrioventricular septal defect (CAVSD) repair have been described, with the double-patch technique being most frequently employed. More recently a newer technique using a modified single-patch repair has been advocated. We hypothesized that the modified single-patch technique would result in an increased incidence of the two major post-repair comorbidities, namely, distortion of the left AV valve (LAVV) leaflets and narrowing of the left-ventricular outflow tract (LVOT). We studied 14 patients with CAVSD who underwent either traditional double-patch technique [group 1 (n = 7)] or modified single-patch technique [group 2 (n = 7)]. Preoperative and immediate postoperative two-dimensional (2D) echocardiograms, as well as follow-up 2D and three-dimensional (3D) studies, were reviewed. For group 1, the median age at repair was 4.1 months with a median duration from surgical repair and last echocardiogram of 44 months. For group 2, the median age at repair was 3 months with a median duration from surgical repair and last echocardiogram of 28 months. The two groups had similar demographics and ventricular septal defect size before surgery. For the LAVV, no significant difference was observed with respect to LAVV annulus size, tenting height, and the size of the vena contracta. Furthermore, there was no significant difference in the 2D echocardiographic areas and volumes of the LVOT between pre-repair and immediate post-repair studies for both groups. At the last evaluation, although there had been growth of the LVOT in both groups, no significant difference between areas and volumes were observed. Areas of the LVOT measured by 3D echocardiography on the final study showed no significant statistical difference between both groups. There was good correlation of the areas measured by 2D and 3D echocardiography within each group. In this small group, modified single-patch technique does not appear to tether the LAVV or promote an increase in regurgitation. In the short term, LVOT growth is unaffected, and the repair does not promote LVOT obstruction. 3D echocardiography is useful for area measurements of the LVOT and showed good correlation with areas measured by assumption of the LVOT shape as determined using 2D techniques.

Single patch technique versus double patch technique in repair of complete atrioventricular septal defect

Abstract Methods This retrospective study included 145 consecutive patients who underwent complete atrioventricular (CAVSD) repair between January 2002 and January 2012. Peri-operative data were analyzed. Ninety-two patients had a two-patch technique (group A); 53 patients had a single-patch technique (group B). Results Mean age was 13.17 ± 4.94 months (group A) versus 5.15 ± 1.52 months (group B), ( p p p = 0.315). Aortic cross-clamp times in group A was 135.3 ± 19.6 min and group B 107.7 ± 21.4 min ( p p value p = 0.623). Hospital mortality was seen in 6 patients (6.5%) in group A and 3 patients (5.7%) in group B ( p = 0.606). Conclusions Single-patch technique can be performed with the same results like the two patch technique with a significantly shorter aortic cross clamp and bypass time.

Reoperations for left atrioventricular valve dysfunction after repair of atrioventricular septal defect

Postoperative left atrioventricular valve (LAVV) dysfunction is known to be the principal risk factor influencing outcome after repair of all types of atrioventricular septal defect (AVSD). The purpose of the present study was to identify the risk factors for reoperation and to assess the outcomes after reoperation for LAVV dysfunction. Records of 412 patients who underwent anatomical repair for different types of AVSD from January 2000 to July 2012 were reviewed. The study group (n = 60) included 13 additional patients for whom repair ± LAVV reoperation was performed in a primary institution. Outcomes, independent risk factors, reoperation and death were analysed. There were 7 early, (1.7%) and 1 late death. Forty-seven (11.4%) required 64 reoperations for LAVV dysfunction. The median delay for the first LAVV reoperation was 3.5 months (range: 5 days to 10.0 years). Unbalanced ventricles with small left ventricle [odds ratio (OR) = 4.06, 95% confidence interval (CI): 1.58-10.44, P = 0.004], double-orifice LAVV (OR = 5.04, 95% CI: 1.39-18.27, P = 0.014), prior palliative surgery (OR = 3.5, 95% CI: 1.14-10.8, P = 0.029) and discharge echocardiography documenting LAVV regurgitation grade >2 (OR = 21.96, 95% CI: 8.91-54.09, P < 0.001) were found to be independent risk factors for LAVV reoperation. Twelve-year survival and freedom from LAVV reoperation rates were, respectively, 96.1% (95% CI: 94.1-98.1) and 85.8% (95% CI: 81.3-90.3). Survival was significantly worse in patients who underwent LAVV reoperation (P < 0.001) and in those who underwent valve replacement vs valve repair (P = 0.020). After AVSD repair, LAVV dysfunction appears to be the principal factor that influences outcome. It can usually be managed by repair. Need for multiple reoperations is not uncommon. Long-term outcome in patients with repaired LAVV is favourable.