Long-Term Outcomes Following the Ross Procedure in Neonates and Infants: A Multi-Institutional Analysis.

OBJECTIVES: For neonates and infants with aortic valve pathology, the Ross procedure has historically been associated with high rates of morbidity and mortality. Data regarding long-term durability are lacking. METHODS: The international, multi-institutional Ross Collaborative included six tertiary-care centers. Infants who received a Ross operation between 1996-2016 (allowing a minimum five years of follow-up) were retrospectively identified. Serial echocardiograms were examined to study evolution in neoaortic size and function. RESULTS: Primary diagnoses for the 133 patients (n=30 neonates) included isolated aortic stenosis (AS; 14%, n=19), Shone complex (14%, n=19), and AS+other (excluding Shone complex; n=95, 71%) including arch obstruction (n=55), left ventricular hypoplasia (n=9), and mitral disease (>moderate stenosis or regurgitation, n=31). At the time of Ross, median age was 96 (IQR 36-186) days and median weight was 4.4 (3.6-6.5) kg. In-hospital mortality occurred in 13/133 (10%) patients (4/30 [13%] neonates). Post-discharge mortality occurred in 10/120 (8%) patients at a median 298 days post-Ross. Post-Ross neoaortic dilatation occurred, peaking at 4-5 standard deviations above normal at 2-3 years before returning to near-baseline z-score at a median follow-up of 11.5 [6.4-17.4] years. Autograft/LVOT reintervention was required in 5/120 (4%) patients at a median 10.3 [4.1-12.8] years. Freedom from >moderate neoaortic regurgitation (AR) was 86% at 15 years. CONCLUSIONS: Neonates and infants experience excellent post-discharge survival and long-term freedom from autograft reintervention and AR following Ross. Neoaortic dilatation normalizes in this population in the long-term. Increased consideration should be given to Ross in neonates and infants with aortic valve disease.

Association of left ventricular outflow tract size with arch morphology in interrupted aortic arch.

OBJECTIVES: Left ventricular outflow tract obstruction (LVOTO) is a major cause of morbidity and mortality in infants with interrupted aortic arch (IAA). Left Ventricular Outflow Tract (LVOT) development may be flow-mediated, thus IAA morphology may influence LVOT diameter and subsequent reintervention. We investigated the association of IAA morphology [type and presence of aortic arch aberrancy (AAb)] with LVOT diameter and reintervention. METHODS: All surgical patients with IAA (2001-2022) were reviewed at a single institution. We compared IAA-A versus IAA-B; IAA with aortic AAb versus none; IAA-B with aberrant subclavian (AAbS) artery versus others. Primary outcomes included LVOT diameter (mm), LVOTO at discharge (≥50 mmHg), and LVOT reintervention. RESULTS: Seventy-seven infants (mean age 10 ± 19 days) were followed for 7.6 (5.5-9.7) years. Perioperative mortality was 3.9% (3/77) and long-term mortality was 5.2% (4/77). Out of 51 IAA-B (66%) and 22 IAA-A (31%) patients, 30% (n = 22) had AAb. Smaller LVOT diameter was associated with IAA-B [IAA-A: 5.40 (4.68-5.80), IAA-B: 4.60 (3.92-5.50), P = 0.007], AAb [AAb: 4.00 (3.70-5.04) versus none: 5.15 (4.30-5.68), P = 0.006], and combined IAA-B + AAbS [IAA-B + AAbS: 4.00 (3.70-5.02) versus other: 5.00 (4.30-5.68), P = 0.002]. The likelihood of LVOTO was higher among AAb [N = 6 (25%) vs N = 1 (2%), P = 0.004] and IAA-B + AAbS [N = 1 (2%) vs N = 6 (30%), P = 0.002]. Time-to-event analysis showed a signal towards increased LVOT reintervention in IAA-B + AAbS (P = 0.11). CONCLUSIONS: IAA-B and AAb are associated with small LVOT diameter and early LVOTO, especially in combination. This may reflect lower flow in the proximal arch during development. Most reinterventions occur in IAA-B + AAbS, hence these patients should be carefully considered for LVOT intervention at the time of initial repair.

Surgical Melody Mitral Valve: A Paradigm Shift for Infants with Unrepairable Mitral Valve Disease.

BACKGROUND: Melody valve (Melody) for mitral valve replacement (MVR)(MelodyMVR) has been an effective strategy to treat unrepairable mitral valve disease in small children. The purpose of this study is to analyze survival, durability, and complications of MelodyMVR strategy. METHODS: Patients who underwent MelodyMVR between 2014 and 2023 were included. Transplant-free survival was analyzed with Kaplan-Meier analysis. Fine and Gray sub-distribution method was applied to quantify the cumulative incidence. RESULTS: Twenty-five patients underwent MelodyMVR. Median age and weight were 6.3 (4.4-15.2) months and 6.36 (4.41-7.57) kg. 60% had congenital mitral valve disease and 52% had dominant mitral regurgitation. The median diameter of the implanted Melody was 16 (14-18) mm. Mortality at 6 months, 1 year, and 5 years was 8.3% (95% CI, 2.2%-29.4%), 12.5% (4.2%-33.9%), and 17.6% (7.0%-40.7%), respectively. Two (8%) hospital survivors required early Melody replacement. Competing risk analysis showed that approximately 50% of patients underwent mechanical MVR by 3.5 years after MelodyMVR. Freedom from bleeding and thrombosis at 4 years was 87.5% (95%CI, 74.2%-100%). Eleven patients underwent mechanical MVR with no mortality. One (9%) required pacemaker implantation after mechanical MVR. CONCLUSIONS: MelodyMVR provides reasonable early and medium-term survival in small children and a high rate of successful bridge to mechanical MVR. MelodyMVR is associated with minimal pacemaker requirement, bleeding, and thrombosis. Early Melody functional deterioration necessitates early re-MVR, which can be achieved with minimal mortality and morbidity.

Bilateral remote ischemic conditioning in children: A two-center, double-blind, randomized controlled trial in young children undergoing cardiac surgery.

Objective: The study objective was to determine whether adequately delivered bilateral remote ischemic preconditioning is cardioprotective in young children undergoing surgery for 2 common congenital heart defects with or without cyanosis. Methods: We performed a prospective, double-blind, randomized controlled trial at 2 centers in the United Kingdom. Children aged 3 to 36 months undergoing tetralogy of Fallot repair or ventricular septal defect closure were randomized 1:1 to receive bilateral preconditioning or sham intervention. Participants were followed up until hospital discharge or 30 days. The primary outcome was area under the curve for high-sensitivity troponin-T in the first 24 hours after surgery, analyzed by intention-to-treat. Right atrial biopsies were obtained in selected participants. Results: Between October 2016 and December 2020, 120 eligible children were randomized to receive bilateral preconditioning (n = 60) or sham intervention (n = 60). The primary outcome, area under the curve for high-sensitivity troponin-T, was higher in the preconditioning group (mean: 70.0 ± 50.9 µg/L/h, n = 56) than in controls (mean: 55.6 ± 30.1 µg/L/h, n = 58) (mean difference, 13.2 µg/L/h; 95% CI, 0.5-25.8; P = .04). Subgroup analyses did not show a differential treatment effect by oxygen saturations (pinteraction = .25), but there was evidence of a differential effect by underlying defect (pinteraction = .04). Secondary outcomes and myocardial metabolism, quantified in atrial biopsies, were not different between randomized groups. Conclusions: Bilateral remote ischemic preconditioning does not attenuate myocardial injury in children undergoing surgical repair for congenital heart defects, and there was evidence of potential harm in unstented tetralogy of Fallot. The routine use of remote ischemic preconditioning cannot be recommended for myocardial protection during pediatric cardiac surgery.

Longitudinal Evaluation of Congenital Cardiovascular Surgical Performance and Skills Retention Using Silicone-Molded Heart Models.

Objective: Hands-on surgical training (HOST) for congenital heart surgery (CHS), utilizing silicone-molded models created from 3D-printing of patients' imaging data, was shown to improve surgical skills. However, the impact of repetition and frequency of repetition in retaining skills has not been previously investigated. We aimed to longitudinally evaluate the outcome for HOST on two example procedures of different technical difficulties with repeated attempts over a 15-week period. Methods: Five CHS trainees were prospectively recruited. Repair of coarctation of the aorta (CoA) and arterial switch operation (ASO) were selected as example procedures of relatively low and high technical difficulty. Procedural time and technical performance (using procedure-specific assessment tools by the participant, a peer-reviewer, and the proctor) were measured. Results: Coarctation repair performance scores improved after the first repetition but remained unchanged at the follow-up session. Likewise, CoA procedural time showed an early reduction but then remained stable (mean [standard deviation]: 29[14] vs 25[15] vs 23[9] min at 0, 1, and 4 weeks). Conversely, ASO performance scores improved during the first repetitions, but decreased after a longer time delay (>9 weeks). Arterial switch operation procedural time showed modest improvements across simulations but significantly reduced from the first to the last attempt: 119[20] versus 106[28] min at 0 and 15 weeks, P = .049. Conclusions: Complex procedures require multiple HOST repetitions, without excessive time delay to maintain long-term skills improvement. Conversely, a single session may be planned for simple procedures to achieve satisfactory medium-term results. Importantly, a consistent reduction in procedural times was recorded, supporting increased surgical efficiency.

Comparing apples to apples: Exploring public reporting of congenital cardiac surgery outcomes based on common congenital heart operations.

OBJECTIVE: We sought to simplify reporting of outcomes in congenital heart surgery that compares well-defined patient groups and accommodates multiple stakeholder needs while being easily understandable. METHODS: We selected 19 commonly performed congenital heart surgeries ranging in complexity from repair of atrial septal defects to the Norwood procedure. Strict inclusion/exclusion criteria ensured the creation of 19 well-defined diagnosis/procedure cohorts. Preoperative, procedural, and postoperative data were collected for consecutive eligible patients from 9 centers between January 1, 2016, and December 31, 2021. Unadjusted operative mortality rates and hospital length of stay for each of the 19 diagnosis/procedure cohorts were summarized in aggregate and stratified by each center. RESULTS: Of 8572 eligible cases included, numbers in the 19 diagnosis/procedure cohorts ranged from 73 for tetralogy of Fallot repair after previous palliation to 1224 for ventricular septal defect (VSD) repair for isolated VSD. In aggregate, the unadjusted mortality ranged from 0% for atrial septal defect repair to 28.4% for hybrid stage I. There was significant heterogeneity in case mix and mortality for different diagnosis/procedure cohorts across centers (eg, arterial switch operation/VSD, n = 7-42, mortality 0%-7.4%; Norwood procedure, n = 16-122, mortality 5.3%-25%). CONCLUSIONS: Reporting of institutional case volumes and outcomes within well-defined diagnosis/procedure cohorts can enable centers to benchmark outcomes, understand trends in mortality, and direct quality improvement. When made public, this type of report could provide parents with information on institutional volumes and outcomes and allow them to better understand the experience of each program with operations for specific congenital heart defects.

Incremental value of machine learning for risk prediction in tetralogy of Fallot.

OBJECTIVE: Machine learning (ML) can facilitate prediction of major adverse cardiovascular events (MACEs) in repaired tetralogy of Fallot (rTOF). We sought to determine the incremental value of ML above expert clinical judgement for risk prediction in rTOF. METHODS: Adult congenital heart disease (ACHD) clinicians (≥10 years of experience) participated (one cardiac surgeon and four cardiologists (two paediatric and two adult cardiology trained) with expertise in heart failure (HF), electrophysiology, imaging and intervention). Clinicians identified 10 high-yield variables for 5-year MACE prediction (defined as a composite of mortality, resuscitated sudden death, sustained ventricular tachycardia and HF). Risk for MACE (low, moderate or high) was assigned by clinicians blinded to outcome for adults with rTOF identified from an institutional database (n=25 patient reviews conducted by five independent observers). A validated ML model identified 10 variables for risk prediction in the same population. RESULTS: Prediction by ML was similar to the aggregate score of all experts (area under the curve (AUC) 0.85 (95% CI 0.58 to 0.96) vs 0.92 (0.72 to 0.98), p=0.315). Experts with ≥20 years of experience had superior discriminative capacity compared with <20 years (AUC 0.98 (95% CI 0.86 to 0.99) vs 0.80 (0.56 to 0.93), p=0.027). In those with <20 years of experience, ML provided incremental value such that the combined (clinical+ML) AUC approached ≥20 years (AUC 0.85 (95% CI 0.61 to 0.95), p=0.055). CONCLUSIONS: Robust prediction of 5-year MACE in rTOF was achieved using either ML or a multidisciplinary team of ACHD experts. Risk prediction of some clinicians was enhanced by incorporation of ML suggesting that there may be incremental value for ML in select circumstances.

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction.

BACKGROUND: Hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) is an initial management strategy for infants with critical left heart obstruction and serves as palliation until subsequent operations are pursued. OBJECTIVES: This study sought to determine patient characteristics and factors associated with subsequent outcomes for infants who underwent hybrid palliation. METHODS: From 2005 to 2019, 214 of 1,236 prospectively enrolled infants within the Congenital Heart Surgeons' Society's critical left heart obstruction cohort underwent hybrid palliation across 24 institutions. Multivariable hazard modeling with competing risk methodology was performed to determine risk and factors associated with outcomes of biventricular repair, Fontan procedure, transplantation, or death. RESULTS: Preoperative comorbidities (eg, prematurity, low birth weight, genetic syndrome) were identified in 70% of infants (150 of 214). Median follow-up was 7 years, ranging up to 17 years. Overall 12-year survival was 55%. At 5 years after hybrid palliation, 9% had biventricular repair, 36% had Fontan procedure, 12% had transplantation, 35% died without surgical endpoints, and 8% were alive without an endpoint. Factors associated with transplantation were absence of ductal stent, older age, absent interatrial communication, smaller aortic root size, larger tricuspid valve area z-score, and larger left ventricular volume. Factors associated with death were low birth weight, concomitant genetic syndrome, cardiopulmonary bypass use during hybrid palliation, moderate to severe tricuspid valve regurgitation, and smaller ascending aortic size. CONCLUSIONS: Mortality remains high after hybrid palliation for infants with critical left heart obstruction. Nonetheless, hybrid palliation may facilitate biventricular repair for some infants and for others may serve as stabilization for intended functional univentricular palliation or primary transplantation.

Machine Learning for Prediction of Adverse Cardiovascular Events in Adults With Repaired Tetralogy of Fallot Using Clinical and Cardiovascular Magnetic Resonance Imaging Variables.

BACKGROUND: Existing models for prediction of major adverse cardiovascular events (MACE) after repair of tetralogy of Fallot have been limited by modest predictive capacity and limited applicability to routine clinical practice. We hypothesized that an artificial intelligence model using an array of parameters would enhance 5-year MACE prediction in adults with repaired tetralogy of Fallot. METHODS: A machine learning algorithm was applied to 2 nonoverlapping, institutional databases of adults with repaired tetralogy of Fallot: (1) for model development, a prospectively constructed clinical and cardiovascular magnetic resonance registry; (2) for model validation, a retrospective database comprised of variables extracted from the electronic health record. The MACE composite outcome included mortality, resuscitated sudden death, sustained ventricular tachycardia and heart failure. Analysis was restricted to individuals with MACE or followed ≥5 years. A random forest model was trained using machine learning (n=57 variables). Repeated random sub-sampling validation was sequentially applied to the development dataset followed by application to the validation dataset. RESULTS: We identified 804 individuals (n=312 for development and n=492 for validation). Model prediction (area under the curve [95% CI]) for MACE in the validation dataset was strong (0.82 [0.74-0.89]) with superior performance to a conventional Cox multivariable model (0.63 [0.51-0.75]; P=0.003). Model performance did not change significantly with input restricted to the 10 strongest features (decreasing order of strength: right ventricular end-systolic volume indexed, right ventricular ejection fraction, age at cardiovascular magnetic resonance imaging, age at repair, absolute ventilatory anaerobic threshold, right ventricular end-diastolic volume indexed, ventilatory anaerobic threshold % predicted, peak aerobic capacity, left ventricular ejection fraction, and pulmonary regurgitation fraction; 0.81 [0.72-0.89]; P=0.232). Removing exercise parameters resulted in inferior model performance (0.75 [0.65-0.84]; P=0.002). CONCLUSIONS: In this single-center study, a machine learning-based prediction model comprised of readily available clinical and cardiovascular magnetic resonance imaging variables performed well in an independent validation cohort. Further study will determine the value of this model for risk stratification in adults with repared tetralogy of Fallot.

Medium-term Outcome of Prenatally Diagnosed Hypoplastic Left-Heart Syndrome and Impact of a Restrictive Atrial Septum Diagnosed in-utero.

OBJECTIVE: Surgical outcome data differs from overall outcomes of prenatally diagnosed fetuses with hypoplastic left heart syndrome (HLHS). Our aim was to describe outcome of prenatally diagnosed fetuses with this anomaly. METHODS: Retrospective review of prenatally diagnosed classical HLHS at a tertiary hospital over a 13-year period, estimated due dates 01/08/2006 to 31/12/2019. HLHS-variants and ventricular disproportion were excluded. RESULTS: 203 fetuses were identified with outcome information available for 201. There were extra-cardiac abnormalities in 8% (16/203), with genetic variants in 14% of those tested (17/122). There were 55 (27%) terminations of pregnancy, 5 (2%) intrauterine deaths and 10 (5%) babies had prenatally planned compassionate care. There was intention to treat (ITT) in the remaining 131/201(65%). Of these, there were 8 neonatal deaths before intervention, two patients had surgery in other centers. Of the other 121 patients, Norwood procedure performed in 113 (93%), initial hybrid in 7 (6%), and 1 had palliative coarctation stenting. Survival for the ITT group from birth at 6-months, 1-year and 5-years was 70%, 65%, 62% respectively. Altogether of the initial 201 prenatally diagnosed fetuses, 80 patients (40%) are currently alive. A restrictive atrial septum (RAS) is an important sub-category associated with death, HR 2.61, 95%CI 1.34-5.05, p = 0.005, with only 5/29 patients still alive. CONCLUSION: Medium-term outcomes of prenatally diagnosed HLHS have improved however it should be noted that almost 40% do not get to surgical palliation, which is vital to those doing fetal counselling. There remains significant mortality particularly in fetuses with in-utero diagnosed RAS.

Hybrid palliation versus nonhybrid management for a multi-institutional cohort of infants with critical left heart obstruction.

OBJECTIVE: To compare patient characteristics and overall survival for infants with critical left heart obstruction after hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) versus nonhybrid management (eg, Norwood, primary transplantation, biventricular repair, or transcatheter/surgical aortic valvotomy). METHODS: From 2005 to 2019, 1045 infants in the Congenital Heart Surgeons' Society critical left heart obstruction cohort underwent interventions across 28 institutions. Using a balancing score propensity analysis, 214 infants who underwent hybrid palliation and 831 infants who underwent nonhybrid management were proportionately matched regarding variables significantly associated with mortality and variables noted to significantly differ between groups. Overall survival between the 2 groups was adjusted by applying balancing scores to nonparametric estimates. RESULTS: Compared with the nonhybrid management group, infants who underwent hybrid palliation had lower birth weight, smaller gestational age, and higher prevalence of in-utero interventions, noncardiac comorbidities, preoperative mechanical ventilation, absent interatrial communication, and moderate or severe mitral valve stenosis (all P values < .03). Unadjusted 12-year survival after hybrid palliation and nonhybrid management, was 55% versus 69%, respectively. After matching, 12-year survival after hybrid palliation versus nonhybrid management was 58% versus 63%, respectively (P = .37). Among matched infants born weighing <2.5 kg, 2-year survival after hybrid palliation versus nonhybrid management was 37% versus 51%, respectively (P = .22). CONCLUSIONS: Infants born with critical left heart obstruction who undergo hybrid palliation have more high-risk characteristics and anatomy versus infants who undergo nonhybrid management. Nonetheless, after adjustment, there was no significant difference in 12-year survival after hybrid palliation versus nonhybrid management. Mortality remains high, and hybrid palliation confers no survival advantage, even for lower-birth-weight infants.

Outcomes of the arterial switch for transposition during infancy using a standardized approach over 30 years.

OBJECTIVES: The aim of this study was to describe the early and late outcomes of the arterial switch for transposition. METHODS: A single-centre retrospective cohort study was conducted to assess the early and late outcomes of arterial switch performed during infancy using a standardized institutional approach between 1988 and 2018, compared by morphological groups. RESULTS: A total of 749 consecutive patients undergoing arterial switch during infancy were included, 464 (61.9%) with intact septum, 163 (21.8%) with isolated ventricular septal defect and 122 (16.3%) with complex transposition with associated lesions, including 67 (8.9%) with Taussig-Bing anomaly. There were 34 early deaths [4.5%, 95% confidence interval (CI) 3.1-6.1] with only 10 (2.6%) early deaths since 2000. Complex morphology (odds ratio 11.44, 95% CI 4.76-27.43) and intramural coronary artery (odds ratio 5.17, 95% CI 1.61-15.91) were identified as the most important risk factors for 90-day mortality. Overall survival was 92.7% (95% CI 90.8-94.6) at 5 years and 91.9% (95% CI 89.9-94.1) at 20 years; in hospital survivors, there were 15 (2.1%) late deaths during a median follow-up of 13.7 years. Cumulative incidence of surgical or catheter reintervention was 16.0% (95% CI 14.5-17.5) at 5 years and 22.7% (95% CI 21.0-24.0) at 20 years; early and late reinterventions were more common in the complex group, with no difference between the other groups. CONCLUSIONS: Using a standardized approach, the arterial switch can be performed with low early mortality, moderate rates of reintervention and excellent long-term survival. Concomitant lesions were the most important risk factor for early death and were associated with increased risk of late reintervention.

Training on Congenital 3D Cardiac Models - Will Models Improve Surgical Performance?

Technical skill development in congenital heart surgery (CHS) is challenging due to numerous factors which potentially limit the hands-on operative exposure in surgical training. These challenges have stimulated the growth of simulation-based training through the development of 3D-printed models, providing hands-on surgical training (HOST). From its inception in 2015, the models used in the HOST program have constantly improved, and now include valvar/subvalvar apparatus and better materials that mimic real tissue. Evidence shows that deliberate, regular simulation practice can improve a surgeon's technical skills across the spectrum of CHS. Furthermore, surgical trainees who undergo simulation training are able to translate this improved performance into the operative environment with improved patient outcomes. Despite evidence to support the incorporation of simulation methods into congenital training, its widespread adoption into training curricula remains low. This is due to numerous factors including funding, lack of dedicated time or proctorship and access to models-all of which can be overcome with the newer generation of models and committed trainers. Training programs should consider incorporating simulation-methods as a routine component of congenital training programs.

Norwood operation versus comprehensive stage II after bilateral pulmonary artery banding palliation for infants with critical left heart obstruction.

OBJECTIVE: To determine patient characteristics and outcomes after Norwood versus comprehensive stage II (COMPSII) for infants with critical left heart obstruction who had prior hybrid palliation (bilateral pulmonary artery banding ± ductal stent). METHODS: From 23 Congenital Heart Surgeons' Society institutions (2005-2020), 138 infants underwent hybrid palliation followed by either Norwood (n = 73, 53%) or COMPSII (n = 65). Baseline characteristics were compared between Norwood and COMPSII groups. Parametric hazard model with competing risk methodology was used to determine risk and factors associated with outcomes of Fontan, transplantation, or death. RESULTS: Infants who underwent Norwood versus COMPSII had a higher prevalence of prematurity (26% vs 14%, P = .08), lower birth weight (median 2.8 vs 3.2 kg, P < .01) and less frequent ductal stenting (37% vs 99%; P < .01). Norwood was performed at a median age of 44 days and median weight of 3.5 kg, versus COMPSII at 162 days and 6.0 kg (both P < .01). Median follow-up was 6.5 years. At 5 years after Norwood and COMPSII, respectively; 50% versus 68% had Fontan (P = .16), 3% versus 5% had transplantation (P = .70), 40% versus 15% died (P = .10), and 7% versus 11% are alive without transition, respectively. For factors associated with either mortality or Fontan, only preoperative mechanical ventilation occurred more frequently in the Norwood group. CONCLUSIONS: Higher prevalence of prematurity, lower birth weight, and other patient-related characteristics in the Norwood versus COMPSII groups may influence differences in outcomes that were not statistically significant for this limited risk-adjusted cohort. The clinical decision regarding Norwood versus COMPSII after initial hybrid palliation remains challenging.

The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot.

OBJECTIVE: Despite decades of experience, aspects of the management of tetralogy of Fallot with pulmonary stenosis (TOF) remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision making. Therefore, the TOF Clinical Practice Standards Committee was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. In addition, the group was tasked with identifying pertinent research questions for future investigations. It is recognized that variability in institutional experience could influence the application of this framework to clinical practice. METHODS: The TOF Clinical Practice Standards Committee is a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF. With the assistance of a medical librarian, a citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to TOF and its management; the search was restricted to the English language and the year 2000 or later. Articles pertaining to pulmonary atresia, absent pulmonary valve, atrioventricular septal defects, and adult patients with TOF were excluded, as well as nonprimary sources such as review articles. This yielded nearly 20,000 results, of which 163 were included. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of the member votes with 75% agreement on each statement. RESULTS: In asymptomatic infants, complete surgical correction between age 3 and 6 months is reasonable to reduce the length of stay, rate of adverse events, and need for a transannular patch. In the majority of symptomatic neonates, both palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities such as intracranial hemorrhage, sepsis, or other end-organ compromise as high-risk patients. In these high-risk patients, palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical palliation. CONCLUSIONS: Ongoing research will provide further insight into the role of catheter-based interventions. For complete surgical correction, both transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be utilized. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention. Given our current knowledge and the gaps identified, we propose several key questions to be answered by future research and potentially by a TOF registry: When to palliate or proceed with complete surgical correction, as well as the ideal type of palliation; the optimal surgical approach for complete repair for the best long-term preservation of right ventricular function; and the utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques.

Quantification of lymphatic burden in patients with Fontan circulation by T2 MR lymphangiography and associations with adverse Fontan status.

AIMS: To quantify thoracic lymphatic burden in paediatric Fontan patients using MRI and correlate with clinical status. METHODS AND RESULTS: Paediatric Fontan patients (<18-years-old) with clinical cardiac MRI that had routine lymphatic 3D T2 fast spin echo (FSE) imaging performed from May 2017 to October 2019 were included. 'Lymphatic burden' was quantified by thresholding-based segmentation of the 3D T2 FSE maximum intensity projection image and indexed to body surface area, performed by two independent readers blinded to patient status. There were 48 patients (27 males) with median age at MRI of 12.9 (9.4-14.7) years, time from Fontan surgery to MRI of 9.1 (5.9-10.4) years, and follow-up time post-Fontan surgery of 9.4 (6.6-11.0) years. Intraclass correlation coefficient between two observers for lymphatic burden was 0.96 (0.94-0.98). Greater lymphatic burden correlated with post-Fontan operation hospital length of stay and duration of chest tube drainage (rs = 0.416, P = 0.004 and rs = 0.439, P = 0.002). Median lymphatic burden was greater in patients with chylous effusions immediately post-Fontan (178 (118-393) vs. 113 (46-190) mL/m2, P = 0.028), and in patients with composite adverse Fontan status (n = 13) defined by heart failure (n = 3), transplant assessment (n = 2), recurrent effusions (n = 6), Fontan thrombus (n = 2), and/or PLE (n = 6) post-Fontan (435 (137-822) vs. 114 (51-178) mL/m2, P = 0.003). Lymphatic burden > 600 mL/m2 was associated with late adverse Fontan status with sensitivity of 57% and specificity of 95%. CONCLUSION: Quantification of MR lymphatic burden is a reliable tool to assess the lymphatics post-Fontan and is associated with clinical status.

Growth of unrepaired hypoplastic proximal aortic arch and reintervention rate after aortic coarctation repair.

BACKGROUND: Treatment of tubular hypoplasia of the aortic arch (THAA) associated with aortic coarctation (CoA) remains controversial. We aimed to evaluate growth of unrepaired hypoplastic proximal aortic arches (PAAs) after surgical repair for CoA. METHODS: Preoperative and follow-up echocardiographic images of 139 patients who underwent CoA repairs from 2005 to 2012 were reviewed. THAA was defined as PAA z-score <-3 and non-THAA group z-score ≥-3. Reintervention rates due to aortic obstruction were assessed using competing risk models and diameters of the aorta were compared with Mann-Whitney U tests. RESULTS: Fifty patients (36%) had THAA and 89 (64%) had non-THAA. The survival rate was 94% at 10 years. The overall reintervention rate at 10 years was 9% in the THAA group and 16% in the non-THAA group (P = .54). The catheter reintervention rate at ten years was 2% in the THAA group and 16% in the non-THAA group (P = .031). The surgical reintervention rate at ten years was 7% in the THAA group and 0% in the non-THAA group (P = .016). All 4 patients who required surgical reintervention were in the THAA group and 3 patients with PAA obstruction had preoperative PAA z-scores -3.6, -4.2, and -4.3. Follow-up echocardiograms showed PAA catch-up growth in the THAA group compared with the non-THAA group (preoperative z-score of -3.6 vs -2.3, and at 7 years of -1.1 vs -1.2; P < .001). CONCLUSIONS: Unrepaired PAA hypoplasia grows after CoA repair. Reintervention rates were comparable between groups but those with THAA had higher surgical reintervention rates.

Optimal Surgical Management of Tetralogy of Fallot.

Tetralogy of Fallot with pulmonary stenosis has a diverse clinical spectrum with the degree of right ventricular outflow tract obstruction (RVOTO) and size of the branch pulmonary arteries driving clinical management. Optimal surgical management involves consideration of patient clinical status and degree and location (subvalvar, valvar, and supravalvar) of RVOTO. Timing of repair requires multidisciplinary decision-making and complete surgical repair with relief of RVOTO by either transannular patch or valve sparing repair techniques. The central goals of contemporary surgical management of tetralogy of Fallot incorporate maximizing survival, minimizing reintervention, and preserving right ventricular function across the lifespan.

En contexte de tétralogie de Fallot (TF) avec sténose pulmonaire (SP), le degré d'obstruction à l'éjection du sang par le ventricule droit (OESVD) et le diamètre des branches des artères pulmonaires se caractérisent par divers tableaux cliniques et motivent la prise en charge clinique. Pour une prise en charge chirurgicale optimale, le statut clinique du patient ainsi que le degré et l'emplacement de l'OESVD (avant, après ou au niveau de la valve) doivent être pris en considération. Le moment de l'intervention chirurgicale doit être décidé par une équipe multidisciplinaire, et la technique de greffe en pièce transannulaire ou une approche avec conservation de la valve doit être utilisée pour réaliser une réparation complète. Les objectifs principaux de la prise en charge chirurgicale actuelle de la TF sont de maximiser la survie, de limiter les réinterventions chirurgicales et de préserver la fonction du ventricule droit pour toute la vie du patient.