Congenitally Corrected Transposition of the Great Arteries in Utero: Morphological Spectrum, Outcomes and Pitfalls in Fetal Diagnosis.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare malformation with diverse morphology. We assessed features of fetuses with ccTGA and evaluated neonatal and pediatric outcomes. This was a retrospective review of fetuses with ccTGA at Birmingham Women's and Children's Hospital born from 2005 to 2019. Of thirty-six fetuses identified, six had unavailable prenatal data, one was postnatally diagnosed with isomerism and 29 fetuses were evaluated. ccTGA without associated cardiac lesions was found in 28% (8/29), ccTGA with significant VSD in 31% (9/29), ccTGA with pulmonary obstruction in 24% (7/29) and ccTGA with complex anomalies in 17% (5/29). Tricuspid regurgitation (TR) was observed in 17% (5/29) and heart block (HB) in 10% (3/29) prenatally. Six, that is 21% underwent genetic testing of which one was abnormal. Five extra-cardiac anomalies were reported prenatally and postnatally. Pregnancy was discontinued in five, of which two had moderate TR. There were thirty-one liveborn. Coarctation of the aorta was found in five postnatally but not suspected prenatally. In one, pulmonary stenosis was underestimated; otherwise, prenatal morphology was confirmed. Cardiac interventions were performed in 77% (24/31) liveborn with 39% (12/31) undergoing neonatal intervention. Overall, 6/31 liveborn died including all three with prenatal heart block and one with TR. Estimated survival for all liveborn at 1, 5 and 10 years was 87% (95% CI 76-100%), 83% (95% CI 72-98%) and 80% (95% CI 66-96%) respectively. Accurate prenatal diagnosis of ccTGA is critical for counseling. Early outcomes are favorable with 77% of liveborn undergoing surgery. Fetuses with prenatal diagnosis of complex associated abnormalities, HB and TR appear to do less well.

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.

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.

Linkage of National Congenital Heart Disease Audit data to hospital, critical care and mortality national data sets to enable research focused on quality improvement.

OBJECTIVES: To link five national data sets (three registries, two administrative) and create longitudinal healthcare trajectories for patients with congenital heart disease (CHD), describing the quality and the summary statistics of the linked data set. DESIGN: Bespoke linkage of record-level patient identifiers across five national data sets. Generation of spells of care defined as periods of time-overlapping events across the data sets. SETTING: National Congenital Heart Disease Audit (NCHDA) procedures in public (National Health Service; NHS) hospitals in England and Wales, paediatric and adult intensive care data sets (Paediatric Intensive Care Audit Network; PICANet and the Case Mix Programme from the Intensive Care National Audit & Research Centre; ICNARC-CMP), administrative hospital episodes (hospital episode statistics; HES inpatient, outpatient, accident and emergency; A&E) and mortality registry data. PARTICIPANTS: Patients with any CHD procedure recorded in NCHDA between April 2000 and March 2017 from public hospitals. PRIMARY AND SECONDARY OUTCOME MEASURES: Primary: number of linked records, number of unique patients and number of generated spells of care. Secondary: quality and completeness of linkage. RESULTS: There were 143 862 records in NCHDA relating to 96 041 unique patients. We identified 65 797 linked PICANet patient admissions, 4664 linked ICNARC-CMP admissions and over 6 million linked HES episodes of care (1.1M inpatient, 4.7M outpatient). The linked data set had 4 908 153 spells of care after quality checks, with a median (IQR) of 3.4 (1.8-6.3) spells per patient-year. Where linkage was feasible (in terms of year and centre), 95.6% surgical procedure records were linked to a corresponding HES record, 93.9% paediatric (cardiac) surgery procedure records to a corresponding PICANet admission and 76.8% adult surgery procedure records to a corresponding ICNARC-CMP record. CONCLUSIONS: We successfully linked four national data sets to the core data set of all CHD procedures performed between 2000 and 2017. This will enable a much richer analysis of longitudinal patient journeys and outcomes. We hope that our detailed description of the linkage process will be useful to others looking to link national data sets to address important research priorities.

Defining transposition: What have we learnt?

Understanding transposition is important for all who hope to effectively treat patients with the condition. The variants of the condition are frequently debated in the literature. We describe an unusual variant of transposition, in which despite the arterial roots being supported by morphologically inappropriate ventricles, the roots themselves were normally related, with the intrapericardial arterial trunks spiraling as they extended into the mediastinum. The specimen was identified following the re-categorization of our archive, and we subsequently conducted a detailed analysis of the underlying morphology. Using the principles of sequential segmental analysis, we compared the morphology with standard examples previously described. We show how it was the recognition of such hearts that promoted that concept that the combination of connections across the atrioventricular and ventriculo-arterial junctions was the essence of transposition. In the most common variant, the arrangements are concordant at the atrioventricular junctions, but discordant at the ventriculo-arterial junctions. We suggest that the overall arrangement of discordant ventriculo-arterial connections is best described simply as "transposition." When the discordant ventriculo-arterial connections are combined with similarly discordant connections at the atrioventricular junctions, the transposition is congenitally corrected. We point out that the use of "d" and "l" as prefixes does not distinguish between transposition and its congenitally corrected variant. For those using segmental notations, the correct description for the rare variant found in the setting of a posteriorly located aortic root with the usual atrial arrangement is transposition (S, D, NR).

Four right ventricle to pulmonary artery conduit types.

OBJECTIVE: The most durable valved right ventricle to pulmonary artery conduit for the repair of congenital heart defects in patients of different ages, sizes, and anatomic substrate remains uncertain. METHODS: We performed a retrospective analysis of 4 common right ventricle to pulmonary artery conduits used in a single institution over 30 years, using univariable and multivariable models of time-to-failure to analyze freedom from conduit dysfunction, reintervention, and replacement. RESULTS: Between 1988 and 2018, 959 right ventricle to pulmonary artery conduits were implanted: 333 aortic homografts, 227 pulmonary homografts, 227 composite porcine valve conduits, and 172 bovine jugular vein conduits. Patients weighed 1.6 to 98.3 kg (median 15.3 kg), and median duration of follow-up was 11.4 years, with 505 (52.2%) conduits developing dysfunction, 165 (17.2%) requiring catheter intervention, and 415 (43.2%) being replaced. Greater patient weight, conduit z-score, type and position, as well as catheter intervention were predictors of freedom from replacement. Multivariable analysis demonstrated inferior durability for smaller composite porcine valve conduits, with excellent durability for larger diameter conduits of the same type. Bovine jugular vein conduit longevity was inferior to that of homografts in all but the smallest patients. Freedom from dysfunction at 8 years was 60.7% for aortic homografts, 72% for pulmonary homografts, 51.2% for composite porcine valve conduits, and 41.3% for bovine jugular vein conduits. Judicious oversizing of the conduit improved conduit durability in all patients, but to the greatest extent in patients weighing 5 to 20 kg. CONCLUSIONS: Pulmonary and aortic homografts had greater durability than xenograft conduits, particularly in patients weighing 5 to 20 kg. Judicious oversizing was the most significant surgeon-modifiable factor affecting conduit longevity.

Morphology of vascular ring arch anomalies influences prognosis and management.

OBJECTIVE: This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention. METHODS: This was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 and September 2018 were included. The morphology of the aortic arch was established via analysis of postnatal echocardiography, CT or MRI scans. CT and magnetic resonance studies were evaluated for the presence of a Kommerell diverticulum in those with aberrant vessels. Case notes were reviewed for relevant clinical data. RESULTS: Of those with aberrant subclavian arteries, 24/79 (30.4%) were shown to have a Kommerell diverticulum. Additional forms of congenital heart disease were present in 133/227 (58.6%) individuals. Surgical division of the vascular ring was performed in 30/227 (13.2%), most commonly in the setting of a double aortic arch (70.8%). In those with aberrant subclavian arteries, no children without a Kommerell diverticulum were referred for surgery. In those with a Kommerell diverticulum confirmed on imaging, 11/24 underwent surgery. CONCLUSION: Individuals with a double aortic arch, or an aberrant subclavian artery arising from a Kommerell diverticulum, have the highest requirement for surgical intervention, especially in isolated anomalies. These individuals should remain under monitoring. The subjective nature of symptoms remains problematic. Longitudinal research is required further to understand the natural history of vascular rings and how it links to morphology.

Bilateral Remote Ischaemic Conditioning in Children (BRICC) trial: protocol for a two-centre, double-blind, randomised controlled trial in young children undergoing cardiac surgery.

INTRODUCTION: Myocardial protection against ischaemic-reperfusion injury is a key determinant of heart function and outcome following cardiac surgery in children. However, with current strategies, myocardial injury occurs routinely following aortic cross-clamping, as demonstrated by the ubiquitous rise in circulating troponin. Remote ischaemic preconditioning, the application of brief, non-lethal cycles of ischaemia and reperfusion to a distant organ or tissue, is a simple, low-risk and readily available technique which may improve myocardial protection. The Bilateral Remote Ischaemic Conditioning in Children (BRICC) trial will assess whether remote ischaemic preconditioning, applied to both lower limbs immediately prior to surgery, reduces myocardial injury in cyanotic and acyanotic young children. METHODS AND ANALYSIS: The BRICC trial is a two-centre, double-blind, randomised controlled trial recruiting up to 120 young children (age 3 months to 3 years) undergoing primary repair of tetralogy of Fallot or surgical closure of an isolated ventricular septal defect. Participants will be randomised in a 1:1 ratio to either bilateral remote ischaemic preconditioning (3×5 min cycles) or sham immediately prior to surgery, with follow-up until discharge from hospital or 30 days, whichever is sooner. The primary outcome is reduction in area under the time-concentration curve for high-sensitivity (hs) troponin-T release in the first 24 hours after aortic cross-clamp release. Secondary outcome measures include peak hs-troponin-T, vasoactive inotrope score, arterial lactate and central venous oxygen saturations in the first 12 hours, and lengths of stay in the paediatric intensive care unit and the hospital. ETHICS AND DISSEMINATION: The trial was approved by the West Midlands-Solihull National Health Service Research Ethics Committee (16/WM/0309) on 5 August 2016. Findings will be disseminated to the academic community through peer-reviewed publications and presentation at national and international meetings. Parents will be informed of the results through a newsletter in conjunction with a local charity. TRIAL REGISTRATION NUMBER: ISRCTN12923441.

Anatomical Studies of Transposition - An Argument for a Unifying Morphological Classification.

In the setting of transposition, recognition of the variability in the morphology of the outflow tracts and the arterial trunks is essential for surgical repair. Presence of a unifying system for classification would minimize the risk of misunderstanding. We examined an archive of 142 unrepaired hearts with transposition, defined as the combination of concordant atrioventricular and discordant ventriculoarterial connections, combined with the clinical records of 727 patients undergoing the arterial switch procedures. In the setting of usual atrial arrangement, we found the aortic root in leftward or posterior location in up to 5% of our cohorts, making the common term "d-transposition" ambiguous. Variability in the relationship of the trunks was commoner in the setting of deficient ventricular septation, especially when the trunks were side-by-side (14% vs 5.5% when the ventricular septum was intact). Bilateral infundibulums were present in 14% of cases, and bilaterally deficient infundibulums in 3.5%. Both of these findings were more common with deficient ventricular septation. Fibrous continuity between the tricuspid and pulmonary valves was not always seen with perimembranous defects, particularly when there were bilateral infundibulums. Fibrous continuity between the leaflets of the tricuspid and mitral valves, in contrast, proved a unifying characteristic of the perimembranous defect. The combination of concordant atrioventricular and discordant ventriculoarterial connections is best described simply as "transposition," with precision provided when details are given of atrial arrangement and the relationships of the arterial trunks.

Borders as opposed to so-called geography: which should be used to classify isolated ventricular septal defects?

OBJECTIVES: Ventricular septal defects can be classified according to their borders or according to the fashion in which they open to the right ventricle, so-called geography. As yet, there is no consensus as to how they should be classified. In an attempt to achieve agreement, the International Society for Nomenclature of Congenital and Paediatric Heart Disease, in 2018, proposed a system incorporating both approaches. We have assessed the subjectivity of their suggested terms hoping to determine their suitability in the desired universal system for classification. METHODS: We examined 212 specimens held in the archive of Birmingham Women's and Children's Hospital. Each defect was described by 3 independent examiners on the basis of borders and their relationship to the landmarks of the right ventricle. The interobserver agreement was then calculated using Fleiss' method. RESULTS: Calculations to assess interobserver agreement showed that the examiners were more likely to agree on the borders of the defects than their so-called geography (κ = 0.804 vs κ = 0.518). The landmarks of the right ventricle proved to be highly variable such that the application of 'geographic' terms to hearts with perimembranous defects proved particularly challenging. CONCLUSIONS: Interobserver agreement is lower when using terms based on 'geography' as opposed to borders. Whilst providing important morphological detail, the terms based on right ventricular landmarks are highly subjective. They should not be prioritized in a universal system of classification. Instead, the defects can be classified simply by using 'perimembranous', 'muscular', or 'doubly committed and juxta-arterial' as first-order terms.

Strategies to Minimise Need for Prosthetic Aortic Valve Replacement in Congenital Aortic Stenosis-Value of the Ross Procedure.

To examine the role and outcomes of all interventions for aortic stenosis in children, with focus on freedom from reintervention and the aim to minimise prosthetic aortic valve replacement (pAVR) during childhood. Retrospective analysis of 194 consecutive children who underwent any aortic valve intervention for a biventricular repair strategy at a single institution between 1995 and 2017. Data were obtained from hospital records and follow-up was 100% complete. Over a 22-year period, 194 children underwent total 313 aortic valve procedures: Primary interventions were surgical valvotomy (SV)/surgical repair (SR) in 94 (48.5%), balloon valvuloplasty (BV) in 60 (30.9%), pAVR in 8 (4.1%) and Ross/Ross-Konno procedure in 32 (16.5%). Median age at first intervention was 1.1 years (interquartile range [IQR] 0.1-9.4) and varied with type of intervention: SV/SR were most common in neonates (33, 75%) and infants (35, 68%), whilst BV was most frequent in older children (42, 42%). Operative survival was 99% (2 early deaths, both in neonates with critical aortic stenosis and poor left ventricular function) and 15-year survival was 95%. A Ross procedure was performed in 79 (40.7%) patients over the 15-year study period, 1 of whom required late pAVR for autograft failure. Freedom from any reintervention after SV/SR and BV was 41% and 40% at 10 years, compared to 90% at 10 years with the Ross procedure (P < 0.001). Among neonatal SV/SR and BV, 98% required reintervention during childhood with no difference between groups. Valve morphology did not influence freedom from ultimate valve replacement. In patients who went on to have a Ross procedure, median time from initial intervention to Ross was 2.8 years (IQR 0.1-11.9) in neonates and 6.0 years (IQR 3.1-7.5) in all other age groups. Overall freedom from pAVR was 97% at 10 years and was similar in the SV/SR and BV groups. A strategy of simple valve repair and primary Ross procedure provides excellent survival and good freedom from pAVR. However, reintervention rates after simple interventions for congenital AS are high, especially in younger age groups. The Ross procedure offers the best freedom from reintervention of any technique and wider use of primary Ross in younger age groups should be considered.

Unifocalization cannot rely exclusively on native pulmonary arteries: the importance of recruitment of major aortopulmonary collaterals in 249 cases†.

OBJECTIVES: We sought to define the early and late outcomes of unifocalization based on a classification of the native pulmonary artery (nPA) system and major aortopulmonary collateral arteries (MAPCAs) with a policy of combined recruitment and rehabilitation and to analyse the role of unifocalization by leaving the ventricular septal defect (VSD) open with a limiting right ventricle-pulmonary artery (RV-PA) conduit in borderline cases. METHODS: An analysis of 271 consecutive patients assessed for unifocalization at a single institution between 1988 and 2016 was performed. Patients were classified according to the pulmonary blood supply: group A, unifocalization based on nPA only; group B, based on nPA and MAPCAs; group C, MAPCAs only (absent nPAs). RESULTS: Unifocalization was achieved in 249 (91.9%) cases with an early mortality of 2.8%. Group A included 72 (28.9%) patients, group B 119 (47.8%) patients and group C 58 (23.3%) patients with no difference in early survival between groups. Survival at 5, 10 and 15 years was 90.0% (85.9-94.3), 87.2% (83.5-91.2) and 82.3% (75.2-89.9), respectively. Late survival in groups A and B was similar but 10- and 15-year survival in group C decreased to 79.2% (68.2-92.1) and 74.3% (61.1-90.4) (P = 0.02), respectively. A mean of 1.9 (±0.6) MAPCAs were recruited per patient (range 0-6). The VSD was left open with a limiting RV-PA conduit in 97 (39.0%) cases, but subsequently closed in 48 patients, giving a total of 200 (80.3%) patients achieving VSD closure (full repair). Delaying VSD closure was not associated with increased risk for early or late survival. A central shunt to rehabilitate the nPAs was used in 56 (22.5%) cases. This was associated with a reduction in the number of MAPCAs recruited, but still required a mean of 1.8 (±0.5) MAPCAs recruited per patient to achieve unifocalization. In multivariate risk analysis, those suitable for single-stage full repair had the best long-term outcomes. Group C anatomy was associated with poor late survival compared to groups A and B (hazard ratio 2.7). CONCLUSIONS: Survival is maximized by a combined approach of rehabilitation and recruitment. MAPCAs should always be recruited if they supply areas with absent nPA supply. A strategy of leaving the VSD open with a limiting RV-PA conduit is a safe and effective way of managing borderline cases.

Trends in surgical and catheter interventions for isolated congenital shunt lesions in the UK and Ireland.

OBJECTIVE: To evaluate time trends in the use of catheter and surgical procedures, and associated survival in isolated congenital shunt lesions. METHODS: Nationwide, retrospective observational study of the UK National Congenital Heart Disease Audit database from 2000 to 2016. Patients undergoing surgical or catheter procedures for atrial septal defect (including sinus venosus defect), patent foramen ovale, ventricular septal defect and patent arterial duct were included. Temporal changes in the frequency of procedures, and survival at 30 days and 1 year were determined. RESULTS: 40 911 procedures were performed, 16 604 surgical operations and 24 307 catheter-based interventions. Transcatheter procedures increased over time, overtaking surgical repair in 2003-2004, while the number of operations remained stable. Trends in interventions differed according to defect type and patient age. Catheter closure of atrial septal defects is now more common in children and adults, although surgical interventions have also increased. Patent foramen ovale closure in adults peaked in 2009-2010 before falling significantly since. Surgery remains the mainstay for ventricular septal defect in infants and children. Duct ligation is most common in neonates and infants, while transcatheter intervention is predominant in older children. Excluding duct ligation, survival following surgery was 99.4% and ≈98.7%, and following catheter interventions was 99.7% and ≈99.2%, at 30 days and 1 year, respectively. CONCLUSIONS: Trends in catheter and surgical techniques for isolated congenital shunt lesions plot the evolution of the specialty over the last 16 years, reflecting changes in clinical guidelines, technology, expertise and reimbursement, with distinct patterns according to lesion and patient age.

Tetralogy of Fallot: morphological variations and implications for surgical repair.

OBJECTIVES: Tetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary infundibular stenosis. Archives of retained hearts are an important resource for improving our understanding of congenital heart defects and their morphological variability. This study aims to define variations in aortic override, coronary arterial patterns and ventricular septal defects in tetralogy of Fallot as observed in a morphological archive, highlighting implications for surgical management. METHODS: The Birmingham Children's Hospital archive contains 211 hearts with tetralogy of Fallot, of which 164 were analysed [69 (42.1%) unrepaired and 95 (57.9%) operated specimens]. A detailed morphological and geometric analysis was performed using a rigorous 5-layer review process. RESULTS: Anomalies were observed in the orifices, origins and course of the coronary arteries: 20 hearts (13.0%) had more than 2 orifices and 3 hearts (1.9%) had a single orifice. In 7 hearts (4.3%), a coronary artery crossed the right ventricular outflow tract. The extent of aortic override ranged from 31.0% to 100% (median of 59.5%). The ventricular septal defect was most often perimembranous (139, 84.8%), but we also found muscular (14, 8.5%), atrioventricular (7, 4.3%) and doubly committed juxta-arterial (2, 1.2%) variants. CONCLUSIONS: Anatomical variations are common and can impact surgical management. Anomalous coronary arteries may require a conduit rather than a transannular patch. Variability in aortic override determines the size of patch used to baffle blood to the aorta. The type of ventricular septal defect affects patch closure and the risk of postoperative conduction defects.

Fate of the Left Ventricular Outflow Tract After Rastelli With Selective Infundibular Muscle Resection.

BACKGROUND: The Rastelli procedure has been criticized for a high rate of left ventricular outflow tract obstruction (LVOTO) and translocation procedures have been proposed as an alternative. Infundibular resection can be performed during Rastelli to optimize the outflow tract. This study examines whether a selective policy to enlarge the outflow tract improves Rastelli outcomes across all morphological variants. METHODS: Single institution 29-year experience in 105 patients. Patients were classified into transposition of the great arteries with ventricular septal defect, congenitally corrected transposition of the great arteries with ventricular septal defect, and double outlet right ventricle morphology groups. The infundibular muscle was routinely resected if prominent, in 28 cases. RESULTS: Early mortality was 1.9% (2 of 105) and actuarial survival was 95.4% (95% confidence interval [CI], 89% to 99%) at 1 year, 92.9% (95% CI, 85% to 97%) at 5 years, and 84.5% (95% CI, 74% to 92%) at 10 years. The cumulative freedom from LVOTO was 99% (95% CI, 96% to 100%), 97% (95% CI, 92% to 99%), and 90% (95% CI, 88% to 96%) at 1, 5, and 10 years, respectively. The incidence was similar in all morphological groups and those undergoing infundibular resection were not at higher risk of late LVOTO. Eleven patients required surgical reoperation on the left ventricular outflow tract over a median follow-up period of 8.5 years, with no mortality, although 2 of these patients developed complete heart block. Left ventricular function was well preserved in 98.1% of all cases, including all of those requiring left ventricular outflow tract reoperation. CONCLUSIONS: The Rastelli is a safe procedure that can be applied in a variety of morphological variants. LVOTO remains a late complication of Rastelli, but can be minimized by concomitant infundibular muscle resection. Late reoperation is safe and ventricular function is well preserved in greater than 95% of cases at late follow-up. The operation has stood the test of time and avoids many of the risks of translocation procedures.

Cardioplegia in paediatric cardiac surgery: a systematic review of randomized controlled trials.

OBJECTIVES: Cardioplegia is the primary method for myocardial protection during cardiac surgery. We conducted a systematic review of randomized controlled trials of cardioplegia in children to evaluate the current evidence base. METHODS: We searched MEDLINE, CENTRAL and LILACS and manually screened retrieved references and systematic reviews to identify all randomized controlled trials comparing cardioplegia solutions or additives in children undergoing cardiac surgery published in any language; secondary publications and those reporting inseparable adult data were excluded. Two or more reviewers independently screened studies for eligibility and extracted data; the Cochrane Risk of Bias tool was used to assess for potential biases. RESULTS: We identified 26 trials randomizing 1596 children undergoing surgery; all were single-centre, Phase II trials, recruiting few patients (median 48, interquartile range 30-99). The most frequent comparison was blood versus crystalloid in 10 (38.5%) trials, and the most common end points were biomarkers of myocardial injury (17, 65.4%), inotrope requirements (15, 57.7%) and length of stay in the intensive care unit (11, 42.3%). However, the heterogeneity of patients, interventions and reported outcome measures prohibited meta-analysis. Overall risk of bias was high in 3 (11.5%) trials, unclear in 23 (88.5%) and low in none. CONCLUSIONS: The current literature on cardioplegia in children contains no late phase trials. The small size, inconsistent use of end points and low quality of reported trials provide a limited evidence base to inform practice. A core outcome set of clinically important, standardized, validated end points for assessing myocardial protection in children should be developed to facilitate the conduct of high-quality, multicentre trials. PROSPERO registration: CRD42017080205.

Long-Term Outcome of Interrupted Arch Repair With Direct Anastomosis and Homograft Augmentation Patch.

BACKGROUND: This study analyzed outcomes of interrupted aortic arch (IAA) repair using a standardized technique to interpret the role of the arch repair on late outcomes in a complex and heterogeneous group of patients. METHODS: This single institution study covered the period from 1988 to 2015. A total of 120 cases of IAA were divided into four groups: IAA with ventricular septal defect (VSD) (n = 38), IAA with a Norwood or Damus-Kaye-Stansel procedure (n = 41), IAA with truncus arteriosus (n = 24), and a miscellaneous group (n = 17). Arch repair was performed using a standard technique of direct anastomosis with homograft patch augmentation. RESULTS: IAAs were predominantly type B (n = 81, 68%), and type A (n = 34, 28%), with a significant association of type B with truncus arteriosus and of type A with an aortopulmonary window (p < 0.01). Survival was similar in all groups. The incidence of catheter or surgical reintervention was 18% (confidence interval [CI], 10% to 25%) at 5 years and 18% (CI, 10% to 25%) at 10 years, with catheter reintervention more common and occurring before 18 months. Surgical reintervention occurred in 7% (CI, 2% to 13%) at 5 and 10 years and at 10 years the reintervention rate was lower in the group with truncus arteriosus (0%) and in the group with a Norwood or Damus-Kaye-Stansel procedure (5%). There was no bronchial obstruction or aortic aneurysm. The Cox proportional hazard model showed that weight at surgery <2.5 kg and era of surgery were predictive of outcome, with surgical mortality rates in all variants dropping to 8.3% in the last 15 years of the study. CONCLUSIONS: Repair of IAA using direct anastomosis and patch augmentation is applicable to all variants and provides good long-term arch patency. Survival is strongly associated with weight at surgery.

Randomized controlled trials in children's heart surgery in the 21st century: a systematic review.

OBJECTIVES: Randomized controlled trials are the gold standard for evaluating health care interventions, yet are uncommon in children's heart surgery. We conducted a systematic review of clinical trials in paediatric cardiac surgery to evaluate the scope and quality of the current international literature. METHODS: We searched MEDLINE, CENTRAL and LILACS, and manually screened retrieved references and systematic reviews to identify all randomized controlled trials reporting the effect of any intervention on the conduct or outcomes of heart surgery in children published in any language since January 2000; secondary publications and those reporting inseparable adult data were excluded. Two reviewers independently screened studies for eligibility and extracted data; the Cochrane Risk of Bias tool was used to assess for potential biases. RESULTS: We identified 333 trials from 34 countries randomizing 23 902 children. Most were early phase (313, 94.0%), recruiting few patients (median 45, interquartile range 28-82), and only 11 (3.3%) directly evaluated a surgical intervention. One hundred and nine (32.7%) trials calculated a sample size, 52 (15.6%) reported a CONSORT diagram, 51 (15.3%) were publicly registered and 25 (7.5%) had a Data Monitoring Committee. The overall risk of bias was low in 22 (6.6%), high in 69 (20.7%) and unclear in 242 (72.7%). CONCLUSIONS: The recent literature in children's heart surgery contains few late-phase clinical trials. Most trials did not conform to the accepted standards of reporting, and the overall risk of bias was low in few studies. There is a need for high-quality, multicentre clinical trials to provide a robust evidence base for contemporary paediatric cardiac surgical practice.

Stenting of the Right Ventricular Outflow Tract Promotes Better Pulmonary Arterial Growth Compared With Modified Blalock-Taussig Shunt Palliation in Tetralogy of Fallot-Type Lesions.

OBJECTIVES: This study sought to compare pulmonary arterial (PA) growth during palliation after right ventricular outflow tract (RVOT) stenting versus modified Blalock-Taussig shunt (mBTS) in patients coming forward for complete repair of tetralogy of Fallot-type lesions. BACKGROUND: RVOT stenting is a recent alternative to mBTS in the initial palliation of selected patients with Fallot-type lesions. METHODS: This was a retrospective, single-center study of nonrandomized, consecutive palliated Fallot patients over a 10-year period. Differential left PA (LPA) and right PA (RPA) growth was assessed by serial echocardiograms in 67 patients after mBTS (n = 28) or RVOT stent (n = 39). Statistical data analysis was performed using mixed model analysis. RESULTS: RPA z-scores in the mBTS group improved from median -2.41 (interquartile range [IQR]: -2.97 to -1.32) to -1.13 (IQR: -1.68 to -0.59). LPA z-scores improved from -1.89 (IQR: -2.33 to -1.12) to -0.32 (IQR: -0.88 to -0.05). In the RVOT stenting group RPA z-scores improved from -2.28 (IQR: -3.28 to -1.82) to -0.72 (IQR: -1.27 to +0.48), and LPA z-scores from -2.08 (IQR: -2.90 to -0.61) to -0.05 (IQR: -0.88 to +0.48). Mixed model analysis showed significantly better RPA and LPA growth after RVOT stenting. The benefit of RVOT stenting versus mBTS was 0.599 z-scores for the LPA and 0.749 z-scores for the RPA. Rise in oxygen saturations was greater with RVOT stenting (p = 0.012). Median time to complete repair was shorter in the RVOT stent group (227 [142 to 328] days) compared with the mBTS group (439 [300 to 529] days; p < 0.0003). CONCLUSIONS: RVOT stenting promotes better pulmonary arterial growth and oxygen saturations compared with mBTS in the initial palliation of Fallot-type lesions.