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For decades, bovine jugular vein conduits (BJV) and classic cryopreserved homografts have been the two most widely used options for pulmonary valve replacement (PVR) in congenital heart disease. More recently, decellularized pulmonary homografts (DPH) have provided an alternative avenue for PVR. Matched comparison of patients who received DPH for PVR with patients who received bovine jugular vein conduits (BJV) considering patient age group, type of heart defect, and previous procedures. 319 DPH patients were matched to 319 BJV patients; the mean age of BJV patients was 15.3 (SD 9.5) years versus 19.1 (12.4) years in DPH patients (p = 0.001). The mean conduit diameter was 24.5 (3.5) mm for DPH and 20.3 (2.5) mm for BJV (p p = 0.45). The rate of freedom from endocarditis was significantly lower for BJV patients (87.1 vs. 96.5%, p = 0.006). Freedom from explantation was significantly lower for BJV at 10 years (81.7 vs. 95.5%, p = 0.001) as well as freedom from any significant degeneration at 10 years (39.6 vs. 65.4%, p p = n.s.). DPH showed 20% higher freedom from explantation and degeneration in this subgroup (p = 0.232). Decellularized pulmonary homografts exhibit superior 10-year results to bovine jugular vein conduits in PVR.

Background Most children now survive cardiac surgery, and the focus of quality improvement initiatives has shifted toward more complex outcome measures. The aim of this investigation was to study the impact of early postoperative morbidities on parent-reported patient quality of life and parental anxiety or depression over 6 months. Methods This prospective case-matched cohort study was conducted in 5 UK children’s cardiac centers. Measures of impact for patient categories of “single morbidity,” “multiple morbidities,” and “extracorporeal life support (ECLS)” were compared with “no morbidity.” The measures used were the Pediatric Quality of Life Inventory (PedsQL) and the 4-item Patient Health Questionnaire (PHQ-4) at 6 weeks and 6 months postoperatively. The study modeled the outcomes using mixed effects regression, adjusting for case mix and clustering within centers. Results The study included 666 patients who underwent operation at a median age of 81 days (interquartile range, 10 to 325 days). At 6-week follow-up, significant adjusted differences to the reference group with no morbidity were found for total PedsQL scores, which were lower in patients with ECLS (P = .01), multiple morbidities (P < .001), and a single morbidity (P = .04), as well as the proportion of parents with anxiety and depression, which were higher in the group with multiple morbidities (P = .04 and P = .01, respectively). At 6 months, measures had improved in all morbidity groups. The only significant adjusted difference in the reference group was for physical PedsQL scores in ECLS (P = .04) and multiple morbidities (P < .01). Conclusions Patient and parent well-being are strongly influenced by postoperative morbidities early after surgery, with improvement by 6 months. Family psychological support and holistic rehabilitation are vital for children who experience postoperative morbidities.

OBJECTIVES Early results from the prospective ESPOIR Trial have indicated excellent results for pulmonary valve replacement using decellularized pulmonary homografts (DPH). METHODS A 5-year analysis of ESPOIR Trial patients was performed to provide an insight into the midterm DPH performance. ESPOIR Trial and Registry patients were matched with cryopreserved homografts (CH) patients considering patient age, type of heart defect and previous procedures to present the overall experience with DPH. RESULTS A total of 121 patients (59 female) were prospectively enrolled (8/2014–12/2016), median age 16.5 years (interquartile range 11.2–29.8), and median DPH diameter 24 mm. One death (73 year-old) occurred during a median follow-up of 5.9 years (5.4–6.4), in addition to 2 perioperative deaths resulting in an overall mortality rate of 2.5%. One case of endocarditis in 637 patient-years was noticed, resulting in an incidence of 0.15% per patient-year. At 5 years, the mean peak gradient was 19.9 mmHg (9.9), mean regurgitation 0.9 (0.6, grade 0–3) and freedom from explantation/any reintervention 97.5% (1.5). The combined DPH cohort, n = 319, comprising both Trial and Registry data, showed significantly better freedom from explantation for DPH 95.5% (standard deviation 1.7) than CH 83.0% (2.8) (P CONCLUSIONS The 5-year data of the prospective ESPOIR Trial show excellent performance for DPH and low rates of adverse events. ESPOIR Registry data up to 15 years, including a matched comparison with CH, demonstrated statistically significant better freedom from explantation.

Long-term survival is an important metric for health care evaluation, especially in functionally single-ventricle (f-SV) congenital heart disease (CHD). This study's aim was to evaluate the relationship between center volume and long-term survival in f-SV CHD within the centralized health care service of England and Wales.This was a retrospective cohort study of children born with f-SV CHD between 2000 and 2018, using the national CHD procedure registry, with survival ascertained in 2020.Of 56,039 patients, 3293 (5.9%) had f-SV CHD. Median age at first intervention was 7 days (interquartile range [IQR], 4, 27), and median follow-up time was 7.6 years (IQR, 1.0, 13.3). The largest diagnostic subcategories were hypoplastic left heart syndrome, 1276 (38.8%); tricuspid atresia, 440 (13.4%); and double-inlet left ventricle, 322 (9.8%). The survival rate at 1 year and 5 years was 76.8% (95% confidence interval [CI], 75.3%-78.2%) and 72.1% (95% CI, 70.6%-73.7%), respectively. The unadjusted hazard ratio for each 5 additional patients with f-SV starting treatment per center per year was 1.04 (95% CI, 1.02-1.06), P .001. However, after adjustment for significant risk factors (diagnostic subcategory; antenatal diagnosis; younger age, low weight, acquired comorbidity, increased severity of illness at first procedure), the hazard ratio for f-SV center volume was 1.01 (95% CI, 0.99-1.04) P = .28. There was strong evidence that patients with more complex f-SV (hypoplastic left heart syndrome, Norwood pathway) were treated at centers with greater f-SV case volume (P .001).After adjustment for case mix, there was no evidence that f-SV center volume was linked to longer-term survival in the centralized health service provided by the 10 children's cardiac centers in England and Wales.

Flow-sensitive four-dimensional Cardiovascular Magnetic Resonance Imaging (4D Flow CMR) has increasingly been utilised to characterise patients' blood flow, in association with patiens' state of health and disease, even though spatial and temporal resolutions still constitute a limit. Computational fluid dynamics (CFD) is a powerful tool that could expand these information and, if integrated with experimentally-obtained velocity fields, would enable to derive a large variety of the flow descriptors of interest. However, the accuracy of the flow parameters is highly influenced by the quality of the input data such as the anatomical model and boundary conditions typically derived from medical images including 4D Flow CMR. We previously proposed a novel approach in which 4D Flow CMR and CFD velocity fields are integrated to obtain an Enhanced 4D Flow CMR (EMRI), allowing to overcome the spatial-resolution limitation of 4D Flow CMR, and enable an accurate quantification of flow. In this paper, the proposed approach is validated in a U bend channel, an idealised model of the human aortic arch. The flow patterns were studied with 4D Flow CMR, CFD and EMRI, and compared with high resolution 2D PIV experiments obtained in pulsatile conditions. The main strengths and limitations of 4D Flow CMR and CFD were illustrated by exploiting the accuracy of PIV by comparing against PIV velocity fields. EMRI flow patterns showed a better qualitative and quantitative agreement with PIV results than the other techniques. EMRI enables to overcome the experimental limitations of MRI-based velocity measurements and the modelling simplifications of CFD, allowing an accurate prediction of complex flow patterns observed experimentally, while satisfying mass and momentum balance equations.

Professor Marc de Leval (16 April 1941 –26 June 2022) was a pioneer in the field of congenital cardiothoracic surgery and former chair of the British Congenital Cardiac Association [https://www.bcca-uk.org/]. Professor de Leval was appointed as a Consultant Cardiothoracic Surgeon at Great Ormond Street Hospital in 1974. Throughout his distinguished career at Great Ormond Street Hospital, he worked with dedication to improve outcomes for cardiothoracic surgery. His contribution to academic cardiothoracic surgery was significant and far-reaching with over 300 peer-reviewed publications. Of particular note was his work in improving the palliation of children with cyanotic congenital heart disease by the use of the modified Blalock-Thomas-Taussig shunt and his pioneering work over many years to improve understanding of the Fontan circulation using in vitro modelling and computational fluid dynamics that led to the development of the total cavopulmonary connection. His other significant contributions include the importance of analysing surgical failures, being the co-editor of the most readable textbook in our field, and so much more. During his career, he trained many eminent surgeons from around the world, and a Marc de Leval Fellowship of The American Association for Thoracic Surgery [https://www.aats.org/about-the-foundation/the-reach-of-our-programs/foundation-honoring-our-mentors-program/marc-r-de-leval-md] now allows the opportunity for a trainee to study congenital cardiac surgery in the UK or Europe. Marc is fondly remembered by the staff at Great Ormond Street Hospital. The nurses recall his arrival at the break of day on the intensive care unit, having already been for a swim, and the diligence with which he observed his patients following surgery. Former trainees remember a man who, despite his standing, always remained courteous, approachable, and kind. Professor de Leval will be remembered not only as a skilled and dedicated surgeon but also as a distinguished scholar, one with a quest for knowledge in the pursuit of excellence.

Objectives To evaluate late-term tricuspid valve competence and biventricular function following cone reconstruction for Ebstein anomaly, and to explore biventricular remodeling. Methods Consecutive adult and pediatric patients who underwent cone reconstruction from 2009 to 2019 were reviewed for inclusion in this retrospective cardiac magnetic resonance imaging study. Tricuspid valve competence was assessed with tricuspid regurgitation fraction. Biventricular systolic function was assessed by ejection fraction, cardiac index, indexed stroke volume, and indexed aortic and pulmonary artery beat volume. Biventricular remodeling was assessed by planimetered areas (right atrium, functional right ventricle, left heart), and indexed end-diastolic and end-systolic ventricular volumes. Paired t tests or Wilcoxon signed-rank tests were used for analyses. Results Of 58 included patients, 50 underwent cardiac magnetic resonance imaging. Twelve patients had both preoperative and late postoperative cardiac magnetic resonance imaging with a median follow-up of 5.11 years (interquartile range, 3.12-6.07 years). Focusing on these, tricuspid regurgitation fraction decreased (from 69% to 10%; P = .014), right ventricle ejection fraction remained stable, and antegrade pulmonary artery beat volume increased (from 26.7 to 41.6 mL/beat/m2; P = .037). The left ventricle stroke volume (from 30.4 to 44.1 mL/m2; P = .015) and antegrade aortic beat volume (from 28.5 to 41.1 mL/beat/m2; P = .014) also increased, and the left ventricle stroke volume improved progressively with time since surgery (P = .048). Whereas the right atrium area decreased (P = .004), the functional right ventricle and left heart area increased (cm2, P = .021 and P = .004). Right ventricle volumes showed a tendency to normalize and left ventricle indexed end-diastolic volume increased (from 50 to 69 mL/m2; P = .03) over time. Conclusions Cone valve integrity was sustained. Biventricular function improved progressively during follow-up, and there are positive signs of biventricular remodeling late after cone reconstruction.

Background Surgery in grown-ups with congenital heart disease (GUCH) is characterized by complex anatomy, comorbidities, reoperations, and technical challenges. Although 30-day postoperative mortality is low, this measure might be insufficient to reflect adverse outcome monitoring. Our study aimed to establish whether prolonged intensive care unit (ICU) stay (≥7 days) and 6-month mortality were more clinically meaningful measures than 30-day mortality and to identify predictors of adverse outcome. Methods All consecutive GUCH patients from 1998 to 2015 were identified. Perioperative characteristics, diagnoses, and postoperative data were collected retrospectively. Predictors of 30-day and 6-month mortality and prolonged ICU stay were determined with logistic regression. Era effect was tested for quality assurances by dividing the cohort into 4 time intervals. Results Within 17 years, 1093 consecutive cardiac surgical procedures were identified in 1026 GUCH patients. During the study period, 30-day mortality improved significantly, with an overall 30-day mortality of 1.5%; 6-month mortality and prolonged ICU stay were 2.4% and 6.7%, respectively. Despite a decreased number of preoperative patients in New York Heart Association Functional Classification III or higher, prolonged ICU stay increased over the eras. Predictors of adverse outcome were New York Heart Association class III or higher, preoperative renal failure, disease of great complexity, preoperative ventilator support, cardiopulmonary bypass time, and concomitant procedures. Conclusions In the current era of low 30-day mortality, extended 6-month mortality and prolonged ICU stay reporting may be more realistic measures of adverse outcomes for counseling GUCH patients at risk.

We aimed to assess the change in global and regional myocardial function before and after surgical revascularization and their added value when compared with conventional measures in children with anomalous left coronary artery from the pulmonary artery (ALCAPA).Advanced echocardiographic assessment was performed pre- and postoperatively in 22 children with ALCAPA (eight male; median surgery age, 0.4 years; interquartile range, 0.21-1.05) and 22 healthy controls. Measurements included global and segmental longitudinal, radial, and circumferential two-dimensional speckle-tracking strain and postsystolic index.Global strains were lower in preoperative patients than in controls (longitudinal: -9% vs -21%; P .001; circumferential: -11% vs -21%; P .001; radial: 18% vs 60%; P .001) and improved postoperatively when compared with preoperative findings (longitudinal: -9% pre vs -16% post; P = .002, circumferential:-11% pre vs -17% post; P = .012, radial: 18% pre vs 53% post; P = .001). Preoperatively, patients with normal global systolic function on conventional echocardiography had significantly impaired global longitudinal and radial strain compared with healthy controls. Global mechanical dyssynchrony improved significantly postoperatively (longitudinal postsystolic index 43 pre vs 6 post, P .001; circumferential 15 pre vs 2 post, P = .001; radial 48 pre vs 5 post, P = .003). Despite overall improvement in most segments, global longitudinal and circumferential and segmental peak strain in some of the segments supplied by the ALCAPA remained postoperatively abnormal.This study shows that myocardial deformation indices were a more sensitive measure of LV dysfunction in patients before and after ALCAPA repair than conventional echocardiographic measures. We believe, therefore, they should be added to routine preoperative and serial postoperative follow-up assessment.

OBJECTIVES Following the Ross operation, the pulmonary autograft undergoes structural changes (remodelling). We sought to determine the extent, nature and possible determinants of long-term remodelling in the different components of the pulmonary autograft. METHODS Ten pulmonary autografts and 12 normal control valves (6 pulmonary and 6 aortic) were examined by conventional histology, immunocytochemistry and electron microscopy. The structural changes were quantified by morphometry. RESULTS The leaflets from free-standing root replacement valves demonstrated thickening to levels comparable to the normal aortic leaflets, largely due to the addition of a thin layer of ‘neointima’ formed of radial elastic fibres, collagen bundles and glycoaminoglycans, on the ventricular aspect of the leaflets. The leaflets of valves from sub-coronary implantation demonstrated a significantly thicker fibroelastic layer on the ventricularis and calcium deposition in the fibrosa. The media of the explanted valves showed increased number of lamellar units to levels comparable to normal aortic roots. Electron microscopy of valves inserted as free-standing roots showed increased organization into continuous layers. However, intralamellar components showed varying degrees of ‘disorganization’ in comparison to those in the normal aortic media. In addition, there was a marked increase in the number of vasa vasorum with thickened arteriolar wall in the outer media and adventitia. CONCLUSIONS Following the Ross operation, in the very long term, all components of the autograft showed varying degrees of remodelling, which was judged to be largely adaptive. Defining the type, determinants and possible functional effects of remodelling could help in understanding and optimizing the results of the Ross operation.

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.

Background Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. Methods A retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair. Results Of 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14–57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died. A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60o, relative risk of dehiscence was 3.2 (CI 1.3–4.9, p = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3–100, p

ObjectiveGiven the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome.MethodsWe performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000–2018. The primary outcome was mortality, ascertained from the Office for National Statistics in 2020.ResultsOf 53 615 patients, 1557 had FUH: 55.9% were boys and 67.4% were of White ethnic groups. The largest diagnostic categories were tricuspid atresia (28.9%), double inlet left ventricle (21.0%) and unbalanced atrioventricular septal defect (AVSD) (15.2%). The ages at staged surgery were: initial palliation 11.5 (IQR 5.5–43.5) days, cavopulmonary shunt 9.2 (IQR 6.0–17.1) months and Fontan 56.2 (IQR 45.5–70.3) months. The median follow-up time was 10.8 (IQR 7.0–14.9) years and the 1, 5 and 10-year survival rates after initial palliation were 83.6% (95% CI 81.7% to 85.4%), 79.4% (95% CI 77.3% to 81.4%) and 77.2% (95% CI 75.0% to 79.2%), respectively. Higher hazards were present for unbalanced AVSD HR 2.75 (95% CI 1.82 to 4.17), atrial isomerism HR 1.75 (95% CI 1.14 to 2.70) and low weight HR 1.65 (95% CI 1.13 to 2.41), critical illness HR 2.30 (95% CI 1.67 to 3.18) or acquired comorbidities HR 2.71 (95% CI 1.82 to 4.04) at initial palliation.ConclusionAlthough treatment pathways for FUH are complex and variable, nearly 8 out of 10 children survived to 10 years. Longer-term analyses of outcome based on diagnosis (rather than procedure) can inform parents, patients and clinicians, driving practice improvements for complex CHD.

BACKGROUND Intraoperative anti-A/B immunoadsorption (ABO-IA) was recently introduced for ABO-incompatible heart transplantation. Here we report the first case series of patients transplanted with ABO-IA, and compare outcomes with those undergoing plasma exchange facilitated ABO-incompatible heart transplantation (ABO-PE). METHODS Data were retrospectively analysed on all ABO-incompatible heart transplants undertaken at a single centre between January 1, 2000 and June 1, 2020. Data included all routine laboratory tests, demographics and pre-operative characteristics, intraoperative details and post-operative outcomes. Primary outcome measures were volume of blood product transfusions, maximum post-transplant isohaemagglutinin titres, occurrence of rejection and graft survival. Secondary outcome measures were length of intensive care and hospital stay. Demographic and survival data were also obtained for ABO-compatible transplants during the same time period for comparison. RESULTS Thirty-seven patients underwent ABO-incompatible heart transplantation, with 27 (73%) using ABO-PE and 10 (27%) using ABO-IA. ABO-IA patients were significantly older than ABO-PE patients (p < 0.001) and the total volume of blood products transfused during the hospital admission was significantly lower (164 [126-212] ml/kg vs 323 [268-379] ml/kg, p < 0.001). No significant differences were noted between methods in either pre or post-transplant maximum isohaemagglutinin titres, incidence of rejection, length of intensive care or total hospital stay. Survival comparison showed no significant difference between antibody reduction methods, or indeed ABO-compatible transplants (p = 0.6). CONCLUSIONS This novel technique appears to allow a significantly older population than typical to undergo ABO-incompatible heart transplantation, as well as significantly reducing blood product utilization. Furthermore, intraoperative anti-A/B immunoadsorption does not demonstrate increased early post-transplant isohaemagglutinin accumulation or rates of rejection compared to ABO-PE. Early survival is equivalent between ABO-IA, ABO-PE and ABO-compatible heart transplantation.

Background Patients with repair of tetralogy of Fallot (rToF) who are approaching adulthood often exhibit pulmonary valve regurgitation, leading to right ventricle (RV) dilatation and dysfunction. The regurgitation can be corrected by pulmonary valve replacement (PVR), but the optimal surgical timing remains under debate, mainly because of the poorly understood nature of RV remodeling in patients with rToF. The goal of this study was to probe for pathologic molecular, cellular, and tissue changes in the myocardium of patients with rToF at the time of PVR. Methods and Results We measured contractile function of permeabilized myocytes, collagen content of tissue samples, and the expression of mRNA and selected proteins in RV tissue samples from patients with rToF undergoing PVR for severe pulmonary valve regurgitation. The data were compared with nondiseased RV tissue from unused donor hearts. Contractile performance and passive stiffness of the myofilaments in permeabilized myocytes were similar in rToF‐PVR and RV donor samples, as was collagen content and cross‐linking. The patients with rToF undergoing PVR had enhanced mRNA expression of genes associated with connective tissue diseases and tissue remodeling, including the small leucine‐rich proteoglycans ASPN (asporin), LUM (lumican), and OGN (osteoglycin), although their protein levels were not significantly increased. Conclusions RV myofilaments from patients with rToF undergoing PVR showed no functional impairment, but the changes in extracellular matrix gene expression may indicate the early stages of remodeling. Our study found no evidence of major damage at the cellular and tissue levels in the RV of patients with rToF who underwent PVR according to current clinical criteria.

Background Pulmonary artery sling is commonly associated with tracheal stenosis and intracardiac anomalies. While surgical repair is standardized, coexistent anomalies often determine outcomes. With the paucity of risk stratification, this study aimed to review our experience and stratify risk factors for the surgical outcome of complex pulmonary artery sling repair in the presence of airway or intracardiac lesions. Methods Seventy-nine consecutive children with pulmonary artery sling were evaluated retrospectively following surgical repair. Median age at surgery was 5 months (interquartile range 3–9). Surgical approaches included pulmonary artery sling alone ( n = 10), pulmonary artery sling with tracheoplasty ( n = 41), and pulmonary artery sling with both intracardiac and tracheal surgery ( n = 28). Results There were 7 early (8.8%) deaths. Two patients after left pulmonary artery reimplantation needed revision of the anastomosis. The median intensive care and hospital stay were 11 (interquartile range 9.2–24.8) and 17.9 (interquartile range 4.3–19.8) days, and considerably longer when associated tracheal surgery ( p = 0.002). Follow-up was complete in 66/69 and 3 (3.8%) children died late: 2.7, 10.2, and 17 months after surgery. Univariate analysis showed abnormal lung and coexisting structural heart disease as risk factors. Multivariate analysis revealed total cardiopulmonary bypass time as an independent predictor of overall mortality. Conclusion Complex pulmonary artery sling repair can be performed with a good surgical outcomes even when associated with airway malformations or structural heart diseases. Lung abnormality and longer cardiopulmonary bypass time as a surrogate marker of complex surgery, are possible risk factors.

ObjectiveEarly mortality rates for paediatric cardiac surgery have fallen due to advancements in care. Alternative indicators of care quality are needed. Postoperative morbidities are of particular interest. However, while health impacts have been reported, associated costs are unknown. Our objective was to calculate the costs of postoperative morbidities following paediatric cardiac surgery.DesignTwo methods of data collection were integrated into the main study: (1) case-matched cohort study of children with and without predetermined morbidities; (2) incidence rates of morbidity, measured prospectively.SettingFive specialist paediatric cardiac surgery centres, accounting for half of UK patients.PatientsCohort study included 666 children (340 with morbidities). Incidence rates were measured in 3090 consecutive procedures.MethodsRisk-adjusted regression modelling to determine marginal effects of morbidities on per-patient costs. Calculation of costs for hospital providers according to incidence rates. Extrapolation using mandatory audit data to report annual financial burden for the health service.Outcome measuresImpact of postoperative morbidities on per-patient costs, hospital costs and UK health service costs.ResultsSeven of the 10 morbidity categories resulted in significant costs, with mean (95% CI) additional costs ranging from £7483 (£3–£17 289) to £66 784 (£40 609–£103 539) per patient. On average all morbidities combined increased hospital costs by 22.3%. Total burden to the UK health service exceeded £21 million each year.ConclusionPostoperative morbidities are associated with a significant financial burden. Our findings could aid clinical teams and hospital providers to account for costs and contextualise quality improvement initiatives.

Vascular phantoms mimicking human vessels are commonly used to perform in vitro hemodynamic studies for a number of bioengineering applications, such as medical device testing, clinical simulators, and medical imaging research. Simplified geometries are useful to perform parametric studies, but accurate representations of the complexity of the in vivo system are essential in several applications as personalized features have been found to play a crucial role in the management and treatment of many vascular pathologies. Despite numerous studies employing vascular phantoms produced through different manufacturing techniques, an economically viable technique, able to generate large complex patient-specific vascular anatomies, accessible to nonspecialist laboratories, still needs to be identified. In this work, a manufacturing framework to create personalized and complex phantoms with easily accessible and affordable materials and equipment is presented. In particular, three-dimensional (3D) printing with polyvinyl alcohol (PVA) is employed to create the mold, and lost core casting is performed to create the physical model. The applicability and flexibility of the proposed fabrication protocol is demonstrated through three phantom case studies—an idealized aortic arch, a patient-specific aortic arch, and a patient-specific aortic dissection model. The phantoms were successfully manufactured in a rigid silicone, a compliant silicone, and a rigid epoxy resin, respectively; using two different 3D printers and two casting techniques, without the need of specialist equipment.

Coronary anomalies are uncommon congenital variations in coronary anatomy, occurring in 0.2–1.2% of the general population, the majority of which are detected incidentally and have little clinical significance. A minority of congenital coronary anomalies, primarily due to an interarterial course, are clinically significant, and may present with symptoms of myocardial ischemia, malignant ventricular arrhythmias, and even sudden cardiac death. This chapter focuses on the anatomic features, mode of presentation, diagnostic findings, and surgical treatments that are available for the two most common types of isolated congenital coronary anomalies that are associated with myocardial ischemia during childhood: (1) anomalous origin of a coronary artery from the pulmonary artery and (2) anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course between the great arteries. A brief discussion of coronary artery fistulous connections completes the chapter.

Background Since 1996, ABO-incompatible heart transplantation has been undertaken by performing whole-body plasma exchange to remove isohemagglutinins using the cardiopulmonary bypass (CPB) circuit at the time of transplantation. This requires large volumes of donated blood and blood products, causes hemodynamic instability during the exchange transfusion, and limits practical use to small children. We sought to determine the efficacy of anti-A/B immunoadsorption within the CPB circuit on removal of isohemagglutinins in an ex vivo setting before its use clinically. Methods An anti-A/B immunoadsorption column was placed into a CPB circuit mimicking a typical ABO-incompatible transplant patient, which had been primed with type O whole human blood. Samples were taken for determination of isohemagglutinin titers following each plasma volume pass through the anti-A/B immunoadsorption column. Results There was a linear decrease of at least 1 dilution seen in both anti-A and anti-B IgG and IgM antibodies with each plasma volume pass through the column. This predictable removal allowed the formulation of selection criteria for ABO-incompatible heart transplantation given the reciprocal of titer and patient weight. This degree of predictability allowed us to use it successfully in the clinical setting, reducing antibodies to an undetectable level during ABO-incompatible heart transplantation. Conclusions The incorporation of an anti-A/B immunoadsorption column into the extracorporeal circuit reduces allogeneic blood product requirement for ABO-incompatible heart transplantation, while providing efficacious removal of anti-A and anti-B isohemagglutinins. This can be undertaken within the time period of CPB before graft reperfusion and expands the potential recipient pool to larger patients with higher isohemagglutinin titers.

IntroductionThe objective of this study was to prospectively validate the “Brief Developmental Assessment”, which is a new early recognition tool for neurodevelopmental abnormalities in children with heart disease that was developed for use by cardiac teams.MethodsThis was a prospective validation study among a representative sample of 960 pre-school children with heart disease from three United Kingdom tertiary cardiac centres who were analysed grouped into five separate age bands.ResultsThe “Brief Developmental Assessment” was successfully validated in the older four age bands, but not in the youngest representing infants under the age of 4 months, as pre-set validation thresholds were met – lower 95% confidence limit for the correlation coefficient above 0.75 – in terms of agreement of scores between two raters and with an external measure the “Mullen Scales of Early Learning”. On the basis of American Association of Pediatrics Guidelines, which state that the sensitivity and specificity of a developmental screening tool should fall between 70 and 80%, “Brief Developmental Assessment” outcome of Red meets this threshold for detection of Mullen scores >2 standard deviations below the mean.ConclusionThe “Brief Developmental Assessment” may be used to improve the quality of assessment of children with heart disease. This will require a training package for users and a guide to action for abnormal results. Further research is needed to determine how best to deploy the “Brief Developmental Assessment” at different time points in children with heart disease and to determine the management strategy in infants younger than 4 months old.

ObjectiveTo describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales.MethodsThe UK’s national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code.ResultsThere were 976 patients with HLHS. Of these, 9.6% had a prepathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing prepathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Prepathway intervention was associated with the highest in-hospital mortality (34.0%).44.6% of patients had an off-pathway procedure after their primary procedure, most frequently stenting or dilation of residual or recoarctation and most commonly occurring between stage 1 and stage 2.The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5 to 63.7) and 56.3% (95% CI 53.0 to 59.5), respectively. Patients with an antenatal diagnosis (multivariable HR (MHR) 1.63 (95% CI 1.12 to 2.38)), low weight (ConclusionTreatment pathways among patients with HLHS are complex and variable. It is essential that the long-term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement.

ObjectivesInfants with symptomatic partial and transitional atrioventricular septal defect undergoing early surgical repair are thought to be at greater risk. However, the outcome and risk profile of this cohort of patients are poorly defined. The aim of this study was to investigate the outcome of symptomatic infants undergoing early repair and to identify risk factors which may predict mortality and reoperation.MethodsThis multicentre study recruited 51 patients (24 female) in three tertiary centres between 2000 and 2015. The inclusion criteria were as follows: (1) partial and transitional atrioventricular septal defect, (2) heart failure unresponsive to treatment, (3) biventricular repair during the first year of life.ResultsMedian age at definitive surgery was 179 (range 0–357) days. Sixteen patients (31%) had unfavourable anatomy of the left atrioventricular valve: dysplastic (n=7), double orifice (n=3), severely deficient valve leaflets (n=1), hypoplastic left atrioventricular orifice and/or mural leaflet (n=3), short/poorly defined chords (n=2). There were three inhospital deaths (5.9%) after primary repair. Eleven patients (22%) were reoperated at a median interval of 40 days (4 days to 5.1 years) for severe left atrioventricular valve regurgitation and/or stenosis. One patient required mechanical replacement of the left atrioventricular valve. After median follow-up of 3.8 years (0.1–11.4 years), all patients were in New York Heart Association (NYHA) class I. In multivariable analysis, unfavourable anatomy of the left atrioventricular valve was the only risk factor associated with left atrioventricular valve reoperation.ConclusionsAlthough surgical repair is successful in the majority of the cases, patients with partial and transitional atrioventricular septal defect undergoing surgical repair during infancy experience significant morbidity and mortality. The reoperation rate is high with unfavourable left atrioventricular valve anatomy.

OBJECTIVES Surgical strategies to treat drug refractory left ventricular outflow tract obstruction (LVOTO) in hypertrophic cardiomyopathy include septal myectomy (SM) and, less frequently, mitral valve (MV) repair or replacement. The primary aim of this study was to report the surgical technique and management outcomes in a consecutive group of patients with variable phenotypes of hypertrophic cardiomyopathy in a broad national specialist practice. METHODS A total of 203 consecutive patients, 132 men (mean age 48.6 ± 14.6 years) underwent surgery for the management of LVOTO. Surgical approaches included SM (n = 159), SM with MV repair (n = 25), SM with MV replacement (n = 9) and MV replacement alone (n = 10). Specific surgical approaches were performed based on the underlying mechanism of obstruction. Eleven (5.4%) patients had previous alcohol septal ablation for the management of LVOTO. Concomitant non-mitral cardiac procedures were carried out in 22 (10.8%) patients. RESULTS Operative survival rate was 99.0% with 2 deaths within 30 days. The mean bypass time was 92.9 ± 47.8 min, with a mean length of hospital stay of 10.5 ± 7.8 days. Surgical complications included 3 ventricular septal defects requiring repair (1.5%), 1 Gerbode defect surgically repaired, 2 aortic valve repairs (1.0%), 2 transient ischaemic attacks (1.0%) and 4 strokes (2.0%). Thirty-nine (19.2%) patients had perioperative new-onset atrial fibrillation and 8 (3.9%) patients had unexpected atrioventricular block requiring a permanent pacemaker. Mean resting left ventricular outflow tract gradient improved from 70.6 ± 40.3 mmHg preoperatively to 11.0 ± 10.5 mmHg at 1 year postoperatively (P

Aims Surgical intervention is used to treat dynamic left ventricular outflow tract obstruction (LVOTO) in hypertrophic cardiomyopathy. This study assesses the effect of different surgical strategies on long-term mortality and morbidity. Methods and results In total, 347 patients underwent surgical intervention for LVOTO (1988-2015). Group A (n = 272) underwent septal myectomy; Group B (n = 33), septal myectomy and mitral valve (MV) repair; Group C (n = 22), myectomy and MV replacement; and Group D (n = 20), MV replacement alone. Median follow-up was 5.2 years (interquartile range 1.9-7.9). The mean resting LVOT gradient improved post-operatively from 71.9 ± 39.6 mmHg to 13.4 ± 18.5 mmHg (P 1 New York Heart Association (NYHA) class; 58.9% of patients undergoing MV replacement alone did not improve their NYHA class. There were 5 perioperative deaths and 20 late deaths (>30 days). Survival rates at 1, 5 and 10 years respectively were 98.4, 96.9, 91.9% in Group A; 97.0, 92.4, 61.6% in Group B; 100.0, 100.0, 55.6% in Group C; and 94.7, 85.3, 85.3% in Group D (log-rank, P 30 days) complications included atrial fibrillation (29.6%), transient ischaemic attack/stroke (2.4%) and heart failure hospitalisation (3.2%). There were 16 repeat surgical interventions at 3.0 years. Conclusion Septal myectomy is a safe procedure resulting in symptomatic improvement in the majority of patients. The annual incidence of non-fatal disease-related complications after surgical treatment of LVOTO is relatively high. Patients who underwent MV replacements had poorer outcomes with less symptomatic benefit in spite of a similar reduction in LVOT gradients.

Aims The Cone reconstruction in Ebstein's anomaly (EA) aims to reduce tricuspid valve regurgitation (TR) and reposition the valve to the anatomic annulus, but post-operative progress of ventricular function is poorly understood. This study evaluated biventricular function after Cone reconstruction using echocardiographic techniques. Methods and results A retrospective study assessing longitudinal change was conducted from 2009 to 2014. All symptomatic patients with EA and severe TR undergoing surgery were included. Transthoracic advanced echocardiography was performed pre- and post-operatively (at short-term (

OBJECTIVES: The support of the pulmonary autograft root by the fibromuscular left ventricular outflow tract is emphasized to address the concern related to the dilatation of the pulmonary autograft structures in the paediatric population. METHODS: This retrospective study analyses the outcomes of 75 children who were operated between 1998 and 2012 with the subannular interrupted sutures technique at a median age of 10.2 years (range, 5.3 months-18.0 years). Median follow-up time was 5.2 years (range, 3 days-13.2 years). RESULTS: There were no deaths, but there were 3 reinterventions on the autograft for regurgitation and 2 resections of left ventricular outflow tract obstruction. There was no significant autograft stenosis, and freedom from moderate-to-severe regurgitation was 95% (95% confidence interval: 89-100) and 88% (95% confidence interval: 77-99) at 5 and 10 years, respectively. Median z-scores at the latest follow-up examination were, at the annulus, 0.31 [interquartile range (IQR) = -0.81 to 1.2]; at the sinus of Valsalva, 2.7 (IQR = 1.5-3.5); and at the sinotubular junction, 3.1 (IQR = 1.7-4.2). The correlation between z-scores and time after the operation was negative at the level of the annulus (r = -0.29, P = 0.034) but positive at the level of the sinus (r = +0.37, P = 0.005) and the sinotubular junction (r = +0.26, P = 0.068). The median rate of change in the z-score at the annulus was low, 0.065 z-score/year (IQR = -0.13 to 0.43). CONCLUSIONS: The subannular interrupted sutures implantation technique is associated with acceptable risks and, in the midterm, delivers limited annular dilatation, autograft regurgitation and delayed need for autograft reintervention. (Less)

With their three-dimensional imaging technique, Yoshitake et al. showed that damage resulting in heart block during surgery for atrioventricular septal defect might even occur when taking superficial bites around the location of the conduction system. Sutures for the patch repair should be placed well away from the conduction tissues.

Background Survival with critical aortic valve stenosis (CAS) can be successfully achieved in the short term. Long-term outcome however remains uncertain. We sought to study the long-term survival and reinterventions; exercise capacity and myocardial performance in a subgroup of long-term survivors. Methods Retrospective over 40 years of all patients (n=96) requiring intervention for CAS. A subgroup (n=25) of long-term survivors underwent cardiopulmonary exercise test, echocardiography and magnetic resonance imaging. Results Mean age at first intervention was 9±7.5 days. Early death occurred in 19 (19.8%) and overall reported death was 29 (32.9%). At 20 years, survival rate was 65.8% and freedom from reintervention was 24% (figure 1A and 1B). Median age of our long-term survivors, median age was 15.7±6.4 years, 16(64%) had a Ross procedure and 3(12%) had a mechanical aortic valve. Sixteen patients were in NYHA I, 3 NYHA II, 6 NYHA III. Overall peak VO2 was mildly depressed (84.6±24% predicted; 32.1±8.2 ml/kg/min), normal in 9(45%), severely depressed in 6 (30%). Mean left ventricle (LV) ejection fraction was 65.5±11.22% and mean LV end-diastolic volume Z score was 0.02±1.4. Mean LV outflow tract Vmax was 2.27±1.17 m/s. Four patients (16%) had moderate aortic regurgitation. Mean right ventricular outflow gradient was 19.23±23.57 mmHg. Five patients (20%) had severe LV diastolic dysfunction on echocardiography and confirmed by invasive measurement. Severe diastolic dysfunction was not associated with an older age (p=0.15), small ventricular dimension (p=0.2) or residual obstruction (p=0.39) but was associated with the presence of endocardial fibroelastosis (p=0.00014). Conclusions After an early mortality, long-term survival of patients with critical aortic stenosis is good at the expense of a high rate of reinterventions. Despite a good clinical status, myocardial assessment revealed a high rate of LV diastolic dysfunction that could be a marker of irreversible intrinsic myocardial damage.

The analysis of the blood flow in the great thoracic arteries does provide valuable information about the cardiac function and can diagnose the potential development of vascular diseases. Flow-sensitive four-dimensional flow cardiovascular magnetic resonance imaging (4D flow CMR) is often used to characterize patients' blood flow in the clinical environment. Nevertheless, limited spatial and temporal resolution hinders a detailed assessment of the hemodynamics. Computational fluid dynamics (CFD) could expand this information and, integrated with experimental velocity field, enable to derive the pressure maps. However, the limited resolution of the 4D flow CMR and the simplifications of CFD modeling compromise the accuracy of the computed flow parameters. In this article, a novel approach is proposed, where 4D flow CMR and CFD velocity fields are integrated synergistically to obtain an enhanced MR imaging (EMRI). The approach was first tested on a two-dimensional (2D) portion of a pipe, to understand the behavior of the parameters of the model in this novel framework, and afterwards in vivo, to apply it to the analysis of blood flow in a patient-specific human aorta. The outcomes of EMRI are assessed by comparing the computed velocities with the experimental one. The results demonstrate that EMRI preserves flow structures while correcting for experimental noise. Therefore, it can provide better insights into the hemodynamics of cardiovascular problems, overcoming the limitations of MRI and CFD, even when considering a small region of interest. EMRI confirmed its potential to provide more accurate noninvasive estimation of major cardiovascular risk predictors (e.g., flow patterns, endothelial shear stress) and become a novel diagnostic tool.

ObjectiveTo describe neurodevelopment and follow-up services in preschool children with heart disease (HD).DesignSecondary analysis of a prospectively collected multicentre dataset.SettingThree London tertiary cardiac centres.PatientsPreschool childrenMethodsWe analysed results of Mullen Scales of Early Learning (MSEL) and parental report of follow-up services in a representative convenience sample evaluated between January 2014 and July 2015 within a previous study.ResultsOf 971 preschool children: 577 (59.4%) had ≥1 heart operation, 236 (24.3%) had a known diagnosis linked to developmental delay (DD) (‘known group’) and 130 (13.4%) had history of clinical event linked to DD. On MSEL assessment, 643 (66.2%) had normal development, 181 (18.6%) had borderline scores and 147 (15.1%) had scores indicative of DD. Of 971 children, 609 (62.7%) were not receiving follow-up linked to child development and were more likely to be under these services with a known group diagnosis, history of clinical event linked to DD and DD (defined by MSEL). Of 236 in known group, parents of 77 (32.6%) and of 48 children not in a known group but with DD 29 (60.4%), reported no child development related follow-up. DD defined by MSEL assessment was more likely with a known group and older age at assessment.ConclusionsOur findings indicate that a ‘structured neurodevelopmental follow-up pathway’ in preschool children with HD should be considered for development and evaluation as children get older, with particular focus on those at higher risk.

Aortic coarctation/arch hypoplasia is a relatively common congenital heart disease that leads to severe cardiovascular complications if left untreated. During the modern era, the mortality of the primary surgical repair is very low but the long-term issues, such as recurrent coarctation/arch reobstruction and hypertension, are still significant challenges. The former is related to the surgical repair performed particularly in the management of the smallish distal aortic arch, and for the latter, despite the "successful" repair of the aortic coarctation, the intrinsic vascular anomaly remains a significant long-term morbidity.

Background Despite awareness of neurodevelopmental abnormalities in children with Congenital Heart Disease (CHD), there is no routine developmental monitoring of children with CHD in the United Kingdom. Method An early recognition tool (Brief Developmental Assessment – BDA) was therefore developed using quality improvement methodology involving several iterations and rounds of pilot testing. An expert group of community paediatricians were then engaged to refine the tool and a scoring system developed which enabled categorisation as green (appropriate for age), red (delayed) or amber (equivocal). A convenience sample of 960 pre-school children with CHD from each of five age bands (0–4 months, 5–8 months, 9–14 months, 15 months-2.9 years, 3–4.9 years) were recruited from three UK tertiary cardiac centres as part of an NIHR-funded mixed-methods study. The BDA was prospectively validated using Mullen Scales of Early Learning (MSEL). Construct validity was based on BDA detection of children with known neurodevelopmental abnormalities, and sensitivity and specificity of BDA was evaluated against MSEL. The BDA was successfully validated in the older four age bands (not those 0.75) between two raters and with MSEL. Based on American Association of Paediatrics Guidelines, which state that the sensitivity and specificity of a developmental screening tool should fall between 70%–80%, the BDA outcome of ‘red’ met threshold for detection of MSEL scores>2 SD below the mean. A Delphi survey detailing two scenarios (a child identified with red BDA and a child identified with amber BDA) was sent to 80 professionals and stakeholders and agreement was obtained about a clear referral pathway. Conclusion The BDA, along with a user-training package and an action guide, may be used to improve the quality of neurodevelopmental assessment of children with CHD, facilitating early detection and increased access to services.

Unplanned reintervention (uRE) is used as an indicator of patient morbidity and quality of care in pediatric cardiac surgery. We investigated associated factors and early mortality after uREs.Morbidity data were prospectively collected in 5 UK centers between 2015 and 2017; uRE included surgical cardiac, interventional transcatheter cardiac, permanent pacemaker, and diaphragm plication procedures. Mortality (30-day and 6-month) in uRE/no-uRE patients was reported before and after matching. Predicted 30-day mortality was calculated using the Partial Risk Adjustment in Surgery score.A total of 3090 procedures (2861 patients) were included (median age, 228 days). There were 146 uREs, resulting in an uRE rate of 4.7%. Partial Risk Adjustment in Surgery score, 30-day mortality and 6-month mortality in uRE and no-uRE groups were 2.4% versus 1.3%, 8.9% versus 1%, and 17.1% versus 2.4%, respectively. After matching, mortality at 6 months remained higher in uRE compared with no-uRE (12.2% vs 1.4%; P = .02; 74 pairs). In the uRE group, 21 out of 25 deaths at 6 months occurred when at least 1 additional postoperative complication was present. In multivariable analysis, neonatal age (P = .002), low weight (P = .009), univentricular heart (P .001), and arterial shunt (P .001) were associated with increased risk of uRE, but Partial Risk Adjustment in Surgery score was not (only in univariable analysis).uREs are a relatively frequent complication after pediatric cardiac surgery and are associated with some patient characteristics, but not the Partial Risk Adjustment in Surgery risk score. Early mortality was higher after uRE, independent of preoperative factors, but linked to other postoperative complications.

BackgroundMorbidity is defined as a state of being unhealthy or of experiencing an aspect of health that is “generally bad for you”, and postoperative morbidity linked to paediatric cardiac surgery encompasses a range of conditions that may impact the patient and are potential targets for quality assurance.MethodsAs part of a wider study, a multi-disciplinary group of professionals aimed to define a list of morbidities linked to paediatric cardiac surgery that was prioritised by a panel reflecting the views of both professionals from a range of disciplines and settings as well as parents and patients.ResultsWe present a set of definitions of morbidity for use in routine audit after paediatric cardiac surgery. These morbidities are ranked in priority order as acute neurological event, unplanned re-operation, feeding problems, the need for renal support, major adverse cardiac events or never events, extracorporeal life support, necrotising enterocolitis, surgical site of blood stream infection, and prolonged pleural effusion or chylothorax. It is recognised that more than one such morbidity may arise in the same patient and these are referred to as multiple morbidities, except in the case of extracorporeal life support, which is a stand-alone constellation of morbidity.ConclusionsIt is feasible to define a range of paediatric cardiac surgical morbidities for use in routine audit that reflects the priorities of both professionals and parents. The impact of these morbidities on the patient and family will be explored prospectively as part of a wider ongoing, multi-centre study.

With the imbalance between donation rates and potential recipients growing, transplant programs are increasingly using non-ideal organs from so-called marginal donors. This is the first reported case of the intentional use of a donor heart with ALCAPA. The recipient was aged one yr with restrictive cardiomyopathy who had been supported with BiVAD for over six months. Function of the donor left ventricle was shown to be well preserved, with no obvious signs of ischemia, except for a fibrotic layer on the anterolateral papillary muscle of the mitral valve. To prevent coronary steal, the anomalous left coronary artery ostium from the MPA was oversewn prior to implantation. The transplanted heart spontaneously regained sinus rhythm immediately following cross-clamp release and showed good contractility from the first postoperative echocardiogram. The patient continues to do well 18 months post-transplant, with excellent function on echocardiography, and good flow on coronary angiography.

Among congenital heart diseases, none has undergone a more drastic change in diagnostic approach, management, and outcomes over the last 30 years than hypoplastic left heart syndrome (HLHS), with comfort care replaced as the predominant form of management by a palliative surgical treatment pathway.1 The outcomes of palliative procedures for HLHS have been well documented, but the longer-term picture is less clear. The UK National Congenital Heart Diseases Audit (NCHDA) captures every procedure undertaken for congenital heart disease and updated life status for patients resident in England and Wales from death certifications. From this data set, we identified patients born between 2000 and 2015 who fulfilled the criteria for HLHS: a small left ventricle, stenosis or atresia of the left-sided heart valves, normally aligned great arteries, and no common atrioventricular junction.2 All patients with HLHS require surgical palliation with a traditional Norwood or hybrid pathway or heart transplantation to survive. Hence, we were able to use procedure information in combination with diagnostic information to identify patients with HLHS within the NCHDA data set using the International Paediatric and Congenital Cardiac Codes. Because the NCHDA is a procedure-based data set, patients with HLHS who did not undergo any interventional treatment are not included. Because the hybrid procedure was introduced in the United Kingdom in 2006, we considered 2 eras for …

Given excellent 30-day survival for pediatric cardiac surgery, other outcome measures are important. We aimed to study important early postoperative morbidities selected by stakeholders following a rigorous and evidenced-based process, with a view to identifying potential risk factors.The incidence of selected morbidities was prospectively measured for 3090 consecutive pediatric cardiac surgical admissions in 5 UK centers between October 2015 and June 2017. The relationship between the candidate risk factors and the incidence of morbidities was explored using multiple regressions. Patient survival, a secondary outcome, was checked at 6 months.A total of 675 (21.8%) procedure episodes led to at least 1 of the following: acute neurologic event, unplanned reoperation, feeding problems, renal replacement therapy, major adverse events, extracorporeal life support, necrotizing enterocolitis, surgical infection, or prolonged pleural effusion. The highest adjusted odds ratio of morbidity was in neonates compared with children, 5.26 (95% confidence interval, 3.90-7.06), and complex heart diseases (eg, hypoplastic left heart), 2.14 (95% confidence interval, 1.41-3.24) compared with low complexity (eg, atrial septal defect, P .001 for all). Patients with any selected morbidity had a 6-month survival of 88.2% (95% confidence interval, 85.4-90.6) compared with 99.3% (95% confidence interval, 98.9-99.6) with no defined morbidity (P .001).Evaluation of postoperative morbidity provides important information over and above 30-day survival and should become a focus for audit and quality improvement. Our results have been used to initiate UK-based audit for 5 of these 9 morbidities, co-develop software for local monitoring of these morbidities, and parent information about these morbidities.

Ebstein’s anomaly is a developmental anomaly of the tricuspid valve that primarily involves failure of delamination of the leaflets. However this label encompasses a huge range of valve and ventricular components, in terms of morphology, cellular composition and function, and therefore a great spectrum of haemodynamic situations. On pathologic analysis, increased fibrosis of the right and left ventricular myocardium is a common finding. This is related to intrinsic disease and is progressive, resulting from the abnormal loading conditions for both ventricles. Assessing the underlying function of the RV myocardium is confounded by the loading conditions, and the load-dependency of all conventional echocardiographic and cardiac MRI parameters. Surgical repair of the tricuspid valve, with procedures such as the cone reconstruction, promises to improve TV function and ventricular mechanics. Prudent case selection for this type of surgical repair is crucial and long-term comparative outcome is not yet available, but this type of intervention offers a unique opportunity to examine RV function within the complex haemodynamics of this valve and ventricular anomaly.

Cone reconstruction is advocated to treat severe tricuspid valve (TV) regurgitation associated with Ebstein's anomaly. Data on postoperative clinical status, ventricular adaptation, and objective cardiopulmonary testing are lacking in these patients.The clinical characteristics, echocardiography, magnetic resonance imaging, and exercise data from 27 consecutive cone reconstructions, undertaken from 2009 to 2013, were retrospectively compared between preoperative baseline and follow-up.There were no deaths. The cone TV functioned well in all but 1 patient with late dehiscence of inferior annuloplasty sutures that were subsequently repaired. Four patients required pacemaker insertion (3 for new complete heart block). At median follow-up of 2.7 ± 1.5 years, tricuspid regurgitation was reduced in all patients, without causing stenosis. Global left ventricle function remained unchanged (pre-operative fraction 60% ± 4% vs postoperative fraction 61% ± 3%; P = .96). MRI showed enhanced forward pulmonary flow (pre 26 ± 1 mL/beat vs post 36 ± 10 mL/beat; P.005) and increased left ventricle filling (body surface area-indexed left ventricle end-diastolic volume pre 49 ± 14 mL/m(2) vs post 60 ± 14 mL/m(2); P.005). New York Heart Association functional class improved (pre 2.5 ± 0.6 vs post 1.3 ± 0.6; P.0001) and there was significant improvement in peak oxygen uptake (pre 54% ± 18% vs post 66% ± 22%; P = .02).Cone reconstruction of TV offers an effective repair in patients with severe regurgitation associated with Ebstein's anomaly. The patients' clinical status improved with better left ventricle filling and objective exercise capacity. The durability of repair, and mechanisms by which the ventricles adapt to the new loading conditions, need longer-term study.

OBJECTIVES: Twenty years ago our institution published an analysis of a cluster of failures associated with the arterial switch operation (ASO). The concept of ‘near miss’ was explored to detect warning signs of suboptimal performance. The aim of the current study was to review the parameters best suited to monitoring early ASO outcomes in the modern setting and re-examine the concept of near misses as failure equivalents. METHODS: All ASOs performed in our institution between 1983 and 2012 were reviewed. The experience was divided into three eras (1983–92, 1993–2002 and 2003–12). The cumulative sum graphic for sequential monitoring was used for early mortality. The need to reestablish cardiopulmonary bypass (CPB), CPB time >240 min and extracorporeal membrane oxygenation (ECMO) were explored as variables of near misses. RESULTS: The cohort consisted of 606 patients. The 30-day mortality rate was 23% (n= 29) in Era 1, 6% (n= 14) in Era 2 and 1% (n=3) in Era 3. There were further 4, 8 and 6 deaths between 30 and 90 days in the three eras, respectively. In Era 3, the majority of deaths occurred between 30 and 90 days. In the current era, ECMO and CPB time >240 min as a marker of near miss was associated with an increased risk of death both within 30 days and 90 days after ASO. CONCLUSIONS: The 30-day outcomes of ASO have significantly improved over the last 30 years. As life-saving mechanical support after surgery has been implemented more often, an extended 90-day window of reporting can offer a more realistic outcome indicator of performance.