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.

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.

This study describes single-unit experience and explores risk stratification, with protocolised inter-stage cardiac magnetic resonance imaging for infants with hypoplastic left heart syndrome. Survival was retrospectively analysed among the cohort of locally followed survivors of Norwood Stage I procedure, who underwent magnetic resonance imaging under general anaesthesia from 2003 to 2008. This included 32 patients: 17 with Sano conduit and 15 with arterio-pulmonary shunt. The median (inter-quartile range) age and weight at scan were 3.1 (2.6–4.6) months and 5.0 (4.5–5.3) kilograms, respectively. Using morphologic definitions, the median coarctation index was 0.71 (0.57–0.83). The degree of proximal right and left pulmonary artery narrowing was 25% (14–44%) and 25% (11–50%), respectively. The median right ventricular ejection fraction was 54% (48–59%). The ejection fraction was not related to the coarctation index or to pulmonary artery narrowing. Patients were followed up for a median of 19.2 (10.8–46.0) months, during which 13 (41%) had an intervention in addition to routine Norwood Stage II surgery and seven died. Risk of death was related to reduced right ventricular ejection fraction, with a hazard ratio of 0.91 (95% confidence interval 0.85–0.98, p = 0.02), and the cumulative number of focal stenoses of neo-aortic arch and pulmonary arteries (hazard ratio 2.71, 95% confidence interval 1.14–6.44, p = 0.02). Conclusions: In addition to comprehensive three-dimensional morphologic imaging, inter-stage cardiac magnetic resonance imaging provides a ventricular functional index that may predict outcome in patients with hypoplastic left heart syndrome. Measures to preserve right ventricular systolic function and relieve stenoses are paramount within the complex management strategies for these patients.

When there is obstruction of the left ventricular outflow tract, there are several surgical approaches to the repair of discordant ventriculo-arterial connections in the setting of concordant atrioventricular connections. Choosing the optimal technique demands not only knowledge of the different surgical procedures, but also the understanding of the particular anatomic features present in a specific patient. These requisites are then essential to plan the operation, to foresee some difficult situations, and to avoid post-operative complications. In this review, we assess all these surgical and anatomic aspects, focussing on their relative importance in clinical assessment.

The appropriate timing of intervention in patients with chronic aortic incompetence allows recovery of ventricular function. We sought to determine the optimal timing of the Ross procedure for chronic aortic incompetence in young patients. We retrospectively analysed case notes, and measured pre- and postoperative echocardiographic indexes of left ventricular function, in patients who had undergone the Ross procedure for chronic aortic incompetence. Methods and results: We found 21 patients with preoperative and postoperative data suitable for analysis. Their age at operation ranged from 5.6 to 26 years, with a median of 13.8 years, and the duration of follow-up was from 0.5 to 6.8 years, with a median of 2.4 years. The preoperative left ventricular end-diastolic dimension was converted to a z-score, and this was used as a threshold to divide the population. Using the threshold of a preoperative left ventricular z-score of more than 3 to divide the population did not show any difference in postoperative parameters of left ventricular function. Significant differences were found postoperatively, however, in both the left ventricular z-score and the ratio of left ventricular end-diastolic radius to posterior wall thickness in diastole, with a cutoff preoperative threshold z-score greater than 4. Conclusion: The increase in the ratio of left ventricular end-diastolic radius to the thickness of the posterior wall in diastole would suggest that there is disruption of left ventricular short axis architecture and myocardial contractile function when intervention is postponed. The significantly larger left ventricular dimension at end-diastole, despite the reduction in volume loading post surgery, may also demonstrate irreversible structural changes. Our data would suggest that recovery of left ventricular function is less likely when the left ventricular z-score has reached the value of 4, and that, ideally, intervention should be performed when the z-score approaches or exceeds 3.

Repair of complex malformations that necessitate restoration of continuity between the right ventricle and the pulmonary arteries can now safely be performed with low morbidity and mortality. Major concerns still remain on the long-term outlook for these patients, and about the durability of the different prostheses used to restore that continuity, whether during initial correction or at the time of reintervention for failure of the conduit or pulmonary regurgitation. In this review, we discuss the salient morphologic features of the right ventricular outflow tract, and then focus on the indications for early and late intervention, current therapeutic options, and outcomes.