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Background: Although various determinants of exercise limitation in Fontan patients have been studied, most research has been performed in patients who underwent different surgical procedures with differing haemodynamic characteristics. The aim of the current study was to evaluate non-invasively measured cardiovascular parameters and their influence on exercise performance in paediatric Fontan patients with an extracardiac conduit and moderate-good systolic ventricular function., Methods: Fontan patients, between 8 and 18 years of age, with moderate to good systolic ventricular function and an extracardiac conduit were included. Exercise performance and cardiovascular assessment, comprising echocardiography, aortic stiffness measurement and ambulatory measurement of cardiac autonomous nervous activity were performed on the same day. Healthy subjects served as controls., Results: Thirty-six Fontan patients (age 14.0 years) and thirty-five healthy subjects (age 12.8 years) were included. Compared to controls, Fontan patients had reduced diastolic ventricular function and increased arterial stiffness. No differences were found in heart rate (HR) and cardiac parasympathetic nervous activity. In Fontan patients, maximal as well as submaximal exercise capacity was impaired, with the percentage of predicted capacity ranging between 54 and 72%. Chronotropic competence, however, was good with a peak HR of 174 (94% of predicted). Lower maximal and submaximal exercise capacity was correlated with a higher HR at rest, higher pulse wave velocity of the aorta and a lower ratio of early and late diastolic flow velocity., Conclusion: Contemporary paediatric Fontan patients have an impaired exercise capacity with preserved chronotropic competence. Exercise performance correlates with heart rate at rest, diastolic function and aortic stiffness., (Copyright © 2021 The Author(s). Published by Elsevier B.V. All rights reserved.)

Background: The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses., Methods: A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated., Results: Mitral valve replacement was performed in 61 infants (15 mm, n = 17 [28%]; 16 mm, n = 18 [29%]; 17 mm, n = 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n = 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n = 9 [15%]), subaortic stenosis resection (n = 4 [7%]), aortic valve repair (n = 3 [5%], and aortic valve replacement (n = 6 [10%])., Conclusions: Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The aim of this study was to evaluate early and long-term outcomes (mortality and prosthetic valve replacement) after mitral valve replacement with the 15-mm St Jude Medical prosthesis (St Jude Medical, St Paul, MN)., Methods: A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15-mm St Jude Medical Masters prosthesis at 4 congenital cardiac centers in The Netherlands. Operative results were evaluated and echocardiographic data studied at 0.5, 1, 2, 3, 5, and 10 years after surgery., Results: Surgery was performed in 17 infants. Ten patients (59%) were treated in the intensive care unit before surgery; 8 (47%) were on ventilator support. Median age at surgery was 3.2 months (interquartile range [IQR], 1.2-5.6), and median weight was 5.2 kg (IQR 3.9-5.7). There was 1 early cardiac death and 1 late noncardiac death. Median follow-up time was 9.6 years (IQR, 2.4-13.2), including 8 patients with a follow-up more than 10 years. The first prosthetic valve explantation (n = 11) occurred at a median of 2.9 years (IQR, 2.0-5.4). Other reinterventions were permanent pacemaker implantation (n = 3), subaortic stenosis resection (n = 2), and paravalvular leak repair (n = 1). Prosthetic valve gradients increased from a mean of 5.0 mm Hg (at discharge) to a mean of 14.3 mm Hg (at 5-year follow-up)., Conclusions: Mitral valve replacement with the 15-mm prosthesis can safely be performed in infants and even in neonates. Median freedom from prosthesis replacement for outgrowth is 3.5 years. Thromboembolic complications were rare., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: An anomalous coronary artery is reported in 2% to 23% of patients with tetralogy of Fallot (TOF). Knowledge of coronary anatomy prior to corrective surgery is vital to avoid damage to vessels crossing the right ventricular outflow tract (RVOT). A meta-analysis on the prevalence of anomalous coronary arteries in TOF is lacking to date. Here, an overview of coronary anomalies in TOF is provided and implications for patient management are discussed., Methods: PubMed, Embase and Web of Science were searched. Analysis was done using Revman 5.3 (Cochrane Community, London). The primary analysis focused on the origin and proximal course of the right and left coronary arteries. In addition, the prevalence of large conus arteries and coronary arteriovenous fistulas (CAVF) was calculated., Results: Twenty-eight studies, encompassing 6956 patients, were included; 6% of TOF patients have an anomalous coronary artery. Hereof, 72% cross the RVOT; the majority of the remaining 28% courses behind the aorta. Six percent of patients have a large conus artery and 4% a CAVF. Other coronary anomalies include a left or right coronary artery from the pulmonary trunk or left or right pulmonary artery, coronary tree hypoplasia and anastomoses between coronary and bronchial arteries., Conclusions: The prevalence of coronary anomalies in TOF is 4-6%. In patients with an anomalous coronary artery, 72% cross the RVOT. The combined risk of encountering an anomalous coronary artery or a large conus artery crossing the RVOT is 10.3%. Coronary anatomy should be defined before surgery and the surgical approach adapted accordingly., Competing Interests: Declaration of competing interest None., (Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: We hypothesize that dobutamine-induced stress impacts intracardiac hemodynamic parameters and that this may be linked to decreased exercise capacity in Fontan patients. Therefore, the purpose of this study was to assess the effect of pharmacologic stress on intraventricular kinetic energy (KE), viscous energy loss (EL) and vorticity from four-dimensional (4D) Flow cardiovascular magnetic resonance (CMR) imaging in Fontan patients and to study the association between stress response and exercise capacity., Methods: Ten Fontan patients underwent whole-heart 4D flow CMR before and during 7.5 μg/kg/min dobutamine infusion and cardiopulmonary exercise testing (CPET) on the same day. Average ventricular KE, EL and vorticity were computed over systole, diastole and the total cardiac cycle (vorticity&#95;vol avg cycle , KE avg cycle, EL avg cycle ). The relation to maximum oxygen uptake (VO 2 max) from CPET was tested by Pearson's correlation or Spearman's rank correlation in case of non-normality of the data., Results: Dobutamine stress caused a significant 88 ± 52% increase in KE (KE avg cycle : 1.8 ± 0.5 vs 3.3 ± 0.9 mJ, P < 0.001), a significant 108 ± 49% increase in EL (EL avg cycle : 0.9 ± 0.4 vs 1.9 ± 0.9 mW, P < 0.001) and a significant 27 ± 19% increase in vorticity (vorticity&#95;vol avg cycle : 3441 ± 899 vs 4394 ± 1322 mL/s, P = 0.002). All rest-stress differences (%) were negatively correlated to VO 2 max (KE avg cycle : r = - 0.83, P = 0.003; EL avg cycle : r = - 0.80, P = 0.006; vorticity&#95;vol avg cycle : r = - 0.64, P = 0.047)., Conclusions: 4D flow CMR-derived intraventricular kinetic energy, viscous energy loss and vorticity in Fontan patients increase during pharmacologic stress and show a negative correlation with exercise capacity measured by VO 2 max.

Background: Atrioventricular septal defect (AVSD) has an incidence of 4-5.3 per 10.000 live births and is associated with Down syndrome (DS). Data on arrhythmias and sudden cardiac death (SCD) after AVSD correction is scarce., Aim: To analyse the incidence of post-operative arrhythmias and SCD after AVSD correction and explore risk factors., Methods: This is a retrospective multicenter study including patients after biventricular AVSD correction. Univariate and multivariate analyses were performed to explore risk factors., Results: A total of 415 patients were included with a mean follow-up duration of 9years (range; <30days-47years). Early post-operative SVTs were documented in 33 patients (8%) and late post-operative SVTs in 15 patients (3.6%). Non-syndromic AVSD (p=0.022, HR=2.64; 95% CI=1.15-6.04) and cAVSD (p=0.005, HR=3.7; 95% CI=1.39-7.51) were independent risk factors for early post-operative SVTs and significant more late post-operative SVTs occurred in non-syndromic patients (p=0.016, HR=6.38; 95% CI=1.42-28.71) and in pAVSD (p=0.045, HR=3.703; 95% CI=1.03-13.32). Fifteen patients (3.6%) received a pacemaker. Non-syndromic AVSD (p=0.008, HR=15.82; 95% CI=2.04-122.47), pAVSD (p=0.017, HR=6.26; 95% CI=1.39-28.28) and re-operation (p=0.007, HR=4.911; 95% CI=1.54-15.64) were independent risk factors for postoperative pacemaker implantation. Late life-threatening ventricular arrhythmias and SCD occurred in 0.5% and 1.7% respectively., Conclusion: There is good long-term survival after AVSD correction and incidence of SCD is low. Non-syndromic AVSD and cAVSD are independent risk factors for early post-operative SVTs. Non-syndromic AVSD patients have significant more early 3rd degree AVB and late post-operative SVTs. Non-syndromic patients with partial AVSD who have undergone reoperation have a significant higher risk of pacemaker implantation., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: The aim of this study was to analyze long-term outcomes after the Ross procedure, focusing on autograft function and risk of reoperation in time., Methods: Between February 1994 and February 2016, 154 patients underwent the Ross (n = 105) and Ross-Konno (n = 49) procedure at our institution and were included in this study. Data were collected retrospectively from patients' medical records or through telephone contact. Competing risks analyses were performed to determine incidences of death and reoperation. A multistate model was constructed to provide insights in the clinical trajectory after operation., Results: Median age was 12 years, 74% were pediatric patients, and 66% had previous surgical procedures. There were 8 (5%) early deaths, 6 of whom underwent the Ross-Konno procedure, and 10 (7%) late deaths. Survival rates at 15 and 20 years were 86% in the total cohort and 91% in the isolated Ross subgroup. Linearized occurrence rates of endocarditis and valve thrombosis, thromboembolism, and bleeding events combined were 0.30% per patient-year and 0.15% per patient-year, respectively. Cumulative incidences of all-cause reoperation at 15 and 20 years were 35.2% and 45.3%, respectively. Twenty-six patients needed autograft reoperation, 20 due to dilatation. Cumulative incidences of autograft reoperation at 15 and 20 years were 20.1% and 31.1%, respectively. At latest echocardiogram, 4 patients had moderate aortic regurgitation and none had stenosis., Conclusions: The Ross procedure can be performed safely in young patients with low number of valve-related events. Autograft function remains stable in the first decade after operation, but autograft dilatation in the second decade necessitates reintervention., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Atrioventricular septal defect (AVSD) covers a spectrum of heart anomalies with a common atrioventricular connection and has an incidence of 4-5.3 per 10.000 live births. About half of the AVSDs occur in patient with Down syndrome. This review provides a bench to bedside overview of AVSD. Developmental aspects, nomenclature, anatomy, and classification of AVSD are discussed. Furthermore an overview of genetic and maternal risk factors for AVSD is provided, and available literature on (fetal) diagnosis, surgical techniques and follow-up is presented. Special attention is given to differences in developmental, anatomical and prognostic factors of AVSD between non-syndromic and Down syndrome patients., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Background: Infants and neonates with severe left ventricular outflow tract obstruction may require pulmonary autograft replacement of the aortic root. In this retrospective multicenter cohort study, we present our experience with the Ross procedure in neonates and infants with a focus on midterm survival and pulmonary autograft durability., Methods: A retrospective observational study was performed in 76 infants (aged less than 1 year) operated on in six congenital cardiac centers in The Netherlands and Germany between 1990 and 2013., Results: Patients had a pulmonary autograft replacement of the aortic valve with (68%) or without (32%) septal myectomy. Median patient age was 85 days (range, 6 to 347). Early mortality (n = 13, 17%) was associated with neonatal age, preoperative use of intravenous inotropic drugs, and congenital aortic arch defects. Five patients (9%) died during follow-up. Freedom from autograft reintervention was 98% at 10 years. Echocardiography demonstrated good valve function, with no or trace regurgitation in 73% of patients. Freedom from right ventricular outflow tract reintervention was 51% at 10 years. Univariable analysis demonstrated superior freedom from reintervention of pulmonary homografts compared with aortic homografts or xenografts., Conclusions: Pulmonary autograft replacement of the aortic valve in neonates and infants is a high-risk operation but offers a durable neoaortic valve. Midterm durability reflects successful adaptation of the autograft to the systemic circulation. Late mortality associated with heart failure was an unexpected finding., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: To characterize and directly quantify regurgitant jets of left atrioventricular valve (LAVV) in patients with corrected atrioventricular septal defect (AVSD) by four-dimensional (4D)Flow Cardiovascular Magnetic Resonance (CMR), streamline visualization and retrospective valve tracking., Methods: Medical ethical committee approval and informed consent from all patients or their parents were obtained. In 32 corrected AVSD patients (age 26 ± 12 years), echocardiography and whole-heart 4DFlow CMR were performed. Using streamline visualization on 2- and 4-chamber views, the angle between regurgitation and annulus was followed throughout systole. On through-plane velocity-encoded images reformatted perpendicular to the regurgitation jet the cross-sectional jet circularity index was assessed and regurgitant volume and fraction were calculated. Correlation and agreement between different techniques was performed with Pearson's r and Spearman's rho correlation and Bland-Altman analysis., Results: In 8 patients, multiple regurgitant jets over the LAVV were identified. Median variation in regurgitant jet angle within patients was 36°(IQR 18-64°) on the 2-chamber and 30°(IQR 20-40°) on the 4-chamber. Regurgitant jets had a circularity index of 0.61 ± 0.16. Quantification of the regurgitation volume was feasible with 4DFlow CMR with excellent correlation between LAVV effective forward flow and aortic flow (r = 0.97, p < 0.001) for internal validation and moderate correlation with planimetry derived regurgitant volume (r = 0.65, p < 0.001) and echocardiographic grading (rho = 0.51, p = 0.003)., Conclusions: 4DFlow CMR with streamline visualization revealed multiple, dynamic and eccentric regurgitant jets with non-circular cross-sectional shape in patients after AVSD correction. 4DFlow with retrospective valve tracking allows direct and accurate quantification of the regurgitation of these complex jets.

Background: Management of pulmonary atresia with intact ventricular septum (PAIVS) is challenging and depends on the individual patient's morphologic characteristics. We evaluated outcomes of 25 years of experience in morphologically driven management of PAIVS., Methods: Between January 1985 and December 2011, 60 patients were treated for PAIVS. Patients were retrospectively categorized into mild (n=27), moderate (n=18), and severe (n=15) right ventricular (RV) hypoplasia subgroups based on morphologic characteristics at first presentation. Tricuspid valve (TV) annulus z score increases were analyzed in patients who underwent biventricular repair and in patients who underwent 1.5-ventricular repair. Median follow-up time was 9.8 years (range, 0-25 years). One patient was lost to follow-up., Results: Fifty-nine (98.3%) patients underwent operation. There were 7 early postprocedure deaths and 1 late death. Estimated overall survival was 86.5% at 10 years after initial admission for the total group, and 96.3%, 77.8%, and 79.4% for the subgroups, respectively. In the respective subgroups, biventricular repair was achieved in 24 (88.9%), 3 (16.7%), and 0 patients. In the patients who underwent biventricular repair, there was a significant increase of the TV annulus z score. At latest follow-up, mean New York Heart Association (NYHA) class was 1.3 for the total group and 1.1, 1.2, and 1.5 for the patients who underwent biventricular, 1.5-ventricular, and univentricular repair, respectively., Conclusions: Our individualized management approach to PAIVS showed good early and long-term results. Early decision on the management goal, determined by individual morphologic characteristics at presentation, is of paramount importance. In specific patients, however, taking into account RV development at a later point in time may improve outcome., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To study the changes over time in biventricular size and function, and clinical parameters in patients after repair of tetralogy of Fallot (TOF) without subsequent pulmonary valve replacement (PVR)., Methods: We prospectively included 78 non-PVR patients (age 20(6-60)years at baseline), who were studied twice with a 5-year interval. Patients underwent magnetic resonance imaging for assessment of biventricular size and function. Exercise testing and electrocardiography were performed to determine peak oxygen uptake (peak VO2) and QRS duration. N-terminal prohormone brain natriuretic peptide (NT-proBNP) was assessed additionally., Results: Pulmonary regurgitation (PR), right ventricular (RV) volumes and QRS duration increased during 5-year follow-up (RV end-diastolic volume (EDV) 130 ± 30 ml/m(2) to 138 ± 34 ml/m(2); QRS 132 ± 27 msec to 139 ± 27 msec); peak VO2 decreased (96 ± 19% to 91 ± 17%). RV ejection fraction, RV effective stroke volume (eff.SV), and NT-proBNP levels remained unchanged. The slope of RVEDV increase was 1.6 ± 3.0 ml/m(2)/year, and depended on RVeff.SV, not on RVEDV, at baseline. Increase in RVEDV correlated with increase in QRS duration over time (r=0.28, p=0.016), and with decrease in RV mass/EDV ratio over time (r=-0.42, p<0.001), not with decrease in peak VO2. In subgroup analysis, patients with larger RVs at baseline showed larger increase in PR during follow-up and larger decrease in NYHA class over time., Conclusions: In TOF patients with moderate RV dilatation, RVEDV increased by 1.6 ± 3.0 ml/m(2)/year, irrespective of RV size at baseline, but depended on RVeff.SV at baseline. Despite limited progression in RV size, unfavourable changes occurred during 5 years follow-up, which suggests there is a need for close follow-up., (© 2013.)

Background: The arterial switch operation is the current treatment for transposition of the great arteries. Long-term outcome mainly depends on the patency of the transferred coronary arteries. This study assessed the presence of abnormal coronary findings and neoaortic root dilation late after arterial switch operation., Methods: In 30 adult patients after arterial switch operation (22 men, 22 ± 3 years), computed tomography angiography was performed to assess the coronary anatomy and abnormal coronary findings, defined as significant stenosis, interarterial coronary course, and acute angled coronary origins. Neoaortic root dimensions and coronary takeoff height were also assessed., Results: The most common coronary anatomy pattern was found in 24 of 30 patients. Variant anatomy patterns were seen in 6 patients (5 with aberrant circumflex artery, 1 with a single ostium). The prevalence of abnormal coronary findings was higher in patients with variant coronary pattern as compared with patients with common coronary pattern (100% and 29%, respectively; p = 0.003). In particular, an acute angle of the coronary origin was frequently observed. In patients with an acute angle, larger dimensions of the aortic annulus (p = 0.016) and the sinus of Valsalva (p = 0.002) were observed. Moreover, a higher takeoff of the right (p = 0.030) and left (p = 0.002) coronary ostium was noted in patients with acute angles., Conclusions: Abnormal coronary findings were frequently observed in adult patients after arterial switch operation, especially in patients with a variant coronary anatomy pattern. Neoaortic root dilation and a higher coronary takeoff may explain part of the pathophysiology. Long-term follow-up is needed to determine the clinical significance of these findings., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Within the group of patients undergoing coarctectomy today, two subgroups can be identified: neonates with a critical coarctation and nonneonatal patients. We hypothesize that patients who have to undergo repair in the neonatal period will have more persistent impairment of ventricular performance postoperatively. Accordingly, we aimed to characterize biventricular performance after coarctectomy in neonatal and nonneonatal patients., Methods: Children (aged 0 to 17 years) undergoing a coarctectomy were prospectively included and classified as neonatal (<1 month old) or nonneonatal patients. Age-matched controls were included for each measurement occasion. To evaluate left (LV) and right ventricular (RV) performance, fractional shortening, peak systolic (S') and early diastolic (E') tissue Doppler imaging velocities, and E/E' were assessed preoperatively, at discharge, and 1 year postoperatively (11.4 ± 8.3 months)., Results: In neonatal (n = 18) and nonneonatal (n = 19) patients LV performance significantly improved within the first postoperative year. Yet 1 year postoperatively, LV S' was still lower in neonatal patients vs controls (4.8 ± 1.1 vs 6.1 ± 1.6 cm/s; p = 0.036), whereas comparable results were observed in nonneonatal patients and controls. One year postoperatively, LV diastolic performance was impaired in neonatal (LV E' 8.7 ± 3.1 vs 13.2 ± 3.9 cm/s, p = 0.005) and nonneonatal patients (LV E' 12.1 ± 3.5 vs 15.1 ± 2.4 cm/s, p = 0.008) vs controls. In RV performance variables, no differences were observed 1 year postoperatively between neonatal and nonneonatal patients and controls., Conclusions: In both subgroups, LV diastolic performance does not recover to normal values within the first postoperative year. However, LV systolic performance remains more persistently impaired in patients who have to undergo repair in the neonatal period vs nonneonatal repair., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Current surgical strategies that aim at preventing pulmonary regurgitation in patients with corrected tetralogy of Fallot (cToF) may result in a certain grade of residual pulmonary stenosis (PS). The clinical implications of a postoperative residual PS in cToF patients remain unclear. Pulmonary valve replacement (PVR) is frequently needed during follow-up of cToF patients. The aim of the current study was to determine the role of residual PS in the need for PVR during follow-up in cToF patients., Methods: cToF patients were included if clinical follow-up after primary surgical correction had taken place for a minimum of 5 years. Patient characteristics, surgical factors, and postoperative factors were reviewed, with a special focus on the transpulmonic systolic gradient. Cox proportional hazards regression analysis was performed to identify predictors of PVR., Results: Of 171 cToF patients, 71 (41.5%) underwent PVR after 24.2 years (interquartile range, 16.8-31.6 years). Year of birth, older age at corrective operation, and patch use significantly predicted PVR during follow-up. By contrast, a mild residual PS in cToF patients (peak systolic gradient, 15-30 mm Hg) independently reduced the risk of PVR, as compared with patients without PS (hazard ratio, 0.47; p=0.02) and with moderate-to-severe PS (hazard ratio, 0.35; p=0.01)., Conclusions: In addition to the known risks factors for PVR, a postoperative mild residual PS reduces the risk of PVR during follow-up of cToF patients. This finding provides clinical evidence for a conservative PS relief during correction of ToF., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Long-term results of reoperation for left atrioventricular valve regurgitation (LAVVR) after previous correction of atrioventricular septal defect (AVSD) are scarce. We evaluated long-term outcome of reoperation for LAVVR and identified risk factors for reoperation., Methods: Between December 1976 and July 2006, 45 of 312 patients with correction of different AVSDs underwent reoperation for LAVVR. The cohort of 267 patients who did not need reoperation for LAVVR allowed for the identification of risk factors for reoperation and evaluation of overall survival after primary AVSD repair in a competing risk scenario. Clinical data were obtained by retrospective review., Results: The left atrioventricular valve (LAVV) was repaired in 31 patients (68.9%) and replaced in 14 (31.1%). There were 3 in-hospital deaths (6.7%) and 2 late deaths (4.4%). Estimated overall survival was 88.1% at 15 years after the reoperation, and estimated incidence of death after reoperation in the total patient cohort was 2% at 15 years after the primary AVSD repair. Overall survival was significantly higher after LAVV repair than after replacement (p=0.010). Ten patients with LAVV repair required a second reoperation for LAVVR. At follow-up, survivors were in New York Heart Association functional class I (n=36) or II (n=4). Independent risk factors for first reoperation for LAVVR were associated cardiovascular anomalies (p<0.001), LAVV dysplasia (p<0.001), and nonclosure of the cleft (p=0.027)., Conclusions: After previous correction of AVSD, LAVVR can usually be corrected by valve repair. A very dysplastic valve may necessitate replacement. Overall survival is higher after repair than after replacement. In general, overall survival of patients reoperated on for LAVVR is favorable. The overall mortality rate after primary repair of AVSD is explained only for a small part by mortality after reoperation for LAVVR., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: We evaluated the results of the arterial switch operation (ASO) being performed at our institution for more than 30 years and identified risk factors for mortality and reoperation., Methods: Clinical outcome of 332 consecutive patients with transposition of the great arteries undergoing ASO was retrospectively analyzed, using surgical reports, medical charts, and latest follow-up echocardiography. Statistical analysis was performed using the Kaplan-Meier method and univariable and multivariable binary logistic and Cox regression analyses., Results: In-hospital mortality was 11.4%. At 15 years, estimated overall survival was 85.2%, and estimated freedom from reoperation was 74.0%. Cross-clamp time (p=0.001) and absence of the Lecompte maneuver (p=0.001) were identified as independent risk factors for in-hospital mortality, whereas coronary problems during surgery (p=0.009) and postoperative pacemaker implantation (p<0.001) were independent risk factors for late mortality. Independent risk factors for reoperation were higher age at the time of the ASO (p=0.002), presence of arch abnormalities (p<0.001), coronary problems during surgery (p=0.005), and duration of ventilation (p<0.001). At latest echocardiography, moderate or severe neoaortic regurgitation was present in 3.4% of the patients., Conclusions: Overall, 30 years of experience with the ASO shows good survival and event-free survival rates. Coronary transfer problems during surgery were found to be an important risk factor for late mortality and reoperation. However, coronary anatomy other than 1LCx-2R and an intramural course of the left coronary artery or left anterior descending artery were not risk factors for mortality or reoperation. Neoaortic regurgitation does not seem to form a major problem., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Outcome of surgical correction of atrioventricular septal defects (AVSD) still varies despite enhanced results. We reviewed our 30-year experience with AVSD repair and identified risk factors for mortality and reoperation., Methods: Between 1975 and 2006, 312 patients underwent surgery for complete AVSD (n = 209; 67.0%), partial AVSD (n = 76; 24.4%), or intermediate AVSD (n = 27; 8.6%). Mean age was 2.4 ± 3.9 years; 142 patients (45.5%) were younger than 6 months. Follow-up was 99.0% complete., Results: There were 26 in-hospital deaths (8.3%) and 6 late deaths (2.1% of 283). Estimated overall survival for the total study population was 91.3%, 90.6%, and 88.6% at 1, 5, and 15 years, respectively. In the multivariable logistic regression analysis, surgical era 1975 to 1995 (p < 0.001) and younger age (p = 0.004) were found to be independent risk factors for early mortality, whereas preoperative AV valve insufficiency showed a tendency toward statistical significance (p = 0.052). Of the hospital survivors, 43 patients required a late reoperation. Estimated freedom from late reoperation was 96.4%, 89.3%, and 81.8% at 1, 5, and 15 years, respectively. Multivariable Cox regression analysis showed associated cardiovascular anomalies (p < 0.001), left AV valve dysplasia (p < 0.001), and absence of cleft closure (p = 0.003) to be independent risk factors for late reoperation., Conclusions: AVSD repair can be accomplished with good long-term results. Early surgical era, associated cardiovascular anomalies, left AV valve dysplasia, and absence of cleft closure negatively influence survival and risk of reoperation., (Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Neoaortic root dilatation is observed after the arterial switch operation for transposition of the great arteries. Although structural differences in the vessel wall of these patients may be of influence, we hypothesize that a histomorphologic difference in composition and embedding of the fibrous annulus in transposition of the great arteries may play a role in neoaortic root dilatation., Methods: Two normal human hearts and two unoperated human hearts with transposition of the great arteries, 1 day postnatal, were studied. Histologic sections stained for collagen, myocardium, and elastin were prepared, and three-dimensional reconstructions of the outflow tracts were made to enable comparison of the morphologic structures between the normal hearts and those with transposition of the great arteries., Results: The amount of collagen in the arterial roots was diminished in hearts with transposition of the great arteries compared with the normal hearts. In addition, the anchorage and embedding of both arterial roots in the myocardium was less extensive in transposition of the great arteries. The changed position of the arteries in the malformed hearts results in less support for the roots from the surrounding atrioventricular myocardium., Conclusions: The combination of the observed histomorphologic differences in amount of collagen and myocardial support may be an explanation for the neoaortic root dilatation observed after the arterial switch operation. The developmental background of the observed deficient fibrous annulus formation may originate from an epicardial problem.

Background: The outcome of surgical correction of atrioventricular septal defect and tetralogy of Fallot has improved in recent years but is still reported to be associated with high mortality. Controversy exists about the need of a right ventriculotomy or a right ventricular to pulmonary artery conduit. The purpose of this study was to evaluate our results of atrioventricular septal defect and tetralogy of Fallot repair by transatrial-transpulmonary approaches., Methods: Between 1979 and 2007, 20 consecutive patients underwent correction of atrioventricular septal defect and tetralogy of Fallot. Five patients had undergone prior palliative shunts. In all patients, a transatrial-transpulmonary approach was used and repair was accomplished without a conduit. The two-patch technique was used to correct the atrioventricular septal defect. Clinical data were obtained by retrospective review of inpatient and outpatient clinical charts., Results: There was no in-hospital mortality and one late, noncardiac death. Six patients required eight reoperations, six for left atrioventricular valve insufficiency (repair: n = 4; replacement: n = 2), one for residual ventricular septal defect, and one for pulmonary artery branch obstruction. Follow-up was complete for all patients (median, 17 years; range, 1.5 to 28 years). All 19 survivors were in good clinical condition at last control, without medication, and in New York Heart Association class I (n = 18) or II (n = 1). Transesophageal echocardiography revealed good right ventricular function, low right ventricular outflow tract gradients (mean, 9 +/- 7.4 mm Hg), and trace pulmonary valve insufficiency (n = 11)., Conclusions: Atrioventricular septal defect and tetralogy of Fallot can be repaired with low mortality by the transatrial-transpulmonary approach without the use of a conduit.

Objective: To assess the effect of pulmonary valve replacement (PVR) on the repolarization of patients with tetralogy of Fallot., Background: Pulmonary valve regurgitation may cause right ventricular failure in adult patients with Fallot's tetralogy. In these patients, prolonged depolarization and disturbed repolarization are associated with ventricular arrhythmias and sudden cardiac death., Methods: Thirty Fallot patients (age 32+/-9 years, 19 male) eligible for PVR were studied with cardiac magnetic resonance imaging (CMR) before and 6 months after PVR. Electrocardiograms obtained during initial and follow-up CMR were analyzed and occurrence of ventricular arrhythmias was studied., Results: Right ventricular end-diastolic volume (RV EDV) decreased from 322+/-87 to 215+/-57 ml after PVR (P<0.0001). The spatial QRS-T angle normalized from 117+/-34 to 100+/-35 degrees , P=0.0004 (normal angle <105 degrees). QT dispersion and T-wave complexity did not change significantly. T-wave amplitude decreased from 376+/-121 to 329+/-100 microV (P=0.01). T-wave area decreased from 43+/-15 to 38+/-13 microV s (P=0.02). Decreases in T-wave amplitude and area were most prominent in the right precordial leads overlying the RV. Three patients had sustained ventricular arrhythmias and one patient died suddenly. These patients had a QRS duration >160 ms. No severe ventricular arrhythmias were found in patients with a RV EDV <220 ml, QRS-T angle <100 degrees , QT dispersion <60 ms or T-wave complexity <0.30., Conclusion: Normal repolarization indices may be associated with the absence of severe ventricular arrhythmias. PVR in Fallot patients with dilated right ventricles has a beneficial effect on electrocardiographic indices of repolarization heterogeneity.

Background: We analyzed the results in two centers of using bovine jugular vein graft for right ventricular outflow tract reconstruction., Methods: From April 1999 to July 2005, 133 children with a median age of 30.9 months (range, 4 days to 19 years) underwent graft implantation. Echocardiography was performed during follow-up and retrospectively reviewed., Results: Nongraft-related early mortality occurred in 8 patients. Late mortality occurred in 11 patients, 2 late deaths were graft related (endocarditis). Median follow-up was 31.6 months (range, 1 to 73). Twelve patients received a new graft, because of endocarditis (3), distal pulmonary artery branch stenosis (4), graft obstruction caused by fibrosis (4), or thrombosis (1). Echocardiography Doppler studies showed good conduit function, with 92% of the patients having absent, trivial, or only mild valve regurgitation at last follow-up. A moderate degree of conduit stenosis due to external compression was observed in 2 patients. Twenty-five patients with otherwise intact conduits had hemodynamically significant distal stenosis. In most cases, the pulmonary branch stenosis was related to preoperative small pulmonary arteries and young age at operation. At 31.6 months, significant graft dilatation was observed in 4 grafts and was related to pulmonary artery branch obstruction or pulmonary hypertension. Calcification did not occur in 5 years time. Survival was 85.7%, freedom from conduit explantation was 91%, and freedom from intervention for pulmonary artery branch stenosis was 80% after 5 years., Conclusions: The bovine jugular vein graft is a valuable right ventricular outflow tract conduit, but younger age and small pulmonary arteries increase the risk of distal conduit stenosis.

Background: Predicting changes in right ventricular (RV) size and function after pulmonary valve replacement (PVR) is important for timely reintervention in adult tetralogy of Fallot patients., Methods: We analyzed the influence of pulmonary regurgitation severity and RV size and function before PVR on the outcome of RV size and function after PVR in 27 adult Fallot patients who had cardiac magnetic resonance imaging before and after PVR. RV dimensions were indexed for body surface area., Results: Pulmonary regurgitation (48% +/- 11% of RV stroke volume) was not related to RV dimensions and function before PVR. Moreover, severity of pulmonary regurgitation did not influence changes in RV dimensions after PVR. The indexed RV end-systolic volume before PVR (mean, 98 mL/m2; range, 52 to 235 mL/m2) best predicted the indexed RV end-systolic volume after PVR (mean, 59 mL/m2; range, 24 to 132 mL/m2, r = 0.78, p < 0.001) and the indexed RV end-diastolic volume after PVR (mean, 107 mL/m2; range, 70 to 170 mL/m2, r = 0.73, p < 0.001). Baseline RV ejection fraction corrected for valvular insufficiencies and shunting (21% +/- 7%) best predicted the RV ejection fraction after PVR (43% +/- 10%, r = 0.77, p < 0.001)., Conclusions: Timing of PVR should be based on indexed RV end-systolic volume and corrected RV ejection fraction rather than on severity of pulmonary regurgitation.

Background: Contact of blood with the artificial surfaces of the cardiopulmonary bypass (CPB) system is considered to be a main cause of complement activation. Improving the biocompatibility of the system by reduction of contact activation of blood elements and thereby producing less inflammatory response is evidently desired, especially for neonates and infants who are more susceptible to the deleterious effects of CPB. A phosphorylcholine coating, Phisio, is designed to mimic the natural interfaces of blood. The aim of this study is to compare the influence of a phosphorylcholine-coated CPB system versus an uncoated CPB system on complement activation and clinical outcomes., Methods: In this prospective, randomized, blind, one-center study, 28 neonates and infants with a bodyweight between 3 and 6 kg who were undergoing cardiopulmonary bypass were divided in two groups, the phosphorylcholine group and the control group. Thirteen patients were assigned to the phosphorylcholine group and 15 patients to the control group. Patients with Down syndrome, prematurity, cyanosis, or reoperation were excluded. Complement factor C3b/c, human neutrophil elastase (HNE), interleukin-6, and C-reactive protein were measured before, during, and after CPB. Duration of intensive care stay, ventilation time, highest body temperature, and inotropic medication were the clinical variables., Results: No significant differences were found between the groups for complement factor C3b/c, HNE, interleukin-6, or C-reactive protein during and after CPB. No clinical differences were observed between the groups., Conclusions: Phosphorylcholine coating does not attenuate the complement activation during CPB in neonates and infants.

We report the usefulness of a median sternotomy approach for the surgical treatment of residual aortic gradients or recoarctation and aortic aneurysms. This series confirms that excellent surgical results can be obtained in this technically challenging group of patients needing postcoarctation repair.