Aortic coarctation can occur isolated or associated with ventricular septal defect. This study evaluated aortic stiffness in normotensive patients surgically treated for aortic coarctation and ventricular septal defect and in those who underwent simple aortic coarctation repair. Both groups were compared with healthy controls. Again, the two pathological groups were compared with each other regarding aortic stiffness and left ventricular diastolic function. A possible relationship between aortic stiffness and left ventricular diastolic function was investigated.

Twenty-two isolated aortic coarctation patients and 17 aortic coarctation and ventricular septal defect patients were enrolled. Aortic root distensibility and aortic stiffness index were calculated from echocardiography and blood pressure. E wave to A wave (E/A) ratio was measured from mitral valve inflow profile.

Aortic root distensibility and aortic stiffness index in simple aortic coarctation vs healthy controls: both p < 0.0001. Aortic root distensibility and aortic stiffness index in aortic coarctation/ventricular septal defect vs healthy controls: both p < 0.0001. Aortic root distensibility and aortic stiffness index were similar in the two pathological groups (both p = ns). No statistically significant difference was detected in relation to left ventricular diastolic function (p = ns). No correlation was detected between aortic stiffness and diastolic function in simple aortic coarctation and aortic coarctation/ventricular septal defect groups (both p = ns).

In both normotensive isolated aortic coarctation and aortic coarctation/ventricular septal defects subgroups, aortic stiffness is increased in a similar way in comparison with controls. Diastolic function was normal and similar in both groups. Aortic stiffness was not related to left ventricular diastolic function in this specific setting.

To summarise the characteristics and postoperative outcomes in paediatric patients with coronary sinus septal defect.

This retrospective study recruited paediatric patients diagnosed with coronary sinus septal defect from the Guangdong Cardiovascular Institute between 2011 and 2023. Clinical characteristics, echocardiographic parameters, surgical procedures, and postoperative outcomes were collected from electronic health records.

Among the 68 patients, 50% were male, with a median age of 1.0 years. Four cases (5.9%) were diagnosed during the prenatal period. The proportions of patients with type I, II, III, and IV coronary sinus septal defect were 51.5%, 5.9%, 16.1%, and 26.5%, respectively. The most common coexisting cardiac anomalies were persistent left superior caval vein. Twenty-seven cases were either missed or misdiagnosed by echocardiogram, accounting for 39.7% of the overall cases, with type I being the most frequently missed diagnosis. Fifty-four patients underwent surgery, two patients received transcutaneous intervention, while the remaining patients did not undergo any surgery or intervention. At follow-up, two patients with type I coronary sinus septal defect died from multiorgan dysfunction, and one patient underwent reoperation due to narrowing of the extracardiac tunnel. The remaining patients did not experience any major events and recovered well.

Paediatric patients with coronary sinus septal defect often do not exhibit specific clinical manifestations. Enhancing our understanding of the anatomic and haemodynamic characteristics of coronary sinus septal defect can improve the diagnostic accuracy of echocardiography. If diagnosis is suspected, confirmation can be obtained by cardiac CT and cardiac magnetic resonance. Accurate preoperative and intraoperative diagnosis of coronary sinus septal defect contributes to high surgical success rates and favourable treatment outcomes.

Medical and surgical advancements have enabled a 95% survival rate for children with CHD. However, these survivors are disproportionately affected by neurodevelopmental disabilities. In particular, they have behavioural problems in toddlerhood. Because there is a known relationship between behavioural problems and early language delay, we hypothesise that children with critical CHD have early detectable language deficits. To test our hypothesis, we performed a retrospective study on a cohort of children with critical CHD to visualise their early language developmental trajectories.

We identified a cohort of 27 children with two diagnoses: single ventricle physiology (19) and transposition of the great arteries (8). As part of their routine clinical care, all of these children had serial developmental evaluations with the language subsection of the Capute Scales. We visualised their developmental language trajectories as a function of chronologic age, and we used a univariate linear regression model to calculate diagnosis-specific expected developmental age equivalents.

In each group, language development is age-appropriate in infancy. Deviation from age-appropriate development is apparent by 18 months. This results in borderline-mild language delay by 30 months.

Using the Capute Scales, our team quantified early language development in infants and toddlers with critical CHD. Our identification of deceleration in skill acquisition reinforces the call for ongoing neurodevelopmental surveillance in these children. Understanding early language development will help clinicians provide informed anticipatory guidance to families of children with critical CHD.

Children with single ventricle physiology and transposition of the great arteries have measurable early language delays.

To evaluate the outcomes of patients with single ventricle physiology supported with extracorporeal membrane oxygenation as a bridge to first-stage palliation.

This was a retrospective registry-based study. Data from the Extracorporeal Life Support Organization registry were used to identify single ventricle physiology patients supported with extracorporeal membrane oxygenation prior to palliation from 2016 to 2021. Descriptive statistics and multivariate analyses for associations with mortality were conducted.

Primary outcome was death before hospital discharge. Patient characteristics including demographics and associated complications were evaluated as secondary outcomes. Sixty-five patients met inclusion criteria. Survival to discharge was 42%. Twenty-four (37%) patients died while on extracorporeal membrane oxygenation. There was no significant difference in demographics between survivors and non-survivors. Non-survivors had a significantly longer median duration on extracorporeal membrane oxygenation compared to survivors, 99-hrs [IQR (Interquartile Range), 160, 300] vs. 59-hrs [43, 124] (p<0.001). Multivariate analysis demonstrated extracorporeal membrane oxygenation duration (adjusted-OR [Odds Ratio] 1.01, 95% CI [Confidence Interval] 0.98, 0.99; p = 0.03) and requiring renal replacement therapy (42% vs. 19%; p = 0.04) were associated with mortality prior to discharge.

Clinicians managing decompensated patients with single ventricle physiology may consider extracorporeal membrane oxygenation as a bridge to palliation. Survival to discharge was 42%. Evidence of renal injury and longer extracorporeal membrane oxygenation durations were associated with mortality. These data may be used to guide providers and to counsel families. However, more data are needed to refine indications and assess associations related to outcomes and decision-making.

Cardiac catheterisation in the postoperative period emerges as a primary tool, providing effectiveness and safety in diagnosis, treatment guidance, and resolution of major residual lesions.

This is a retrospective case-control study. We collected the clinical records of patients who underwent cardiac catheterisation between January 2003 and December 2022 within the initial 72 hours after surgery in the pediatric cardiac ICU of a national referral hospital in Mexico City. Descriptive, univariate, and multivariate analyses were performed.

A total of 6,243 surgeries were performed, of which 264 were cardiac catheterizations carried out within the first 72 hours of the postoperative period;these included 73 diagnostic procedures and 191 interventional procedures. One hundred and thirty-five (135) catheterisations targeted recent suture intervention sites. The primary indications for cardiac catheterisation included low cardiac output and the suspicion of major residual lesions. Approximately 65% of interventions occurred within the first 24 hours and solved 426 residual lesions. No significant associations were found between mortality, complications, and the need for surgical reintervention in patients who underwent interventional catheterisation at recent suture sites (OR 1.93;95% CI:0.94–4.07:p = 0.076). Seventeen patients (17) were extubated in the initial 24 hours post-catheterisation. Two major complications were identified: rupture of the systemic-to-pulmonary shunt in the anastomosis, and a pulmonary artery laceration requiring emergency surgery. One patient died.

Cardiac catheterisation has evolved into a vital instrument to diagnose and resolve abnormalities and significant residual lesions without increasing the morbidity and mortality risks.

Tetralogy of Fallot is the most common cyanotic congenital malformation of the heart. The right ventricular outflow tract is of great interest in this setting, but most of the focus on this feature has been on the size of the so-called pulmonary valvar “annulus”. We aimed to characterise other aspects of the morphology of the pulmonary root in heart specimens with tetralogy of Fallot.

We reviewed archived hearts with tetralogy of Fallot from four registries. The pulmonary root was examined with specific attention to the number of sinuses, the number of leaflets, presence of any fusion of leaflets, and the direction of the zone of apposition between the leaflets. Cluster analyses were then conducted to see if the features permitted segregation into groups.

We examined a total of 155 hearts. The pulmonary valve had two leaflets in 62%, three leaflets in 34%, and one leaflet in 3%. Irrespective of leaflet morphology, most hearts had two sinuses. Cluster analysis permitted segregation into three groups, with the direction of the zone of apposition being the most important feature for segregation.

In two-thirds of our hearts with tetralogy of Fallot, the pulmonary valve had two leaflets. Most frequently there were three sinuses. In the setting of a valve with two sinuses, the zone of apposition between the leaflets pointing towards the aorta. Cluster analysis permitted statistically sound segregation of the heart and highlights the importance of delineating these features, specifically the leaflet and sinus morphology, with clinical imaging.

Understanding complex three-dimensional cardiac structures is the key to knowing CHD. Many learners have limited access to cadaveric specimens, and most alternative teaching modalities are two-dimensional. Therefore, we have developed virtual cardiac models using photogrammetry of actual heart specimens to address this educational need.

A descriptive study was conducted at a single institution during a week-long cardiac morphology conference in October 2022 and 2023. Conference attendees viewed virtual cardiac models via laptop screen and virtual reality headset. Learners were surveyed on their opinions of the virtual models and their perceived effectiveness compared to existing educational materials.

Forty-six learners completed the survey. Participants reported the virtual cardiac models to be more effective than textbook diagrams (60%), and equally or more effective compared to didactic teaching (78%) and specimen videos (78%). Approximately half of participants (54%) found the virtual models to be less effective than hands-on cadaveric specimen inspection. Attitudes towards the virtual specimens were overall positive with most responders finding the tool engaging (87%) and enjoyable (85%). A majority reported that the models deepened their understanding of cardiac morphology (79%) and that they would recommend them to other trainees (87%).

This study demonstrates that a novel teaching tool, virtual cardiac specimens, is equivalent to or more effective than many current materials for learning cardiac morphology. While they may not replace direct cadaveric specimen review, virtual models are an engaging alternative with the ability to reach a wider audience.

Passive leg raising is used to predict who will benefit from fluid therapy in critically ill patients, including children. Patients with a Fontan circulation may have a different haemodynamic response to a fluid challenge by passive leg raising.

The haemodynamic response of 31 paediatric patients with a Fontan circulation from the outpatient clinic (median age 14.0 years) and 35 healthy controls (median age 12.8 years) to passive leg raising was evaluated non-invasively by echocardiography for the assessment of, e.g., velocity time integral across the (neo)aortic valve, blood pressure measurements, and respiration. Participants were considered responders when the velocity time integral increased ≥ 10.0%.

Overall, patients and controls did not differ in the haemodynamic response. Twelve patients (38.7%) and 8 controls (22.9%) were responders, which was not statistically different (P = 0.22). Responders in the patient and control group also had a similar echo-estimated velocity time integral increase of + 18.9% and + 15.2%, respectively (P = 0.91). There was no difference in echo-estimated velocity time integral change between patient and control non-responders with a decrease of −1.4% and −6.4%, respectively (P = 0.70) and no difference in the amount of patients who were negatively affected by passive leg raising, ith a decrease of ≤−10.0% in 7 patients (22.6%) and 9 controls (25.7%)(P = 0.77).

The haemodynamic response of ambulatory paediatric patients with a Fontan circulation to passive leg raising is like that of healthy controls. Patients who did not respond were similarly affected as healthy controls. Whether the haemodynamic response is different in critically ill patients warrants further investigation.

The Netherlands National Trial Register (NTR), Trial: NL6415; date of registration 20-07-2017; Trial information: https://www.trialregister.nl/trial/6415.

Left bundle branch area pacing is a recent technique gaining rapid acceptance due to its broader target area and excellent electrical parameters. The aim of this study was to demonstrate the feasibility of left bundle branch area pacing in children and share short-term results.

A retrospective study conducted at a single centre between December 2021 and April 2024 involved 19 children who underwent left bundle branch area pacing using Select Secure leads. The study included echocardiographic evaluations, pacing parameters, and follow-up outcomes.

The cohort comprised 10 males and 9 females. Median age was seven years (range 2–18), and median weight was 38 kg (range 13–56). All patients had complete atrioventricular block, with seven having isolated congenital complete atrioventricular block and 12 postoperative complete atrioventricular block. In nine patients, transitioning from epicardial to endocardial pacing resulted in ventricular dysfunction due to chronic right ventricular pacing. The remaining patients received left bundle branch area pacing initially. One patient underwent implantation in a septal position close to the left bundle due to left bundle branch area pacing infeasibility. The median post-procedure QRS duration was 92 msec (range 80–117). Median R wave amplitude, threshold, and impedance values were 14.7 mV (range 13.3–16.8), 0.7 mV (range 0.5–1.1), and 728 ohms (range 640–762), respectively. Atrioventricular (DDD mode) leads were implanted in 10 patients, and ventricular leads (VVIR mode) were implanted in nine patients. Median fluoroscopy dose was 18.7 mGy (13.5–34.52).

Left bundle branch area pacing can be safely conducted in paediatric patients exhibiting a narrow QRS duration and stable pacing parameters.