Search

Objectives: The aim of this study was to assess the early outcomes and risk factors of paediatric patients requiring extracorporeal membrane oxygenation after cardiac surgery (post-cardiotomy)., Methods: Retrospective binational cohort study from the Australia and New Zealand Congenital Outcomes Registry for Surgery database. All patients younger than 18 years of age who underwent a paediatric cardiac surgical procedure from 1 January 2013 to 31 December 2021 and required post-cardiotomy extracorporeal membrane oxygenation (PC-ECMO) in the same hospital admission were included in the study., Results: Of the 12 290 patients included in the study, 376 patients required post-cardiotomy ECMO (3%). Amongst these patients, hospital mortality was 35.6% and two-thirds of patients experienced a major complication. Hypoplastic left heart syndrome was the most common diagnosis (17%). The Norwood procedure and modified Blalock-Taussig shunts had the highest incidence of requiring PC-ECMO (odds ratio of 10 and 6.8 respectively). Predictors of hospital mortality after PC-ECMO included single-ventricle physiology, intracranial haemorrhage and chylothorax., Conclusions: In the current era, one-third of patients who required PC-ECMO after paediatric cardiac surgery in Australia and New Zealand did not survive to hospital discharge. The Norwood procedure and isolated modified Blalock-Taussig shunt had the highest incidence of requiring PC-ECMO. Patients undergoing the Norwood procedure had the highest mortality (48%). Two-thirds of patients on PC-ECMO developed a major complication., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The Bex-Nikaidoh operation can effectively relieve left ventricular outflow tract obstruction. However, if a conduit is used for right ventricular outflow tract reconstruction, a late reoperation can be anticipated. We examined the impact of double root translocation on outcomes., Methods: We performed a retrospective single-centre study of patients who underwent aortic root translocation between 2006 and 2019., Results: Aortic root translocation was performed in 23 patients at a median age of 1.6 years [interquartile range (IQR) 0.9-2.5]. Concomitant repairs were done in 52.2% of patients (12/23) including the Senning atrial switch in 34.8% (8/23). The right ventricular outflow tract was reconstructed with valved conduits in 39.1% (9/23), direct anastomoses in 4.35% (1/23) and pulmonary autografts in 56.5% of patients (13/23). Aortic cross-clamp time was significantly longer in patients with double root translocation [308 min (IQR 270-259) vs 209 min (IQR 179-281), P = 0.02]; 2 patients in this group required temporary mechanical circulatory support. There were no early deaths. Median follow-up time was 7.5 years (IQR 3.3-10.5). The estimated 10-year survival was 90% [95% confidence interval (CI): 47.3%, 98.5%]. There was no recurrent left ventricular outflow tract obstruction. Freedom from any reoperation was 64.2% (95% CI: 40.8%, 80.3%) at 3 years and 44.5% (95% CI: 21.2%, 65.5%) at 6 years. The main indication for late reoperation was conduit degeneration. Freedom from a right ventricular outflow tract reoperation was significantly higher, and the number of reoperations per patient was lower when a double root translocation had been performed (P = 0.03)., Conclusions: The Bex-Nikaidoh operation effectively relieved left ventricular outflow tract obstruction. A double root translocation further increased procedural complexity but was associated with better mid-term freedom from a right ventricular outflow tract reoperation. It should be considered in suitable patients., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Mitral valve infective endocarditis (IE) in children is rare, and there are few reports on the outcomes of surgery in these patients. This study investigated the long-term outcomes of mitral valve repair in children with IE. Data were retrospectively obtained from medical records and correspondence. Univariable regression analyses were performed and outcomes including survival and freedom from reoperation were analyzed using the Kaplan-Meier method. Surgery for native mitral valve IE was performed in 39 patients between 1987 and 2020. Of these, 92.3% (36/39) of patients underwent mitral valve repair, while 7.7% (3/39) required replacement. Median age was 8 years. Preoperatively, 80.5% (29/36) of patients had moderate or greater mitral regurgitation. Congenital heart disease was present in 38.9% (14/36), while 11.1% (4/36) had rheumatic heart disease and 25.0% (9/36) had prior cardiac surgery. Postoperatively, only 1 patient (2.8%, 1/36) had moderate or greater residual mitral regurgitation. There were 2 early deaths (5.6%, 2/36), with survival being 94.1% (95%CI, 78.5-98.5) at 15 years. At 10 years, freedom from reoperation was 62.9% (95%CI, 41.0-78.5) while freedom from mitral valve replacement was 80.2% (95%CI, 55.5-92.3). Larger vegetation size was a risk factor for embolic events both pre- and postoperatively (OR, 1.15, P = 0.02). Mitral valve repair is feasible in the majority of children requiring surgery for mitral valve IE. Survival is excellent, and at 10 years, approximately two-thirds of patients are free from mitral reoperation, and 80% are free from replacement. Larger vegetation size is associated with an increased risk of embolic events., (Copyright © 2022. Published by Elsevier Inc.)

Background: The optimal management strategy for symptomatic young infants with tetralogy of Fallot (TOF) is yet to be determined. We aimed to evaluate the long-term outcomes of a staged approach with initial shunt palliation followed by complete repair., Methods: Between January 1993 and July 2021, 160 children with TOF underwent a systemic-to-pulmonary shunt at our institution, including 65 neonates (41%). The mean duration of follow-up was 12.3 ± 8.1 years., Results: Hospital mortality was 3% (4 of 160), all occurring in patients with a shunt size-to-weight ratio ≥1.2 mm/kg. Composite morbidity-defined as cardiac arrest, postoperative mechanical circulatory support, or unplanned reoperation-occurred in 21% (33 of 160). On multivariable analysis, a shunt size-to-weight ratio ≥1.2 mm/kg and prematurity were independent predictors of composite morbidity. Interstage mortality was 3% (4 of 156). A limited transannular patch was used in 75% (113 of 150) of TOF repairs. Actuarial survival at 20 years after shunt was 90% (95% confidence interval [CI], 79%-95%). Actuarial freedom from reinterventions at 20 years after TOF repair was 40% (95% CI, 28%-52%). Neonates had comparable composite morbidity, mortality, and late risk of reinterventions to older children., Conclusions: Staged repair of TOF in symptomatic young infants results in low mortality but high rates of reinterventions at long-term follow-up. A shunt size-to-weight ratio ≥1.2 mm/kg is a significant risk factor for mortality and morbidity prior to complete repair. Neonates undergoing shunt insertion have comparable outcomes to older children., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)

Background: Analysis of multi-institutional data and benchmarking is an accepted accreditation standard in cardiac surgery. Such a database does not exist for congenital cardiac surgery in Australia and New Zealand (ANZ). To fill this gap, the ANZ Congenital Outcomes Registry for Surgery (ANZCORS) was established in 2017., Methods: Inclusion criteria included all cardiothoracic and extracorporeal membrane oxygenation (ECMO) procedures performed at five participating centres. Data was collected by data managers, validated by the surgical team, and securely transmitted to a central repository., Results: Between 2015 and 2019, 9723 procedures were performed in 7003 patients. Cardiopulmonary bypass was utilized for 59% and 9% were ECMO procedures. Fifty-seven percent (n = 5531) of the procedures were performed in children younger than 1 year of age. Twenty-four percent of procedures (n = 2365) were performed in neonates (≤28 days) and 33% (n = 3166) were performed in children aged 29 days to 1 year (infants). The 30-day mortality for cardiac cases (n = 6572) was 1.3% and there was no statistical difference between the participating centres (P = 0.491). Sixty-nine percent of cases had no major post-operative complications (5121/7456). For cardiopulmonary bypass procedures (n = 5774), median stay in intensive care and hospital was 2 days (IQR 1, 4) and 9 days (IQR 5, 18), respectively., Conclusion: ANZCORS has facilitated pooled data analysis for paediatric cardiac surgery across ANZ for the first time. Overall mortality was low. Non-risk-adjusted 30-day mortality for individual procedures was similar in all units. The continued evaluation of surgical outcomes through ANZCORS will drive quality assessment in paediatric cardiac surgery across ANZ., (© 2022 The Authors. ANZ Journal of Surgery published by John Wiley & Sons Australia, Ltd on behalf of Royal Australasian College of Surgeons.)

Objective: We aimed to assess the long-term outcomes of children with isolated congenital aortic stenosis who underwent primary aortic valve repair., Methods: Records of all children (n = 111) with isolated congenital aortic stenosis who underwent primary aortic valve repair between 1980 and 2016 were reviewed. An optimal operative outcome consisted of a residual left ventricular outflow tract peak systolic gradient <35 mm Hg and trivial or less aortic insufficiency., Results: Median age at surgery was 0.4 years (interquartile range, 1 month-7.9 years) and median weight at surgery was 7.0 kg (interquartile range, 3.7-25.0 kg). Fifty-two patients (46.8%; 52/111) underwent aortic valve repair with the use of patch material. Early mortality was 0.9% (1/111). Late mortality was 0.9% (1/110). Freedom from aortic valve reoperation was 52.1% (95% CI, 38.7-63.8) at 10 years. Freedom from aortic valve replacement was 67.9% (95% CI, 55.4-77.5) at 10 years. An optimal outcome was achieved in 48 patients (43.2%; 48/111). At 10 years, freedom from aortic valve reoperation was 78.2% (95% CI, 63.1-87.8) in patients with an optimal outcome, compared with 39.4% (95% CI, 22.8-55.6) in those with a suboptimal outcome (P = .03). Tricuspid aortic valve was associated with a suboptimal outcome (P = .01)., Conclusions: Aortic valve repair achieves relief of congenital aortic stenosis with very low early mortality and excellent long-term survival, even in neonates. Although nearly half of the patients required aortic valve reoperation by 10 years, two-thirds of the patients remain free from aortic valve replacement. An optimal outcome was more commonly achieved with bicuspid aortic valves compared with tricuspid aortic valves., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)

Objective: We aimed to assess outcomes after aortic valve repair leading to bicuspid valve anatomy in children., Methods: This is a retrospective study of patients who underwent aortic valve repair with creation/preservation of bicuspid aortic valve anatomy or tricuspidization of bicuspid valves between 1980 and 2016., Results: Overall, 127 patients underwent bicuspid repair. Median age was 0.73 years (interquartile range, 0.1-8.9), and median weight was 8.15 kg (interquartile range, 3.9-31.7). The cohort included 22.8% neonates (n = 29), 28.3% infants (n = 36), and 48.8% children (n = 62). Repair was performed without a patch in 54.3% (n = 69). Survival at 10 years was 94.8% (95% confidence interval, 87.5-97.85). Freedom from aortic valve reoperation at 5 and 10 years was 79.9% (95% confidence interval, 71.2-86.2) and 65.6% (95% confidence interval, 53.4-75.3), respectively. Re-repair was undertaken in 53.7% (22/41). Freedom from aortic valve replacement at 5 and 10 years was 90.3% (95% confidence interval, 83.1-94.5) and 75.8% (95% confidence interval, 63-84.7), respectively. Risk factors for reoperation were age less than 1 year, unicuspid valve, and the presence of Shone complex and concomitant aortic arch repair. There were 107 patients (107/127, 84.25%) with preoperative bicuspid aortic valve morphology that was preserved. They were compared with a separate cohort of 44 patients who underwent tricuspidization of bicuspid aortic valve during the same period. There was no difference in survival or freedom from aortic valve reoperation. However, freedom from aortic valve replacement was lower after tricuspidization with 49.7% (95% confidence interval, 26.3-69.3) versus 75.8% (95% confidence interval, 62.1-85.1) after 10 years (P = .0118)., Conclusions: Aortic valve repair leading to bicuspid valve anatomy in children has satisfactory long-term results. Reoperation remains common, but the need for early valve replacement can be effectively delayed., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)

Background: Repair of complete atrioventricular septal defect (cAVSD) is achieved with low mortality. However, there is a high rate of reoperation on the left atrioventricular valve (LAVV), which is often attributed to nonclosure of the cleft. Although nonclosure of the cleft has been reported to be a risk factor for reoperation, no randomized controlled or propensity-matched trials have ever been performed. We investigated the effect of cleft closure on outcomes after cAVSD repair., Methods: We reviewed 455 patients who underwent cAVSD repair between 1990 and 2019. To determine the effect of cleft closure, propensity score matching was performed on risk factors for reoperation after cAVSD repair., Results: Median age was 3.6 months (mean, 9.6 ± 20.4 months), median weight was 4.3 kg (mean, 4.7 ± 4.3 kg) and 41.9% (191 of 455) were male. Early mortality was 2.9% (13 of 455), and survival was 89.8% ± 1.9% at 20 years. Early reoperation was a risk factor for mortality (P = .004). Freedom from reoperation was 72.5% ± 4.0% at 20 years. Freedom from LAVV reoperation was 74.1% ± 4.0% at 20 years. Preoperative severe LAVV regurgitation (P < .001) and early postoperative moderate or greater LAVV regurgitation (P = .007) were risk factors for reoperation, while trisomy 21 (P = .03) and recent era of surgery (P = .02) were protective. Propensity score matching yielded 106 pairs. There were no differences in long-term survival (P = .71) or reoperation (P = .26) between the 2 groups., Conclusions: Repair of cAVSD can be achieved with low mortality and good long-term survival, although the reoperation rate remains high. Similar freedom from reoperation can be achieved with or without closure of the LAVV cleft., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Left ventricular outflow tract obstruction (LVOTO) is a recognized complication after complete repair of atrioventricular septal defect (AVSD). This study reviewed the incidence and management of LVOTO following AVSD repair at a single institution., Methods: From 1975 to 2019, 24 patients (3.3%, 24/730) underwent reoperation due to LVOTO following partial AVSD (pAVSD) and complete AVSD (cAVSD) repair. The data were retrospectively reviewed., Results: The incidence of LVOTO following pAVSD and cAVSD repair was 4.4% (12/275) and 2.6% (12/455). Freedom from LVOTO reoperation following pAVSD and cAVSD repair at 25 years was 94.3% [95% confidence interval (CI); 89.7-96.7] and 95% (95% CI; 91.1-97.3). The median time from complete repair of pAVSD and cAVSD to LVOTO reoperation was 4.4 years [interquartile range (IQR): 3.4-6.7] and 2.6 years (IQR: 2.2-4.7). Freedom from second LVOTO reoperation at 5, 10 and 15 years was 83.7% (95% CI; 57.2-98.2), 59.2% (95% CI; 28.7, 80.3) and 39.5% (95% CI; 13.2-65.3). The median time between the first and the second LVOTO reoperation in the groups of pAVSD and cAVSD was 6.1 years (IQR: 3.4-8.9) and 8.6 years (IQR: 5.7-9.8). There was no significant difference regarding the first (P = 0.7406) and subsequent LVOTO (P = 0.7153) following complete repair of pAVSD and cAVSD. Combined access to the left ventricular outflow tract was not protective regarding LVOTO reoccurrence. Survival for both groups after LVOTO reoperation at 15 years was 95.6% (95% CI 99.4-72.9)., Conclusions: Incidence of LVOTO after AVSD repair is low but the reoccurrence rate is high. Standard subaortic resection does not always provide definitive LVOTO relief. The survival after LVOTO reoperation is excellent., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Progressive aortic regurgitation is a common complication in patients supported with continuous flow ventricular assist devices. The risk of neoaortic regurgitation is likely to be particularly high in patients with univentricular hearts owing to the lack of fibrous support for the neoaortic valve. Previously described techniques for addressing neoaortic regurgitation in this setting have required cardiopulmonary bypass and cross clamping, or ligation of the neoaortic root. We present a simple technique of external partial annuloplasty of the neoaortic valve, which can be performed without the need for cardiopulmonary bypass or cross clamping., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Complete atrioventricular septal defect (cAVSD) repair is usually performed between 3 and 6 months of age. However, some children present with early heart failure requiring intervention. It is unclear whether primary complete repair or initial pulmonary artery banding (PAB) provides better outcomes., Methods: All patients (n = 194) who underwent surgery for cAVSD younger than 3 months of age between 1990 and 2019 were included. Propensity score matching was performed on risk factors for mortality., Results: Primary complete repair was performed in 77.8% (151/194), whereas PAB was performed in 22.2% (43/194). Children who had PAB were younger (P < .01), had lower weight (P < .001), and less trisomy 21 (P = .04). Interstage mortality for PAB was 18.6% (8/43), whereas early mortality for primary repair was 3.3% (5/151). Survival at 20 years was 92.0% (95% confidence interval [CI], 85.6%-95.7%) for primary repair and 63.2% (95% CI, 42.5%-78.1%) for PAB (P < .001). There was no difference in left atrioventricular valve (LAVV) reoperation rates (P = .94). Propensity score matching produced 2 well-matched groups. Survival at 20 years was 94.2% (95% CI, 85.1%-98.8%) for primary repair, and 58.4% (95% CI, 33.5%-76.7%) for PAB (P = .001). There was no difference in LAVV reoperation rates (P = .71). Neonatal repair was achieved with no early deaths and 100% survival at 10 years., Conclusions: In children younger than 3 months of age, complete repair of cAVSD is associated with better survival than PAB. Both strategies have similar rates of LAVV reoperation. Neonatal repair of cAVSD can be achieved with excellent results. Primary repair of cAVSD should be the preferred strategy in children younger than 3 months of age., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

Background: The Ross procedure in children is performed either as a primary operation, or a secondary operation after initial aortic valve surgery., Objectives: The study aimed to determine whether the outcomes of primary and secondary Ross procedure are similar., Methods: All patients who underwent Ross procedure between 1995 and 2018 were included in the study. Outcomes were compared between those who had primary Ross procedure and those who had secondary Ross procedure, after aortic valve surgery. Propensity score matching for baseline characteristics and risk factors for death and reoperation was performed., Results: Of 140 Ross procedures, 51.4% (n = 72 of 140) were primary operations, while 48.6% (n = 68 of 140) were secondary operations. Patients undergoing primary Ross procedure tended to be older (median age 8.6 years vs. 7.0 years; p = 0.10) and have a higher weight (28.9 kg vs. 19.4 kg; p = 0.07). There were no significant differences in survival or freedom from reoperation in the unmatched cohort. Propensity score matching resulted in 50 well-matched pairs. In the matched cohort, survival at 10 years was 90.0% (95% confidence interval [CI]: 77.5% to 95.7%) in the primary Ross group, compared with 96.8% (95% CI: 79.2% to 99.5%) in the secondary Ross group (p = 0.04). Freedom from autograft reoperation at 10 years was 82% (95% CI: 64.1% to 91.5%) in the primary Ross group, compared with 97.0% (95% CI: 80.4% to 99.6%) in the secondary Ross group (p = 0.03)., Conclusions: Secondary Ross procedure performed after initial aortic valve surgery achieves superior long-term survival and freedom from autograft reoperation compared with primary Ross procedure. A strategy of initial aortic valve repair followed by delayed Ross procedure may provide better long-term survival and freedom from autograft reoperation., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

Background: Data on the long-term outcomes in children after the Ross operation are limited. This study aimed to assess the long-term outcomes in children who underwent the Ross operation at a single institution., Methods: The study reviewed all children (n = 140) who underwent the Ross operation at the Royal Children's Hospital in Melbourne, Australia between 1995 and 2018., Results: Mean follow-up time was 8.9 years. Median age at operation was 7.4 years. The root replacement (n = 120, Ross-Konno; n = 38), root inclusion (n = 17), and subcoronary implantation (n = 3) techniques were used. Operative mortality was 5.0% (7 of 140; 3 neonates and 4 infants). There were 6 late deaths. Overall survival at 10 years was 96.2% in children older than 1 year of age and 78.9% in children younger than 1 year of age at operation (P = .003). Freedom from autograft reoperation was 86.0% at 10 years. Age younger than 1-year at operation was a risk factor for autograft reoperation (P = .02). Patients younger than 1 year of age at operation experienced a higher incidence of moderate or greater aortic insufficiency compared with patients who were older than 1 year of age (P = .006). In patients who had a poly-(p-dioxanone)-filament band placed around the sinotubular junction, freedom from moderate or greater aortic insufficiency at 10 years was 100%, compared with 83.1% in patients with no band (P = .09)., Conclusions: In children older than 1 year of age, the Ross operation has excellent outcomes with no operative mortality and a low incidence of aortic insufficiency. In children younger than 1 year of age, the Ross operation is associated with higher operative mortality and a higher incidence of aortic insufficiency. Where possible, the Ross operation should be delayed beyond infancy. Poly-(p-dioxanone)-filament banding may reduce the incidence of aortic insufficiency after the Ross operation., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: To evaluate whether the long-term outcomes of modified-single-patch (MSP) repair of complete atrioventricular septal defect are equivalent to double-patch (DP) repair with respect to survival and risk of reoperation for left atrioventricular valve regurgitation or left ventricular outflow tract obstruction., Methods: All patients who underwent biventricular repair of complete atrioventricular septal defect in Australia from 1990 to 2015 using either a MSP or DP technique were identified. Demographic characteristic details, operative data, and outcomes were analyzed. A propensity score analysis was performed to balance the 2 treatment groups according to several baseline covariates. Survival and freedom from reintervention between the 2 groups were compared using Kaplan-Meier curves and log-rank tests., Results: A total of 819 patients underwent repair of complete atrioventricular septal defect (252 MSP and 567 DP) during the study period. There was no significant difference in unmatched survival (P = .85) and event-free survival (P = .49) between MSP and DP repair. Propensity score matching resulted in a total of 223 matched pairs. Matched analysis found no difference in overall survival (P = .59) or event-free survival (P = .90) between repair techniques, with an estimated event-free survival at 5, 10, and 15 years of 83%, 83%, and 74% for DP and 83%, 80%, and 77% for the MSP group, respectively. There was no significant difference between repair techniques in reoperation for left atrioventricular valve regurgitation or left ventricular outflow tract obstruction or need for permanent pacemaker., Conclusions: Overall and event free survival are similar following either MSP or DP repair of complete atrioventricular septal defect. There is no increased risk of reoperation for left ventricular outflow tract obstruction with the MSP technique., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether the transatrial-transpulmonary approach to tetralogy of Fallot repair in non-neonatal patients provides superior outcomes compared with the transventricular approach. Altogether, 175 papers were found using the reported search, of which 11 represented the best evidence to answer the clinical question. Two randomized controlled trials (RCTs) and 3 observational studies showed that the transatrial approach resulted in better preservation of right ventricular (RV) function, whereas 4 observational studies showed no significant difference. Three observational studies showed better attenuation of RV dilatation, whereas 3 showed no difference. One RCT and 2 observational studies showed lower incidence of postoperative ventricular arrhythmias, while 1 RCT and 4 observational studies showed no difference. Two observational studies demonstrated greater freedom from reoperation, 1 RCT and 2 observational studies showed no difference, while 1 retrospective study observed a higher incidence of residual RV outflow tract obstruction and lower freedom from reoperation in infants. Two observational studies reported lower risk of requiring pulmonary valve replacement, whereas 2 reported no difference. Three observational studies reported superior exercise capacity, while 1 reported no difference. No difference in long-term survival was demonstrated. The results presented suggest that transatrial repair of tetralogy of Fallot confers superior or equivalent outcomes in terms of preservation of RV function and volume, ventricular arrhythmias, need for pulmonary valve replacement, and exercise capacity compared with transventricular repair. However, the incidence of residual RV outflow tract obstruction may be higher in infants undergoing transatrial repair., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: In 2011 we reported unfavourable outcomes of second-run extracorporeal life support (ECLS) in children. We wanted to investigate whether this previous report affected our strategy and modified our long-term outcomes., Methods: Between 1988 and 2015, 31 patients underwent a second-run ECLS. Median age at the time of first support was 9days (0-16 years). Median length of support for the first and second runs were 4.7days (0.1-10) and 3.6days (0.5-8.7) respectively, with an interval of 1.8days (0.1-66) between supports., Results: There was an increasing trend in the number of patients undergoing second-run ECLS after our report: 21 patients between 1988 and mid-2010 (0.9 patients/year) and 10 between mid-2010 and 2015 (two patients/year) (p=0.06). However, among all the patients who underwent ECLS, the proportion of second-run ECLS was not different before and after 2010 (4% vs. 4.2% respectively, p=0.92). While 58% of patients (18/31) survived weaning of support, only 23% (7/31) survived to hospital discharge and 14% (5/31) were still alive after hospital discharge at a median of 6.5 years (1.2-11.6). The three patients who had positive long-term outcomes had the second-run ECLS instituted to allow for major cardiac operations., Conclusions: Compassionate use of second-run ECLS is difficult to refuse but one should be aware that its outcomes are dismal. In our centre, benefits seem to be limited to cases where the second-run ECLS allows for a major cardiac intervention., (Copyright © 2018 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Surgical treatment of multiple ventricular septal defects (VSDs) has advanced significantly in recent years, yet remains technically challenging. With high rates of complications and reoperations, we sought to assess the outcomes of patients undergoing a variety of management techniques for multiple VSDs. From 1988 to 2015, 157 consecutive patients underwent surgical management of multiple VSDs at a median age of 2.2 months (2 days-16 years). Sixty-nine patients (44%) had exclusively multiple VSDs, 62 patients (39%) had multiple VSDs with concomitant intracardiac anomalies, and 26 patients (17%) had multiple VSDs with aortic arch anomalies. The predominant techniques used at the initial operations were patch closure (84 patients), pulmonary artery band (83 patients), suture closure (37 patients), and sandwich technique (13 patients). Eighteen patients underwent ventriculotomies. There were 3 hospital deaths (2%). Mean follow-up time was 8.6 ± 6 years (1 day-22 years). Four patients died during follow-up, whereas freedom from reoperations was 52% (95% confidence interval 42-61%) at 16 years. Freedom from reoperation was significantly lower in the 1988-2002 era than in the post-2002 era (38% vs 73%, P = 0.016). Pacemaker implantation was ultimately required in 9% (14 of 150) of patients. No deleterious impact of a ventriculotomy could be detected. Surgical treatment of multiple VSDs can be performed with excellent short- and long-term survival, and normal late functional outcome, however, carries a significant rate of reoperation. The recent inclusion of absorbable pulmonary artery bands and the sandwich technique appear safe and are useful adjuncts in these patients., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objectives: Preoperative moderate or greater truncal valve (TV) insufficiency is one of the most important factors influencing mortality in children with truncus arteriosus. We therefore sought to determine the impact of TV insufficiency and concomitant TV surgery on children who underwent truncus arteriosus repair at a single institution., Methods: We reviewed 180 patients who underwent truncus arteriosus repair between 1979 and 2016. Preoperative echocardiography demonstrated TV insufficiency in 80 patients (mild: 33.9%, 61/180; moderate: 9.4%, 17/180 and severe: 1.1%, 2/180)., Results: Twenty-one patients had concomitant TV surgery with an early mortality of 19% (4/21) and overall survival of 70.8 ± 10.1% at 25 years. There were 60 neonates, 11 of whom had concomitant TV surgery with an early mortality of 27% (3/11) and overall survival of 62.3 ± 15.0% at 20 years. Concomitant TV repair (P = 0.5) was not a risk factor for death. TV reoperation was common in those who had concomitant TV surgery, with freedom from reoperation of 19.2 ± 14.9% at 20 years. In the remaining 159 patients, 14 required subsequent TV surgery, and the freedom from TV surgery was 84.0 ± 4.6% at 20 years. At a median follow-up of 18.5 years, TV insufficiency was none or trivial in 79.6% (109/137) and mild or less in 98.5% (135/137) of patients., Conclusions: Most patients with mild TV insufficiency are free from TV surgery up to 25 years. The durability of TV repair is poor. Most patients with moderate or greater TV insufficiency and a quadricuspid TV will require TV surgery.

Background: Patients with atrioventricular septal defect (AVSD) may require pulmonary artery banding (PAB), either as a part of a staged univentricular palliation or to allow delayed biventricular repair in patients presenting with early heart failure. The long-term outcomes of PAB in children with AVSD have not been previously reported., Methods: All children with AVSD who underwent PAB at a single institution were included in the study. Data were obtained from medical records and correspondence with general practitioners and cardiologists., Results: A total of 68 patients with complete AVSD underwent PAB, of whom 58.8% of patients (40 of 68) had balanced AVSD (bAVSD) and underwent PAB with intent to subsequently perform biventricular repair. The remaining 41.2% of patients (28 of 68) had unbalanced AVSD (uAVSD) and underwent PAB as part of staged univentricular repair. PAB was not associated with a short-term increase in atrioventricular valve (AVV) regurgitation (p = 0.24). In patients with bAVSD, 83.8% (95% confidence interval [CI]: 67.4% to 92.4%) achieved biventricular repair. Survival was 73.4% (95% CI: 54.3% to 85.5%) and freedom from left AVV operation was 60.0% (95% CI: 36.1% to 77.4%) at 20 years of follow-up. In patients with uAVSD, 61.9% (95% CI: 40.5% to 77.5%) had achieved Fontan completion at 10 years of follow-up. Survival was 60.9% (95% CI: 36.2% to 78.5%) and freedom from AVV operation was 78.6% (95% CI: 55.5% to 90.6%) at 20 years., Conclusions: PAB can be used in patients with AVSD without compromising AVV function. Most patients with bAVSD progress to biventricular repair, albeit with a high rate of AVV reoperation. Patients with uAVSD who undergo PAB have similar outcomes to the overall uAVSD population., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Partial atrioventricular septal defect (pAVSD) is usually repaired between 2 and 4 years of age with excellent results. Repair during infancy has been associated with poorer outcomes. However, most infants in reported series had heart failure or significant left atrioventricular valve (LAVV) regurgitation. The impact of surgery during infancy on outcomes remains unclear., Methods: All children at three institutions who underwent repair of pAVSD from 1975 to 2015 were included. Infants (aged <1 year) were compared with older children in a propensity score matched analysis. Variables used to generate propensity scores were: failure to thrive, congestive heart failure, preoperative LAVV regurgitation, associated congenital heart disease, sex and the presence of trisomy 21., Results: pAVSD repair was performed on 430 children, 17.4% (75/430) were infants. Infants (mean age 0.5±0.3 years) had higher rates of LAVV regurgitation, heart failure and additional cardiac malformations than older children (mean age 4.7±3.5 years). At 30 years, survival for infants was 82.1% (95% CI 70.1% to 89.6%) compared with 95.7% (95% CI 91.3% to 97.9%) in older children (P<0.001).Propensity score matching yielded 52 well-matched pairs. Survival at 30 years was 87.9% (95% CI 75.0% to 94.4%) for infants compared with 98.1% (95% CI 87.1% to 99.7%) for older children (P=0.04). There was no significant difference in freedom from reoperation between the groups., Conclusions: Despite matching for risk factors, survival after repair of pAVSD during infancy is lower than that when repair is performed in older children, with no difference in reoperation rates. This suggests that elective repair of pAVSD should be deferred until after infancy., Competing Interests: Competing interests: Yd’U is a consultant for Actelion and MSD. CPB serves on the advisory board of Admedus., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objectives: To determine the incidence of vocal cord paresis (VCP) after neonatal aortic arch repair/Norwood-type procedure, and the effectiveness of noninvasive laryngeal ultrasound in detecting VCP compared with gold standard invasive nasoendoscopy., Methods: Fifty-two patients who underwent an arch repair (39 of 52; 75%) or Norwood-type procedure (13 of 52; 25%) via sternotomy between April 1, 2015, and April 30, 2017 underwent laryngeal ultrasound (50 of 52; 96%) and/or flexible fiber optic nasoendoscopy (39 of 52; 75%) at 48 to 72 hours after endotracheal extubation. Primary arch diagnoses were coarctation in 56% (29 of 52), hypoplastic left heart syndrome in 17% (9 of 52), isolated hypoplastic arch in 17% (9 of 52), and interrupted aortic arch in 10% (5 of 52). The median patient age at surgery was 5.5 days (interquartile range, 4.0-12.5 days). Fifteen patients (15 of 52; 29%) required preoperative intubation., Results: Left VCP was present in 59% (23 of 39) of patients on nasoendoscopy and in 59% (27 of 46) of patients on laryngeal ultrasound, and 4 additional patients had inconclusive ultrasound results. There was agreement between the results of nasoendoscopy and conclusive ultrasound in all cases. The overall sensitivity, specificity, positive and negative predictive values, and Cohen's kappa coefficient of laryngeal ultrasound compared with nasoendoscopy for the detection of left VCP were 95%, 88%, 91%, 93%, and 0.83, respectively. On multivariable analysis, preoperative intubation and arch repair techniques other than the Norwood procedure were associated with left VCP (odds ratio, 12.7; P = .03; and 14.1; P = .03, respectively)., Conclusions: There is a high incidence of VCP after arch repair via sternotomy. Laryngeal ultrasound seems to be an effective and noninvasive method for detecting VCP in neonates and young children., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: To study the recent trends and outcomes of congenital mitral valve surgery in children., Methods: From 2008 to 2014, 84 procedures in 66 consecutive patients (41 procedures in 31 patients with mitral stenosis and 43 procedures in 35 patients with mitral regurgitation) were retrospectively evaluated. The mean age at surgery was 4.3 ± 5.4 years, and 27 patients (41%) were neonates or infants., Results: Seven (11%) patients died during the follow-up period of 3.2 ± 2.3 years and 5 (71%) were <1 year. Ten mitral valve replacements were performed in 8 patients, including 1 pulmonary valve homograft, 3 Contegra conduits of 12 mm thickness in the intra-annular position and 6 mechanical valves. Shone's syndrome, dysplastic valve, a need for valve replacement and age <1 year were the risk factors for death or reoperation in a univariable analysis, while in a multivariable analysis of all patients, valve replacement and age <1 year remained as risk factors. In a multivariable analysis of 27 patients aged <1 year, mitral valve dysplasia was a significant risk factor for reoperation or death. The 5-year rate of freedom from death or reoperation in neonates or infants was 55% and that in patients aged >1 year was 88% (P = 0.003)., Conclusions: An age of <1 year, mitral valve dysplasia and a need for mitral valve replacement were associated with a higher incidence of death or reoperation. Primary mitral valve replacement or univentricular strategy may have to be considered for symptomatic neonates with Shone's syndrome., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: This study reviews the outcomes of children under 1 year of age who had left atrioventricular valve (LAVV) replacement (LAVVR) in one centre and explores the benefits of an innovative approach for LAVVR in very small patients., Methods: Thirteen consecutive patients operated for LAVV replacement between 1997 and 2016 were reviewed retrospectively. Indication for surgery was regurgitation in 7, stenosis in 5 and both stenosis and regurgitation in 1. Nine patients (69%) had previous LAVV repair. Median age at surgery was 126 days (39-327 days). In the primary surgery, 7 mechanical valves and 1 mitral homograft were implanted. Five inverted semilunar valve conduits were implanted consisting of a Contegra valve in 4 and a pulmonary homograft in 1., Results: Hospital mortality was 31% (4 of 13). Two patients required postoperative extracorporeal membrane oxygenation. Six patients developed complete atrioventricular block, with 2 survivors requiring a pacemaker. Late mortality was 31% (4 of 13). Two of the 4 patients who received an inverted Contegra conduit died. Median follow-up of the 5 survivors was 4 years (2-16 years). Four patients had 10 further replacements consisting of 6 redo conventional mechanical valves replacement, 3 supra-annular valve implantation, and 1 modified Ross II. The 5 inverted semilunar valve conduits implanted lasted for 1, 5, 6, 22 and 37 months., Conclusions: LAVVR below 1 year of age is associated with a considerable operative and late mortality. LAVVR with an inverted conduit bearing semilunar valves may be an alternative strategy for patients with the smallest annuli., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Studies of long-term outcomes of discrete subaortic stenosis are rare. Therefore, we reviewed the long-term outcomes of fibromuscular resection in children with subaortic stenosis over 26 years from a single institution., Methods: We conducted a retrospective review of all children (n=72) who underwent resection of subaortic obstruction for discrete subaortic stenosis between 1989 and 2015., Results: Median age at surgery was 5.0 years (2.7-7.6 years). There were no operative deaths but three late deaths (4.2%, 3/72). Overall Kaplan-Meier survival at 10 years was 93.0 ± 3.9% (95% CI: 79.6, 97.7). Peak instantaneous left ventricular outflow tract Doppler gradient decreased from 74.2±36.7mmHg (16.0-242.0mmHg) preoperatively to 12.8±7.4mmHg (2.6-36.0mmHg) postoperatively (p<0.001). Mean left ventricular outflow tract Doppler gradient decreased from 42.4±17.2mmHg (12.0-98.0) preoperatively to 7.5±2.7mmHg (1.4-19.3mmHg) postoperatively (p<0.001). However, over the mean follow-up period of 7.8±6.1 years (0.1-25.2 years), 29.0% (20/69) of patients had recurrence and 18.8% (13/69) required reoperation at median time of 4.8 years (3.1-9.1 years) after the initial repair. Freedom from reoperation at 10 years was 71.1±7.1% (95% CI: 54.6, 82.3). Risk factors for reoperation were age less than five years at initial repair (p=0.036) and extension of the membrane to the aortic valve (p=0.001). Aortic insufficiency was present in 54.2% (39/72) of patients preoperatively. Progression of aortic insufficiency occurred in 38.9% (28/72). Involvement of the aortic valve at initial repair was associated with need for subsequent aortic valve repair or replacement (p=0.01)., Conclusions: Resection of subaortic obstruction is associated with low mortality and morbidity. Recurrence and reoperation rates are high and progression of aortic insufficiency following subaortic resection is common. Therefore, these patients warrant close follow-up into adult life., (Crown Copyright © 2016. Published by Elsevier B.V. All rights reserved.)

Objectives: Partial atrioventricular septal defect (pAVSD) is repaired with excellent long-term survival. However, up to 25% of patients require reoperations. This study reviews results of reoperation following pAVSD repair at a single institution., Methods: From 1975 to 2012, 40 patients (16%, 40/246) underwent reoperation following pAVSD repair at the study institution. The data were retrospectively reviewed., Results: The mean time to reoperation was 5.4 ± 5.8 years. The most common reoperations were left atrioventricular valve (LAVV) surgery (78%, 31/40) and resection of left ventricular outflow tract obstruction (20%, 8/40). The most common cause for LAVV surgery was regurgitation through the cleft (58%, 18/31), followed by central regurgitation (29%, 9/31). Most cases of LAVV regurgitation were treated by repair (77%, 24/31), rather than replacement (23%, 7/31). Since the introduction of a patch augmentation technique for LAVV repair in 1998, the rate of repair has increased from 54 to 94% (P = 0.012). The early mortality rate was 2.5% (1/40). The survival rate was 90% (95% CI: 76-96) at 10 years and 83% (95% CI: 60-94) at 20 years. The rate of freedom from further reoperation was 66% (95% CI: 46-80) at 10- and 20-year follow-up., Conclusions: The most common cause for reoperation following pAVSD repair was LAVV regurgitation through the LAVV cleft. Reoperation is performed with survival comparable to that of primary pAVSD repair, yet the rate of further reoperations remains high. The patch augmentation technique for LAVVR has significantly increased the rate of successful LAVV repair., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We evaluated the long-term outcomes following repair of truncus arteriosus (TA) from a single institution. We conducted a retrospective review of children (n = 171) who underwent TA repair between 1979 and 2014. Early mortality rate was 11.7% (20/171). There were 19 late deaths. Most deaths (74%, 29/39) occurred within the first year following surgery. The 1-year mortality rate in 1979-2004 was 18% (25/136) and decreased to 11% (4/35) in 2005-2014. The overall survival rate was 73.6% at 30 years. Multivariate analysis identified postoperative extracorporeal membrane oxygenation (P = 0.003), operative weight <2.5kg (P = 0.012), prior surgical intervention (P = 0.018), and coronary artery anomaly (P = 0.037) as risk factors for early mortality. A Cox regression model identified DiGeorge syndrome (P = 0.008) as a risk factor for late mortality. Freedom from right ventricular outflow tract reoperation was 4.6% at 20 years. Concomitant truncal valve (TV) repair or replacement was undertaken in 20 patients. Additionally, 14 patients underwent late TV repair or replacement. The overall survival rate in patients who underwent TV operation was 76.9% at 20 years. A total of 19 patients had concomitant interrupted aortic arch with a survival rate of 89.5% at 20 years. Median follow-up was 19 years (mean = 17 years, range: 1-34 years). All patients were in New York Heart Association Class I/II at last follow-up. Following repair of TA, patients had good long-term functional status but had high reoperation rates. Repair of interrupted aortic arch and TV were not risk factors for mortality. Postoperative extracorporeal membrane oxygenation, operative weight <2.5kg, prior surgical intervention, and coronary artery anomaly were risk factors for early death. DiGeorge syndrome was associated with late death, most commonly from infection., (Crown Copyright © 2016. Published by Elsevier Inc. All rights reserved.)

Objectives: To identify determinants of adverse outcomes in patients with atrial isomerism., Methods: Determinants of survival were analyzed for the group as a whole as well as separately for left and right atrial isomerism., Results: There were 78 cases with right and 104 with left atrial isomerism. Nineteen patients were not offered surgery; 49 (47%) of those with left atrial isomerism and 60 (77%) with right atrial isomerism were directed to single ventricle palliation. A total of 67 patients died. Survival to 25 years was 62% (95% confidence interval [CI], 53%-69%). Independent predictors of mortality were obstructed total anomalous pulmonary venous drainage (P < .001; hazard ratio [HR], 5.2; 95% CI, 2.7-9.7) and asplenia (P = .008; HR, 2; 95% CI, 1.2-3.3). There was no evidence that patients born in the recent era had improved survival (P = .47; HR, 1.1; 95% CI, 0.8-1.5). Survival was better for patients with left than right atrial isomerism: 18 years survival 74% (95% CI, 63%-82%) versus 50% (95% CI, 38%-60%). Independent predictors of mortality for patients with left atrial isomerism were dextrocardia (P = .009; HR, 3.0; 95% CI, 1.3-6.7) and pulmonary stenosis (P = .042; HR, 0.3; 95% CI, 0.1-0.9) and patients with right atrial isomerism, biventricular repair (P < .001; HR, 6.0; 95% CI, 2.8-13), and obstructed total anomalous pulmonary venous drainage (P < .001; HR, 4.2; 95% CI, 2.0-8.6)., Conclusions: A significant proportion of patients with isomerism still die before reaching adulthood. Only a fraction of those with obstructed pulmonary veins survive. Having biventricular repair does not confer a survival advantage to those born with right atrial isomerism., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Partial atrioventricular septal defect (pAVSD) is routinely repaired with a low mortality. However, limited data are available on the long-term follow-up of these patients. The current study was designed to determine long-term survival and morbidity of a large cohort of patients operated on at a single institution., Methods: From 1975 to 2012, 249 consecutive patients underwent pAVSD repair at the Royal Children's Hospital. The follow-up data were obtained from hospital records, correspondence with cardiologists and primary care physicians, patient surveys and the state death registry., Results: The early mortality rate was 1.2% (3/249), while the long-term survival rate was 96% (95% CI: 93-98%) at 10 years and 94% (95% CI: 89-97%) at 30 years. Freedom from reoperation was 84% at 10 years and 75% at 30 years. The most common reoperations were left atrioventricular valve surgery (30/249, 12.1%), resection of left ventricular outflow tract obstruction (12/249, 4.8%) and closure of residual atrial septal defects (5/249, 2.0%). Implantation of a permanent pacemaker was required in 3.2% (8/249) of patients. Despite a substantial reoperation rate, only 43% of patients older than 18 years of age were seen by a cardiologist within the most recent 2 years of the study period, compared with 80% of those younger than 18 years (P < 0.001)., Conclusions: Repair of pAVSD is performed with a low mortality and excellent long-term survival. However, a substantial reoperation rate warrants close follow-up into adulthood., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: To evaluate the outcomes associated with surgical repair of scimitar syndrome., Methods: From 1974 to 2012, 21 patients with scimitar syndrome underwent corrective surgery. Outcomes included mortality, reoperation rate, hospital length of stay and long-term functional status., Results: The mean age of surgery was 5.4 years (2.5 months to 16.7 years). Sixteen (76.2%) patients were symptomatic at presentation. Fifteen (71.4%) patients presented with the infantile form and 6 (28.6%) with the adult form of scimitar syndrome. Operative techniques included baffle repair in 15 (71.4%) patients, reimplantation in 5 (23.8%) patients and pneumonectomy in 1 (4.8%) patient. Six (28.6%) patients were operated in infancy at a mean of 4 months (2.5-9.6 months) of age. There was 1 (4.8%) hospital death and 3 (15%) late deaths. The risk factors for mortality included preoperative pulmonary hypertension (P = 0.006) and surgery during infancy (P = 0.003). The incidence of postoperative pulmonary vein stenosis was 23.8% (n = 5). Three (14.3%) patients underwent reoperation for pulmonary vein stenosis. Neither baffle (P = 0.6) nor reimplantation (P = 0.55) surgical techniques influenced the rate of stenosis. The follow-up was complete in 15 (88%) survivors at a mean of 13.7 years (1.3-38.5 years). All surviving patients were asymptomatic and had a New York Heart Association functional status Class I., Conclusions: Mortality was highest in patients who had preoperative pulmonary hypertension and those who were operated on before 1 year of age. Surgical technique did not influence the occurrence of pulmonary vein stenosis. Survivors were asymptomatic at the long-term follow-up., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The incidence of congenital cardiac abnormalities remains high. Paediatric patients with congenital cardiac defects often require surgery at a young age. The surgeries are often long and complex, rendering this population particularly vulnerable to the deleterious effects of cardiopulmonary bypass and cardiac surgery. The search for cardioprotective strategies is ongoing in an attempt to reduce the morbidity in this population. In the post-genomic era, it is apparent that simply determining the genomic sequences holds little diagnostic potential and means to determine progression of disease and response to treatment. The field of proteomics is expanding and application of proteomic techniques in the clinical setting holds great potential to advance our understanding of the proteomic changes involved in specific disease stages. This review will assess the application of proteomic techniques in the setting of paediatric cardiac surgery and highlight the need to obtain a clear understanding of the role of various proteins in children with cardiac conditions. The success and challenges of the available proteomic technology will be discussed as well as the future potential of proteomic methods for advancing our understanding of protein changes in children requiring cardiac surgery., (© 2014 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.)

Background: The purpose of this study was to assess the outcomes of children undergoing repair of aortopulmonary window (APW)., Methods: We conducted a retrospective review of all children (n=43) who underwent surgical repair of APW between 1980 and 2013., Results: Median age at surgery was 40 days (range, 13 to 125). Simple APW was present in 15 of 43 patients (35%), and 28 of 43 patients (65%) patients had concomitant cardiovascular anomalies. The aorta was repaired by direct suturing in 36 patients (84%) patients and patching in 7 patients (16%). The main pulmonary artery was repaired by direct suturing in 22 patients (51%) patients and by patching in 21 (49%). Cardiopulmonary bypass was used in 42 of the 43 patients (97.7%). Single-staged repair of concomitant cardiovascular anomalies was undertaken in 26 of 28 patients (93%). Only 2 of the 28 patients (7%) underwent repair of interrupted aortic arch before APW repair. Operative mortality was 6.7% (1 of 15 patients) among patients with simple APW and 18% (5 of 28 patients) among patients with concomitant anomalies. Operative weight less than 2.5 kg was associated with mortality on univariable analysis (p=0.02). Median follow-up was 10.1 years (range, 0.17 to 24.2). There were no late deaths. Overall survival was 86% (95% confidence interval: 71.3 to 94.2) at 10 years. Freedom from reoperation was 95.3% (95% confidence interval: 86.2 to 99.9) at 10 years. At last follow-up, all patients were in New York Heart Association functional class I/II., Conclusions: Survival beyond discharge from the hospital is associated with excellent outcomes., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Assessment of diastolic function has not been described after repair of total anomalous pulmonary venous drainage (TAPVD), but studies of exercise capacity demonstrate impaired performance in this population despite normal systolic function. We postulated that diastolic impairment might contribute to this finding., Methods: We analyzed echocardiographic variables from 28 patients with repaired TAPVD and compared these with data from 32 healthy controls (normals) and 21 subjects with repaired transposition of the great arteries (TGA)., Results: Left ventricular (LV) end-diastolic volumes were smaller in the TAPVD group (median, 50 mL/m(2) compared with a median of 64 mL/m(2) in TGA and 67 mL/m(2) in normals; P < .001 in each case). LV diastolic function in the TAPVD group was impaired. Mitral early to late ratio was increased (median, 2.7 in TAPVD compared with a median of 1.9 in TGA [P = .047] and 2.1 in normals [P = .021]). LV isovolumic relaxation time was reduced (median, 50 milliseconds in TAPVD compared with a median of 70 milliseconds in both TGA and normals; P < .001 in each case). Late diastolic and systolic tissue Doppler velocities were lower and the E/e' ratio was higher in the TAPVD group., Conclusions: Patients with repaired TAPVD are usually regarded as having excellent outcomes, but the finding of LV diastolic dysfunction in this population warrants more careful follow-up. We postulate that the diastolic impairment in these patients is the result of relative unloading of the LV during early cardiac development. These findings may also have implications in considering therapeutic approaches for hypoplastic ventricles in attempting to achieve biventricular repair., (Copyright © 2014 The American Association for Thoracic Surgery. All rights reserved.)

Objective: To compare the long-term reoperation rates among surgeons performing transatrial repair of tetralogy of Fallot., Methods: The data set of 675 patients undergoing transatrial repair of tetralogy of Fallot at 1 institution from 1980 to 2005 was analyzed for intersurgeon differences in the reoperation rates., Results: A follow-up period >15 years was available for 5 surgeons, allowing for comparison (541 patients; >80 patients/surgeon). The reintervention rate at 10 years varied from 8.8% (95% confidence interval [CI], 5.3%-14.5%) to 26.7% (95% CI, 14.9%-44.9%; hazard ratio, 3.4; P = .001). The procedures of 1 surgeon resulted in a reoperation rate of 10.5% at 20 years (95% CI, 5.4%-25.3%). The type of reoperation required varied among the surgeons. One surgeon had had no reoperations for pulmonary artery stenosis. Of the 5 surgeons, 2 (surgeons 2 and 5) had equivalent overall 10-year reoperation rates (24.1%, 95% CI, 12.9%-42.3%; vs 26.7%, 95% CI, 14.9%-44.9%; P = .32). Surgeon 5 had reoperation almost exclusively for right ventricular outflow tract obstruction (20.6%; 95% CI, 12.4%-33.1%) and surgeon 2 for right ventricular dilation (17.4%; 95% CI, 7.8%-36.3%). None of the patients treated by surgeon 5 required implantation of a valved conduit., Conclusions: An analysis of the reoperation rate during the long-term follow-up of transatrial repair of tetralogy of Fallot identified variability in the outcomes among 5 surgeons. The analysis of these differences suggested that an optimal amount of opening of the right ventricular outflow tract can lead to a decreased reintervention rate. The analysis of intersurgeon variability in outcomes should be encouraged, because it will lead to improvements in cardiac surgery outcomes., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: The study sought to evaluate the late outcomes of a policy of transatrial repair delayed beyond the neonatal period., Background: Long-term outcomes of transatrial repair of tetralogy of Fallot are unknown., Methods: The records of 675 consecutive patients undergoing a transatrial repair of tetralogy of Fallot between 1980 and 2005 were reviewed, their follow-up updated and survival confirmed from national death registries. One-third (220 of 675) had undergone previous palliation. Median age at repair was 2 years in the first 8 years, and 1 year from 1988 onward. A transannular incision was performed in 75% of cases and autologous pericardium was the material used to patch this incision in 92% of cases., Results: There were 7 hospital deaths (1%). Eight patients died during follow-up (2 sudden unexpected and 6 noncardiac deaths). Mean follow-up was 11.7 ± 6.3 years. Twenty-five years' survival was 97% (95% confidence interval [CI]: 95% to 98%). Twenty-five years' freedom from implantation of a valved conduit was 84.6% (95% CI: 77.8% to 89.5%). By multivariable analysis, prior palliation and younger age at repair were predictive of implantation of a valved conduit (hazard ratio: 2.4, 95% CI: 1.3 to 4.6, p = 0.008; hazard ratio: 0.70, 95% CI: 0.50 to 0.96, p = 0.03, respectively)., Conclusions: During long-term follow-up, transatrial repair of tetralogy of Fallot was associated with a minimal risk of sudden death and low rate of reintervention for right ventricular dilation and residual outflow tract obstruction., (Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Remote ischemic preconditioning (RIPC) appears to protect distant organs from ischemia-reperfusion injury. We undertook meta-analysis of clinical studies to evaluate the effects of RIPC on organ protection and clinical outcomes in patients undergoing cardiac surgery., Methods: A review of evidence for cardiac, renal, and pulmonary protection after RIPC was performed. We also did meta-regressions on RIPC variables, such as duration of ischemia, cuff pressure, and timing of application of preconditioning. Secondary outcomes included length of hospital and intensive care unit stay, duration of mechanical ventilation, and mortality at 30 days., Results: Randomized control trials (n = 25) were included in the study for quantitative analysis of cardiac (n = 16), renal (n = 6), and pulmonary (n = 3) protection. RIPC provided statistically significant cardiac protection (standardized mean difference [SMD], -0.77; 95% confidence interval [CI], -1.15, -0.39; Z = 3.98; P < 0.0001) and on subgroup analysis, the protective effect remained consistent for all types of cardiac surgical procedures. However, there was no evidence of renal protection (SMD, 0.74; 95% CI, 0.53, 1.02; Z = 1.81; P = 0.07) or pulmonary protection (SMD, -0.03; 95% CI, -0.56, 0.50; Z = 0.12; P = 0.91). There was no statistical difference in the short-term clinical outcomes between the RIPC and control groups., Conclusions: RIPC provides cardiac protection, but there is no evidence of renal or pulmonary protection in patients undergoing cardiac surgery using cardiopulmonary bypass. Larger multicenter trials are required to define the role of RIPC in surgical practice., (Crown Copyright © 2014. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to compare outcomes after surgical valvuloplasty and balloon dilation of the aortic valve in neonates and infants., Background: Surgical techniques of aortic valve repair have improved and there is today controversy on the best approach to treat neonatal congenital aortic valve stenosis., Methods: Retrospective review of data and follow-up of 123 consecutive neonates and infants (35 females, 88 males) undergoing intervention for congenital aortic stenosis., Results: From 1977 to 2009, 123 consecutive neonates (<30 days) and infants (31 days to 1 year) underwent relief of congenital aortic stenosis. Median age at procedure was 27 days (6 to 76 days). Twenty-year survival was 80 ± 7%. Fifty-four patients required a re-intervention and freedom from re-intervention was 55 ± 6% at 10 years and 40 ± 6% at 20 years. By multivariate analysis, having the relief of stenosis by balloon valvuloplasty and undergoing initial treatment as a neonate were predictive of re-intervention. Freedom from re-intervention at 5 years was 27% after balloon valvuloplasty versus 65% after surgery. At latest follow-up, an additional 16 patients had moderate or severe stenosis and 8 had regurgitation. Freedom from re-intervention or stenosis was 39 ± 5% at 15 years. By multivariate analysis, balloon valvuloplasty (p < 0.001) and treatment as a neonate (p = 0.003) were again predictive of stenosis or re-intervention. Thirty-five patients ultimately needed a valve replacement. Significant predictor of the requirement of valve replacement was unicuspid aortic valve (p < 0.001). Freedom from valve replacement was 55 ± 7% at 20 years., Conclusions: Surgical valvuloplasty remains the best approach to treat neonates and infants with congenital aortic stenosis. After surgery, a higher proportion of patients remain free of re-intervention than after interventional catheterization and the relief of their stenosis lasts longer., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: The myocardial protective effect of remote ischemic preconditioning has been demonstrated in heterogeneous groups of patients undergoing cardiac surgery. No studies have examined this technique in neonates. The present study was performed to examine the remote ischemic preconditioning efficacy in this high-risk patient group., Methods: A preliminary, randomized, controlled trial was conducted to investigate whether remote ischemic preconditioning in cyanosed neonates undergoing cardiac surgery confers protection against cardiopulmonary bypass. Two groups of neonates undergoing cardiac surgery were recruited for the present study: patients with transposition of the great arteries undergoing the arterial switch procedure and patients with hypoplastic left heart syndrome undergoing the Norwood procedure. The subjects were randomized to the remote ischemic preconditioning or sham control groups. Remote ischemic preconditioning was induced by four 5-minute cycles of lower limb ischemia and reperfusion using a blood pressure cuff. Troponin I and the biomarkers for renal and cerebral injury were measured pre- and postoperatively., Results: A total of 39 neonates were recruited-20 with transposition of the great arteries and 19 with hypoplastic left heart syndrome. Of the 39 neonates, 20 were randomized to remote ischemic preconditioning and 19 to the sham control group. The baseline demographics appeared similar between the randomized groups. The cardiopulmonary bypass and crossclamp times were not significantly different between the 2 groups. The troponin I levels were not significantly different at 6 hours after cardiopulmonary bypass nor were the postoperative inotrope requirements. Markers of renal (neutrophil gelatinase-associated lipocalin) and cerebral injury (S100b, neuron-specific enolase) were not significantly different between the 2 groups., Conclusions: Our data suggest that remote ischemic preconditioning in hypoxic neonates undergoing cardiopulmonary bypass surgery does not provide myocardial, renal, or neuronal protection. Additional studies are needed to examine the relationships among developmental age, hypoxia, and the molecular mechanisms of ischemic preconditioning., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: To determine rate of reoperation subsequent to primary valve repair in a pediatric population., Methods: Between 1996 and 2009, 142 consecutive patients underwent aortic valve repair in our institution. Median age at surgery was 9 years, with 30 being younger than age 1 year. Indication for surgery was stenosis (n = 76), regurgitation (n = 55), and both (n = 11). Forty-six patients underwent repair with no addition of patch, whereas 96 patients required addition of patches of glutaraldehyde preserved autologous pericardium for cusp extension (n = 51) and other repair (n = 45)., Results: In the early postoperative period after cusp extension repair, 2 patients had a sudden unexplained death and 1 had cardiac arrest requiring mechanical support and heart transplantation. Two additional patients with cusp extension displayed signs of coronary ischemia. After a mean follow-up of 3.4 ± 3.5 years, only 1 patient died of a noncardiac cause. Seven-year freedom from reoperation was 80% (95% confidence interval [CI], 66-89). By multivariate analysis, the only predictors of reintervention were cusp extension (hazard ratio [HR], 5.4; 95% CI, 1.7-16.8; P = .004) and infants (HR, 5.6; 95% CI, 1.7-18.4; P = .005). At final echocardiography follow-up, 23 of 119 survivors without reoperation had moderate (19%), 1 had moderate-severe (1%), and 1 had severe regurgitation (1%), whereas 12 (10%) had a moderate degree of stenosis., Conclusions: Aortic valve repair in pediatric populations is effective in postponing reintervention. The longevity of the repair is shorter after cusp extension and when performed in infants. Caution should be used when performing tricsupidization and cusp extension of bicuspid valves because it can be responsible for mortality related to occlusion of the coronary ostia by patches., (Crown Copyright © 2013. Published by Mosby, Inc. All rights reserved.)

Objectives: Late outcomes of repair of coarctation with arch hypoplasia have not yet been described. Hypertension and arch reobstruction frequently occur after standard coarctation repair and thus we sought to determine the long-term results of repair in the subset of patients with arch hypoplasia at a single institution over a 20-year period., Methods: We reviewed the files of the 305 consecutive patients quoted to have arch hypoplasia who had undergone a coarctation repair in a single institution between 1984 and 2004. Repair was performed through a sternotomy in 74 patients (24%), 58 of them undergoing a repair consisting of an end-to-side anastomosis., Results: Early mortality was 9% but there was only 1 death among patients without a major associated anomaly. Eight patients required reintervention before discharge due to residual obstruction. The follow-up was available in 96% of the patients. Only 45% of the patients over 15 years of age had cardiology review in the last 2 years. Survival at 10 and 20 years was 94% (95% CI: 91-97%) and 92% (95% CI: 86-95%), respectively. There were a total of 66 late reinterventions in 49 (18%) patients. Ten- and 20-year freedom from reintervention was 84% (95% CI: 78-88%) and 72% (95% CI: 63-80%), respectively. Ten- and 20-year freedom from reobstruction was 75% (95% CI: 69-80%) and 45% (95% CI: 34-55%), respectively. Patients undergoing end-to-side repair from sternotomy had less arch reobstruction than those undergoing extended end-to-end repair by thoracotomy (92 vs 61% freedom from reobstruction at 10 years, P < 0.001). Only 68% of the patients were normotensive at the last follow-up. Arch obstruction on echocardiogram was associated with an increased prevalence of hypertension (P = 0.018)., Conclusions: After coarctation repair, half of the patients with hypoplasia of the transverse arch will develop arch reobstruction and a third will become hypertensive. The technique of end-to-side repair performed through a sternotomy seems to alleviate these issues, and could be offered to a larger proportion of patients with hypoplasia of the aortic arch. Many of these patients are lost to follow-up during adolescence, at a time when ongoing care seems mandatory.