Arrhythmias in congenitally corrected transposition of the great arteries: an international study.

Background And Aims: There is a paucity of data on arrhythmia burden in patients with congenitally corrected transposition of the great arteries (ccTGAs). The present study sought to quantify the incidence of atrial arrhythmia (AA), ventricular arrhythmia (VA), and complete atrioventricular block (CAVB) in patients with ccTGA and identify associated factors.

Methods: An international, multi-centre, retrospective cohort study was conducted in 29 tertiary hospitals (six countries) between 1990 and 2018.

Systems biology approaches investigating mitochondrial dysfunction in cyanotic heart disease: a systematic review.

Background: Cyanotic congenital heart disease (CCHD) affects over 3 million individuals globally and can progress to heart failure. Mitochondrial dysfunction is well established in adult heart failure and is also a central feature of CCHD. CCHD cyanosis itself contributes to further mitochondrial dysfunction.

Borderline left ventricular hypoplasia without significant aortic or mitral stenosis: cardiac magnetic resonance criteria for biventricular repair.

Objectives: Bi-ventricular (Bi-V) repair is viable in some patients with borderline left ventricular (LV) hypoplasia. We aimed to identify cardiovascular magnetic resonance (CMR) criteria predictive of successful primary Bi-V repair in neonates with borderline LV hypoplasia without significant mitral valve (MV) & aortic valve (AV) stenosis.

Methods: Retrospective study (2003-2024) of patients with borderline LV hypoplasia with CMR for decision-making.

Long-term effectiveness of SGLT2 inhibitors in type 2 diabetes patients with myocardial injury: A population-based study.

Background: Sodium-glucose cotransporter 2 (SGLT2) inhibitors have demonstrated cardiovascular benefits in randomized trials. However, real-world evidence on their cardioprotective effect in high-risk populations with documented myocardial injury-such as heart failure (HF), acute coronary syndrome (ACS), myocardial revascularization, or cardiac surgery-is still limited.

Objective: To evaluate the impact of SGLT2 inhibitor adherence on cardiovascular outcomes in patients with type 2 diabetes mellitus (T2DM) and a history of myocardial injury, in a real-world setting.

Borderline Left Ventricle: Criteria for Surgical Decision Making With an Emphasis on Cardiac Magnetic Resonance Imaging.

The borderline left ventricle (LV) encompasses a heterogenous group of cardiac defects that result in underdevelopment of the left heart. Data supporting decision making is difficult to interpret because borderline LV hypoplasia is a relatively rare disease comprising of a heterogenous morphologic spectrum with data originating from single-institution retrospective studies that have all used varying inclusion criteria and imaging modalities/analysis methods, whilst further confounded by heterogenous institutional practice patterns and era effects. Long-term data is lacking.

Role of primary sternal closure in early outcomes following the Norwood procedure.

Objective: To evaluate the impact of primary sternal closure (PSC) after the Norwood procedure, focusing on perioperative factors and clinical outcomes.

Methods: This single-institution retrospective review included 122 neonates who underwent the Norwood procedure (PSC, n = 30; delayed sternal closure [DSC], n = 92) between 2010 and 2022. DSC was further categorized as early (≤2 days) or late (>2 days).

Are We Getting Better?: Ongoing Challenges of Atrioventricular Valve Repair in Children With Single Ventricle Physiology.

Background: This study aimed to assess impact of atrioventricular valve (AVV) regurgitation onset, timing of AVV repair (AVVr), ventricular morphology, and era effect on AVVr outcomes in a single ventricle population.

Methods: A retrospective review of 155 patients with single ventricle physiology who underwent AVVr between 1998 and 2022 was conducted. Transplant-free survival, discharge alive, and AVV reoperation were assessed using the Kaplan-Meier survival method, stratified by the timing of AVVr (Group1 [G1], prebidirectional cavopulmonary shunt, N=33; G2, at or post-shunt, N=93; G3, at or post-Fontan, N=29).

Practical approach to using cardiac magnetic resonance imaging for pre-surgical planning in complex paediatric congenital heart disease.

Decision-making in complex congenital heart disease (CHD) is challenging and requires the integration of anatomic and physiological data. Recent advances in cross-sectional imaging, particularly cardiac magnetic resonance imaging (MRI), have refined this process. In addition to anatomic detail, MRI provides quantitative physiological data on cardiac function and flows through volumetry and phase contrast assessment.

Turbulent-like blood flow in neo-aorta in post-norwood patients.

Background And Objectives: Surgical reconstruction of the aortic arch in children born with hypoplastic left heart syndrome (HLHS) can induce disturbed or turbulent-like blood flow patterns characterized by high-frequency velocity fluctuations (turbulent-like flow). However, previous studies have primarily focused on laminar flows as a surrogate marker of aorto-ventricular inefficiencies. In this study, we utilized high-resolution computational fluid dynamics (CFD) simulations and frequency-based analysis to demonstrate that abnormal neo-aortic geometries resulting from post-surgical anatomies can induce turbulent-like blood flow patterns.

Subcoronary versus full-root Ross procedure for the paediatric population: an early-to-midterm bicentric experience.

Objectives: With 2 different Ross autograft implantation techniques: subcoronary versus full-root evolving, the question remains which strategy demonstrates the better early/mid-term performance, especially concerning autograft durability.

Methods: Patients (0-18 years) undergoing Ross procedure from January 2012 to December 2022 in 2 high-volume centres were included. Patients undergoing both subcoronary and full-root Ross were analysed for early mortality, direct postoperative complications, 5-year survival, incidence of reinterventions/reoperations and autograft durability over time.

The Association Between Residual Lesion Score and Long-term Outcomes of Congenital Cardiac Operations.

Background: This study sought to investigate the impact of the residual lesion score (RLS) on the long-term outcomes of 5 common congenital heart operations.

Methods: All infants who underwent definitive operation for complete atrioventricular septal defect, tetralogy of Fallot (TOF), dextro-transposition of the great arteries, single ventricle (Norwood procedure), and coarctation of the aorta with ventricular septal defect between 2000 and 2012 and who survived until discharge were studied. RLS scores (1, no or trivial; 2, minor; 3, major or in-hospital reinterventions or reoperations for such lesions) were assigned on the basis of postrepair clinical and echocardiographic evaluation.

Impact of cardiac position, morphology and operative technique on long-term Fontan outcomes in heterotaxy†.

Objectives: This study aimed to assess the outcomes of heterotaxy patients who underwent the Fontan operation, focusing on morphological features and surgical techniques.

Methods: Eighty-two consecutive heterotaxy patients who underwent the Fontan operation from 1985 to 2021 were compared to 150 patients with tricuspid atresia (TA) and 144 patients with hypoplastic left heart syndrome (HLHS). The Kaplan-Meier method and Cox proportional hazard model were used to analyse transplant-free survival and predictor of outcomes.

Mixed reality for preoperative planning and intraoperative assistance of surgical correction of complex congenital heart defects.

Objectives: Mixed reality (MixR) is an innovative visualization tool that presents virtual elements in a real-world environment, enabling real-time interaction between the user and the combined digital/physical reality. We aimed to explore the feasibility of MixR in enhancing preoperative planning and intraoperative guidance for the correction of various complex congenital heart defects.

Methods: Patients underwent cardiac computed tomography or cardiac magnetic resonance imaging and segmentation of digital imaging and communications in medicine images was performed.

Congenital cardiac surgical simulation: bridging global workforce gaps and optimizing outcomes.

Reaching competency in congenital heart surgery (CHS) requires lengthy and rigorous training. Due to patient safety, time limitations, and procedural complexity, the intraoperative setting is not ideal for technical practice. Surgical simulation using synthetic, biological, or virtual models is an increasingly valuable educational tool for technical training and assessment.

Staged Approach: The Role of Delayed Repair Following Complete Unifocalization of Major Aortopulmonary Collateral Arteries with Ventricular Septal Defect and Pulmonary Atresia.

The presentation of pulmonary vasculature in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) is highly variable-as is the number, size and position of the MAPCAs and their relationship with the native pulmonary artery system. The priority in the management of this disease should be attaining timely and complete unifocalization, as opposed to single-stage full repair in every case. The merit of early unifocalization is that it secures the pulmonary vascular bed by (a) avoiding loss of lung segments from progressive stenosis/atresia of MAPCA origins, (b) preventing lung injury from high pressure/flow in areas fed by large, unobstructed MAPCAs, and (c) restoring central continuity of the pulmonary vasculature.

Extracorporeal Membrane Oxygenation in Pediatric Patients With Congenital Heart Disease: Surgical Considerations.

The use of extracorporeal membrane oxygenation (ECMO) in the paediatric population has increased over time, with the ability to rescue pulmonary and cardiovascular deterioration. ECMO can be utilised by neonates and children with congenital heart disease in both preoperative and postoperative settings to improve survival and minimise morbidity. ECMO cannulation strategy must be tailored to the age, weight, and physiologic state of the patient.

Is there any role for an atrial septal defect in off-loading a borderline left ventricle?

A small atrial septal defect with right-to-left shunt is useful for off-loading a dysfunctional right ventricle postoperatively. However, an atrial septal defect with left-to-right shunt may not be as useful for a dysfunctional left ventricle. Experimental data are limited at present.

Anatomic and Physiologic Repair of Congenitally Corrected Transposition of the Great Arteries.

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac anomaly. The management strategy historically consisted of physiologic repair, leaving the morphologic right ventricle to support the systemic circulation. More recently, anatomic repair has been implemented to bring the left ventricle into the systemic circulation.

Early Outcomes for Management of Atrioventricular Septal Defect-Tetralogy of Fallot in the Last Decade: A Congenital Heart Surgeons' Society Study.

BackgroundWe sought to determine the management and early outcomes of complete atrioventricular septal defect-tetralogy of Fallot (AVSD-TOF) for a contemporary multicenter cohort.MethodsOf 739 participants in the Congenital Heart Surgeons' Society AVSD cohort (January 2012-May 2021), 40 had AVSD-TOF. We first compared survival differences for patients with AVSD-TOF versus those with isolated AVSD using propensity matching.

"We are very family like": How do relationships with colleagues affect career satisfaction for surgeons?

Purpose: The objective of this research was to explore the role of surgeon relationships with their colleagues on career satisfaction.

Methods: This qualitative study employed a thematic analysis based on the core elements of The Grounded Theory Method. Forty-two pediatric neurosurgeons, cardio-thoracic surgeons and ophthalmologists were recruited from 9 countries around the globe and interviewed in-depth about the role of their collegial relationships on their career satisfaction.