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Severe left outflow tract obstruction (LVOTO) is not always associated with hypoplastic left heart syndrome (HLHS). Aortic valvar atresia or complex LVOTO in the presence of a large ventricular septal defect (VSD) are a rare group of lesions that offer the possibility of biventricular repair. The Yasui procedure is the commonest surgical approach which can be performed as a primary neonatal correction or as a staged procedure with a Norwood followed by a subsequent Rastelli. This article reviews the surgical outcomes and decision-making process. Both strategies are reasonable with the trend toward staged procedure in the setting of the additional interrupted arch, with neonatal survival of > 90% in the modern era and excellent long-term survival. Re-intervention is mostly related to conduit revision and the need for re-operation for LVOTO is rare. Deciding between conventional repair and the Yasui in cases of LVOTO/VSD can be difficult and there are no uniform accepted criteria. In a typical neonate, an aortic annulus < 4.5 mm is generally the limit of acceptability for a conventional repair. In selected cases of LVOTO/VSD, an alternative to the Yasui is the Ross-Konno. Retrospective comparisons between the 2 techniques are difficult due to differing patient characteristics (especially associated with mitral valve disease) but the neonatal Ross has been associated with higher early mortality.

Background and AimsPrevious trials evaluating remote ischaemic preconditioning in children undergoing cardiac surgery showed mixed results. We sought to determine whether adequately delivered bilateral preconditioning is cardioprotective in young children, with or without cyanosis, undergoing surgery.MethodsProspective, double-blind, randomised controlled trial at two UK centres. Children aged 3-36 months undergoing tetralogy of Fallot repair or ventricular septal defect closure were randomised in a 1:1 ratio to receive either 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, analysed by intention-to-treat. Right atrial biopsies were obtained in selected patients. Trial registration:ISRCTN12923441.ResultsBetween 24 October 2016 and 8 December 2020, 120 eligible children were randomised to receive either bilateral preconditioning (n=60) or sham intervention (n=60). Participants had a median age of 7 months and 42 (35%) were female. The primary outcome, area under the curve for hs-troponin-T was higher in the preconditioning group (mean: 70.0±50.9µg/L/hr, n=56) than in controls (mean: 55.6±30.1µg/L/hr, n=58), p=0.04. Sub-group analyses did not show a differential treatment effect by oxygen saturations (pinteraction=0.25) but showed evidence of differential treatment effect by underlying defect (pinteraction=0.04). Myocardial metabolism, quantified in atrial biopsies, and secondary outcomes were not different between randomised groups.ConclusionsBilateral 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.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

Aims To quantify thoracic lymphatic burden in paediatric Fontan patients using MRI and correlate with clinical status. Methods and results Paediatric Fontan patients ( 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.

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown cause. We aimed to better understand familial recurrence patterns. Methods: An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, entailing investigation of 1043 unrelated ccTGA probands. Results: Laterality defects and atrioventricular block at diagnosis were observed in 29.9% and 9.3%, respectively. ccTGA was associated with primary ciliary dyskinesia in 11 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (28.4%), laterality defects (13.6%), and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and dextro-transposition of the great arteries, and 9 familial co-segregation of ccTGA and laterality defects. In one family co-segregation of ccTGA, dextro-transposition of the great arteries and heterotaxy syndrome in 3 distinct relatives was found. In another family, twins both displayed ccTGA and primary ciliary dyskinesia. Conclusions: ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, dextro-transposition of the great arteries, laterality defects and in some cases primary ciliary dyskinesia, strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

OBJECTIVES The hands-on surgical training course utilizes 3-dimensional (3D)-printed heart models to simulate complex congenital heart operations. This study aimed to validate a model and assessment tool in the simulation of 2 techniques of the Norwood operation and investigate whether technical performance improves following rehearsal with or without proctor presence. METHODS Five ‘experienced’ and 5 ‘junior’ surgeons performed 2 techniques of the Norwood operation on 3D-printed models of hypoplastic left heart syndrome. Performances were retrospectively assessed by 10 raters with varying experience in congenital heart surgery (CHS) (Medical Doctorate versus non-Medical Doctorate). Assessments were made with the procedure-specific Hands-On Surgical Training-CHS tool. Results were analysed for technical performance and rater consistency. Following validation, 30 surgeons (24 with proctor guidance and 6 with training videos and objective feedback only) simulated the Norwood operation twice. RESULTS Performance scores were consistently higher for experienced surgeons and raters discriminated clearly between the experienced and junior surgeons (P ≤ 0.001). The hands-on surgical training-CHS tool showed high inter-rater (0.86) and intra-rater (0.80) reliability among all raters. Scores for both experienced and junior surgeons were highly consistent across all raters, with no statistically significant difference (P = 0.50). All surgeons successfully performed the Norwood operation. Sixty attempts were scored in total. Eighty-seven percentage (26/30) of surgeons’ scores (mean: attempt 1 = 92, attempt 2 = 104) and times [mean: attempt 1 = 1:22:00, attempt 2 = 1:08:00 (h:mm:ss)] improved between the 2 attempts by 9% and 15% respectively (P ≤ 0.001). Total scores of all surgeons in the non-proctored subgroup (6) improved by 15% on average (mean: attempt 1 = 86, attempt 2 = 105, P = 0.002). CONCLUSIONS Procedure-specific assessment tools can be developed to evaluate technical performance for complex CHS simulation and be performed reliably by non-expert raters. Rehearsal both under supervision and independently leads to technical skill improvement further supporting its value in CHS training.

Interventional cardiology has made extraordinary advances over recent years, but most are still limited to addressing single intracardiac or valvular lesions. This debate considers whether complete interventional repair of more complex congenital defects might become achievable. Tetralogy of Fallot (ToF) is probably the first candidate where complete interventional repair might be achieved-given that various components of the defect have already been successfully addressed-albeit as either a palliative intervention (RVOT stenting) or to address the sequelae of standard surgery (percutaneous PVR). This article considers the challenges that would need to be overcome in terms of the morphology of the condition, the age limitations, and the necessary technological advancements that would be required-while setting these against the benchmark of current surgical outcomes and the parallel progress that is being developed in surgical correction. While complete interventional repair of ToF may still be beyond current techniques, a hybrid approach between surgeons and intentional cardiologists can strive to create a life-long paradigm of care that minimizes the need for surgery and focuses on the maintenance of a healthy right ventricle, such that patients born with ToF can achieve normal life expectancy.

Anecdotal evidence suggests that futsal can aid talent development for 11 a-side soccer through the potential for transfer of the game's skills and constraints. However, additional scientific research is necessary to enhance understanding of the technical and physical demands of futsal and the potential returns for soccer player development. Accordingly, this study examined selected characteristics of physical (internal and external loads) and technical performance in international futsal match-play in order to study the potential of these for skill transfer and aiding soccer player development. Performance was investigated in futsal players (n = 16, 25.74±4.71 years) belonging to a national team during an international tournament. Pre-tournament fitness testing determined maximum heart rate (MHR) via the Yo-Yo IR1 test (194.6±11.1 beats min ) to aid interpretation of internal load via heart rate measures during match-play. External load (accelerations and deceleration events) was measured using an inertial movement unit. Finally, post-tournament analysis of selected technical events was performed. Results reported a mean heart rate value during 'court time' of 164.7±22.3 beats min , which as a percentage of participants' MHR was 87.7%±4.4%, and a mean peak MHR of 98.3±2.5%. Results showed 2.16±0.25 accelerating (>1.5 m·s ) and 2.78±0.13 decelerating (>1.5 m·s ) events per player per minute. Team statistics included 647 passing, ~51 dribbling and 78 set play events per team per game. Match analyses showed that 77.3% of ball receptions were completed with the sole of the foot. Assessment of two-footedness showed 80.1±16.7% of individual possessions used the dominant foot to receive and 84.1±10.7% to pass the ball thereby displaying strong foot dominance. These results have quantified characteristics of elite futsal match-play that are no doubt intrinsically related to the environmental and task constraints of the sport. In addition to enhancing understanding of futsal, there are potential learning returns and implications for skills transfer and the development of soccer players. [Abstract copyright: Copyright © Biology of Sport 2020.]

No area of congenital heart disease has undergone greater change and innovation than single ventricle management over the past 20 years. Surgical and catheterisation laboratory interventions have transformed outcomes such that in some subgroups more than 80% of the patients can survive into adulthood. Driven by parallel development in diagnostic imaging and cardiac intensive care, surgical management is focused on the neonatal period as the key time to creating a balanced circulation and limiting pulmonary blood flow. Different configurations of the circulation, including new types of surgical shunts, and the role of "hybrid" circulations provide greater options and better physiology. This overview focuses on these changes in surgical management and timing, but also looks at the exciting areas of regenerative therapies to improve ventricular function, and the concept of ventricular rehabilitation to achieve biventricular circulations in certain groups of patients. The importance of early (neonatal) intervention and multidisciplinary approach to management is emphasised, as well as looking beyond simply survival to also improving neurodevelopmental outcomes.

Short-term survival after paediatric cardiac surgery has improved significantly over the past 20 years and increasing attention is being given to measuring and reducing incidence of morbidities following surgery. How to best use routinely collected data to share morbidity information constitutes a challenge for clinical teams interested in analysing their outcomes for quality improvement. We aimed to develop a tool facilitating this process in the context of monitoring morbidities following paediatric cardiac surgery, as part of a prospective multi-centre research study in the United Kingdom.We developed a prototype software tool to analyse and present data about morbidities associated with cardiac surgery in children. We used an iterative process, involving engagement with potential users, tool design and implementation, and feedback collection. Graphical data displays were based on the use of icons and graphs designed in collaboration with clinicians.Our tool enables automatic creation of graphical summaries, displayed as a Microsoft PowerPoint presentation, from a spreadsheet containing patient-level data about specified cardiac surgery morbidities. Data summaries include numbers/percentages of cases with morbidities reported, co-occurrences of different morbidities, and time series of each complication over a time window.Our work was characterised by a very high level of interaction with potential users of the tool, enabling us to promptly account for feedback and suggestions from clinicians and data managers. The United Kingdom centres involved in the project received the tool positively, and several expressed their interest in using it as part of their routine practice.

The Fontan circulation has inherent long-term vulnerabilities such that adult Fontan patients now comprise the largest, most rapidly growing subgroup of adult congenital heart disease referred for transplant assessment. Almost all have Fontan Associated Liver Disease (FALD). There is an absence of mid to late hepatic outcome data after heart transplant alone . Therefore, we analyzed outcomes of survivors of heart only transplant in patients with failing Fontan circulation. Including all ten of our adult Fontan patients surviving >1 year after isolated heart transplant, we report evolution of their clinical features, bloodwork, hemodynamic data and liver ultrasound findings over a median of 4.7 years. Non-progression of FALD, resolution of ascites and freedom from hepatocellular carcinoma in the mid-term highlight the outcomes in this selected group once normal cardiac output and venous pressures are established by heart transplant.

Background The management of pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) has significantly changed over the past 20 years. Unifocalization and rehabilitation have been described as diametrically opposed strategies. An updated review focused on the management of this complex and rare condition is needed. Methods Articles related to PA/VSD/MAPCAs issued until December 2017 were screened. Twelve main studies published in the modern era (since 2000) were selected and analyzed. Results Unifocalization and rehabilitation respectively focus on the mobilization of collateral arteries and the growth of native pulmonary vessels. A third strategy, called "combined strategy," was distinguished from the review of the literature. Surgical cohorts and methods of data reporting were found to be heterogenous. Outcomes, regardless of the strategy, have transformed the natural history of the condition, with a complete repair rate of approximately 80% and low rates of early and late mortality. Patients with the most unfavorable anatomy (absent central pulmonary arteries and hypoplastic MAPCAs) remain a challenge and are still left palliated. Conclusions Variable surgical strategies are used in the management of PA/VSD/MAPCAs. Most teams report a repair rate of 70% to 80% and a mortality rate lower than 10%. Standardization in data reporting is necessary to better compare the existing strategies.

The concept of 're-training' the morphologic left ventricle (mLV) is based on the crude principle of applying a fixed afterload by means of pulmonary artery banding. The complex physiological, molecular and structural responses to banding are poorly understood, and complicated by the fact that re-training is undertaken in a variety of different morphological settings and age-groups. This article reviews the evidence for re-training in different situations with particular focus on the age at banding and on the best ways to assess suitability for subsequent repair. Particular importance is placed on the role of re-training in congenitally corrected transposition as this is the commonest current indication - looking at better ways to train the mLV, the role of combined pressure and volume loading, and more sophisticated ways of assessing the adequacy of training. Current evidence suggests that age at banding has a fundamental impact on ability to re-train and long-term mLV function with the best results being achieved in infancy and concerns at any attempt beyond 2 years of age.

There are a number of techniques used in the surgical treatment of supravalvular aortic stenosis with excellent results; however, junior surgeons’ experience may be limited due to its rare nature. Simulation with 3D-printed models provides a safe platform for surgeons to rehearse surgical techniques prior to patient application but must replicate all features of surgical anatomy. Here, we describe how software manipulation can be used to accurately reproduce the surgical anatomy in supravalvular aortic stenosis and be used to train surgeons in 2 different techniques.

BACKGROUND The debate between the best therapeutic approach to congenital aortic stenosis is ongoing. Multiple studies have attempted to answer this question without reaching a definitive conclusion. McCrindle et al performed the landmark analysis of the congenital heart surgeon database and they showed equivalent results in the outcome between surgical aortic valvotomy (SAV) versus balloon aortic valvuloplasty (BAV). Given similar outcomes, many centers preferentially perform BAV due to its minimally invasive nature and faster recovery. Some more recent single-center studies have shown better outcomes with surgical valvotomy; however, their results were attributed to the use of contemporary superior surgical techniques. In this study, we perform a contemporary systematic review and meta-analysis to compare the effects of BAV and SAV on patient morbidity and mortality. METHODS AND RESULTS We searched MEDLINE and EMBASE from inception to March 2021 for studies comparing balloon aortic valvuloplasty versus surgical commissurotomy for critical congenital AS in patients younger than 18. We performed title and abstract screening, full-text assessment, risk of bias assessment using the CLARITY tool, and data collection independently and in duplicate. We pooled data using the random-effects model and Mantel-Haensel statistical method. We evaluated the overall quality of evidence using the GRADE framework. From a total of 439 studies imported for screening, 12 studies (1214 participants) were included that compared outcomes for balloon aortic valvuloplasty and surgical commissurotomy in the pediatric population. Mortality at 30-day and at longest follow-up was not statistically significant between the two groups (RR 0.66 95% CI [0.34, 1.27] and 0.74 95% CI [0.35, 1.56]. Reintervention favoured surgical repair at the longest follow-up but did not reach statistical significance (RR 1.11 95% CI [0.89, 1.38]). Postprocedural aortic insufficiency was more common after balloon valvotomy but failed to reach statistical significance (RR: 1.5 95% CI 0.99 – 2.99). CONCLUSION In pediatric patients with congenital AS, surgical commissurotomy did not offer additional advantages over balloon valvotomy. There was a trend toward an increase in post-procedural AI and an increased rate of reintervention with balloon valvotomy. However, the quality of evidence is very low. An appropriately powered large study is required to adequately address the risks and benefits of the two interventions.

Introduction Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown aetiology. We aimed to better understand familial recurrence pattern. Methods An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, leading to investigate 1043 unrelated ccTGA probands. Results Atrioventricular block at diagnosis and laterality defects were observed in 35.4% and 29.9%, respectively. ccTGA associated with primary ciliary dyskinesia in 10 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (d-TGA: 28.4%), laterality defects (13.6%) and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and d-TGA and 9 familial co-segregation of ccTGA and laterality defects. In one family there was co-segregation of ccTGA, d-TGA and heterotaxy syndrome in 3 distinct relatives. Conclusion ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, d-TGA, laterality defects and in some cases primary ciliary dyskinesia strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.

During repair of atrioventricular septal defect (AVSD), surgeons might leave an atrial level shunt when concerned about postoperative physiology, or as part of routine practice. However, the association of fenestration with outcomes is unclear. We sought to determine factors associated with mortality after biventricular repair of AVSD.We included 581 patients enrolled from 32 Congenital Heart Surgeons' Society institutions from January 1, 2012, to June 1, 2020 in the Congenital Heart Surgeons' Society AVSD cohort. Parametric multiphase hazard analysis was used to identify factors associated with mortality. A random effect model was used to account for possible intersite variability in mortality.An atrial fenestration was placed during repair in 133/581 (23%) patients. Overall 5-year survival after repair was 91%. Patients who had fenestration had an 83% 5-year survival versus 93% for those not fenestrated (P .001). Variables associated with mortality in multivariable hazard analysis included institutional diagnosis of ventricular unbalance (hazard ratio [HR], 2.7 [95% confidence interval (CI): 1.5-4.9]; P = .003), preoperative mechanical ventilation (HR, 4.1 [95% CI, 1.3-13.1]; P = .02), atrial fenestration (HR, 2.8 [95% CI, 1.5-4.9]; P .001), and reoperation for ventricular septal defect (HR, 4.0 [95% CI, 1.3-13.1]; P = .002). There was no difference in measures of ventricular unbalance for comparisons of fenestrated with nonfenestrated patients. No significant interinstitution variability in mortality was observed on the basis of the random effect model (P = .7).An atrial communication at biventricular repair of AVSD is associated with significantly reduced long-term survival after adjusting for other known associated factors, including unbalance. These findings might challenge the routine practice of fenestration.