This research seeks to ascertain the prevalence and determinants of mirror-image dextrocardia in fetuses

With December 2022 as the reference point, we compiled colleted data on pregnant women who carried fetuses with mirror-image dextrocardia in Xi’an, Shaanxi Province: September–October 2022, November 2022, and December 2022–January 2023. An online questionnaire was distributed to 209 pregnant across China who had contracted COVID-19. The case group comprised women whose final menstrual cycle occurred in November 2022 and who had a fetus with mirror-image dextrocardia. Women with a November 2022 final menstrual period and a fetus without this condition made up the control group. To identify the risk factors associated with fetal mirror-image dextrocardia, both univariate and multivariate logistic regression analyses were employed.

A significant difference was noted in the gestational age at COVID-19 infection women with a September to October 2022 and December 2022 to January 2023 final menstrual period who did not bear a fetus with mirror-image dextrocardia, and those with a November 2022 final menstrual period whose fetus exhibited this condition. The univariate and multivariate analyses conducted on pregnant women with a final menstrual period in November 2022 who had contracted COVID-19 revealed significant differences in the presence and duration of fever between those bearing fetuses with mirror-image dextrocardia and those without (P = 0.000).

The findings suggest two critical factors to the increased prevalence of fetal mirror-image dextrocardia: 1) the infection timing which occurs between the 4th and 6th week of pregnancy and 2) the presence of fever and its prolonged duration.

To examine feasibility, acceptability, and preliminary effectiveness of a novel group-based telemedicine psychoeducation programme aimed at supporting psychological well-being among adolescents with Fontan-palliated CHD.

A 5-week telemedicine psychoeducation group-based programme (WE BEAT) was developed for adolescents (N = 20; 13–18 years) with Fontan-palliated CHD aimed at improving resiliency and psychological well-being. Outcome measures included surveys of resilience (Connor–Davidson Resilience Scale), benefit finding (Benefit/Burden Scale for Children), depression, anxiety, peer relationships, and life satisfaction (National Institutes of Health Patient-Reported Outcomes Measurement Information System scales). Within-subject changes in these outcomes were compared pre- to post-intervention using Cohen’s d effect size. In addition, acceptability in the form of satisfaction measures and qualitative feedback was assessed.

Among eligible patients reached, 68% expressed interest in study participation. Of those consented, 77% have been scheduled for a group programme to date with 87% programme completion. Twenty adolescents (mean age 16.1 ± SD 1.6 years) participated across five WE BEAT group cohorts (range: 3–6 participants per group). The majority (80%) attended 4–5 sessions in the 5-session programme, and the median programme rating was a 9 out of 10 (10 = most favourable rating). Following WE BEAT participation, resiliency (d = 0.44) and perceptions of purpose in life increased (d = 0.26), while depressive symptoms reduced (d = 0.36). No other changes in assessed outcome measures were noted.

These findings provide preliminary support that a group-based, telemedicine delivered psychoeducation programme to support psychological well-being among adolescents with CHD is feasible, acceptable, and effective. Future directions include examining intervention effects across diverse centres, populations, and implementation methods.

Transcatheter closure of large and complex atrial septal defect can pose challenges and complications during device placement. To improve stability, several assistive techniques have been developed.

This retrospective study evaluated the efficacy of the device-assisted device closure technique for large secundum atrial septal defects. Patients who underwent device-assisted device closure of atrial septal defect between December 2023 and August 2024 were analysed.

Twenty patients (mean age 38.69 years) underwent device closure of large secundum atrial septal defect with device-assisted device closure technique. The mean atrial septal defect diameter was 31.9 mm. The average thick-to-thick measurement was 38.3 mm, which determined the device size. The majority (18 cases) had thin, floppy margins and two had deficient inferior rim. Successful closure was achieved in 18 patients (90%), while two patients (10%) required other methods of assistance. Based on fluoroscopic guidance, patients were divided into two groups: Group A (8 patients) used anteroposterior projection, and Group B (12 patients) used left anterior oblique-cranial view. After initial two failures with anteroposterior view, all cases were successfully closed using left anterior oblique-cranial projection. Device sizes ranged from 36 to 50 mm (median 40 mm). Cocoon devices were used for sizes up to 42 mm, and Occlunix for larger devices. No significant procedural complications occurred, although two patients had minor post-procedural events.

Device-assisted device closure technique offers a promising and safe dynamic assistance approach for transcatheter closure of large and challenging atrial septal defects. The left anterior oblique-cranial view showed promising results, though without statistical significance. While results are encouraging, larger prospective studies are needed to validate its effectiveness.

The persistence of palpitations in some patients after supraventricular tachycardia ablation is a common challenge despite high procedural success rates. Establishing a correlation between symptoms and tachycardia is especially crucial for adolescent patients, who face challenges in perceiving and articulating their symptoms. This study aimed to evaluate the significance and efficacy of external event recorders in detecting symptom-arrhythmia correlation in paediatric patients experiencing palpitations following successful supraventricular tachycardia ablation.

Among the 1682 patients who underwent successful supraventricular tachycardia ablation at our center between 2013 and 2023, the study included those who met the following criteria: 1) diagnosed with atrioventricular nodal reentrant tachycardia, concealed accessory pathway, or focal atrial tachycardia (excluding substrates identifiable on surface electrocardiogram such as Wolff-Parkinson-White and Mahaim type preexcitations), 2) without CHD, 3) aged over 10 years (as this age is considered sufficient for reliable symptom reporting), and 4) presenting with palpitations post-ablation, with no supraventricular tachycardia detected on electrocardiogram or 24-hour Holter monitoring. Patient data were retrospectively reviewed from medical records.

Among a total of 104 patients reporting palpitations (15%), event recording data of 73 patients were available. Supraventricular tachycardia was documented in eight cases, whereas sinus tachycardia was detected in nine patients. Symptom-arrhythmia correlation was achieved in 23% of patients, nearly half of whom had supraventricular tachycardia recurrence. Patients reporting palpitations with recurrent supraventricular tachycardia episodes were analysed according to diagnostic subgroups. The recurrence rate in patients with palpitations was higher in the focal atrial tachycardia group compared to the others (33%).

Despite successful ablation procedures, palpitations continue to pose a significant concern. To precisely explore the actual symptom-rhythm correlation, we advocate safe and effective utilisation of external event recorders in paediatric patients, particularly those with sporadic symptoms, as opposed to relying solely on electrocardiogram and Holter monitoring.

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.

This study aims to examine the surgical outcome of Kabuki syndrome patients after neonatal congenital heart surgery.

This was a single-centre retrospective study of Kabuki syndrome patients undergoing neonatal congenital heart surgery from 2018 to 2023. Primary outcome was survival to discharge after index surgery. Secondary outcomes were morbidities and complications. Survival and hospital length of stay were compared to neonates with non-Kabuki genetic anomalies undergoing congenital heart surgery in the same time period.

A total of seven patients were reviewed. All Kabuki syndrome patients had left-sided lesions including three with hypoplastic left heart syndrome, three with aortic stenosis and/or aortic arch hypoplasia, and one with an isolated coarctation of aorta. Hospital survival was 5/7 (71% compared to 88% for neonates with non-Kabuki genetic anomalies). To date, four remain alive, including one with hypoplastic left heart syndrome. A higher percentage of Kabuki syndrome patients had unplanned interventions (43% vs 15% in non-Kabuki), abnormal brain imaging (29% vs 5%), and bacteremia (29% vs 9%). Median total ventilator days for Kabuki patients were also longer (16 days vs 6 days in non-Kabuki) as was hospital length of stay (66 days vs 41 days).

Despite survival to discharge after index operation, Kabuki syndrome patients with single ventricle physiology remain at high risk of mortality and morbidity after cardiac surgery. However, they may be discharged without ventilator dependency and survive to toddler years.

Membranous subaortic stenosis is a CHD with high recurrence-rate despite surgical treatment. This study investigated the outcome of operated patients and possible predictors for recurrence.

Retrospective review of all patients (n = 38) ≤ 18 years of age operated for membranous subaortic stenosis between 1994–2019 at Sahlgrenska University Hospital. The primary outcomes were recurrence, reintervention, and mortality. Predictors of recurrence and reintervention were secondary outcomes.

Median age (range) at diagnosis, initial intervention, and last follow-up were 2.3 (0.003–17.2), 5.3 (0.03–17.5) and 17.5 (3.6–20.4) years, respectively. Median follow-up time was 9.9 (0.01–19.5) years. 61% were males, and 53% had other associated CHD. 19 patients (56%) developed recurrence and 7 (21%) underwent reintervention. One patient died peri-operatively. Age <5 years at first intervention increased the likelihood of reintervention. Postoperative peak/mean gradients were higher in patients with disease recurrence.

The median echocardiographic peak-/mean gradients at initial diagnosis, pre-, postoperative, and at last follow-up were 61/36, 83/50, 16/8, and 19/17 mmHg respectively (p < 0.0001 pre/post). Pre-/postoperative peak gradients were linearly correlated, decreasing by 80% pre-/postoperatively (p < 0.01). Presence of symptoms and the preoperative peak gradient were positively associated (p < 0.001) with a peak gradient threshold value of > 90 mmHg. The distance between the subaortic stenosis membrane and the aortic valve was inversely correlated to the preoperative peak-gradient (p < 0.01).

Reintervention following surgical intervention of membranous subaortic stenosis is common. A positive correlation exists between high pre- and postoperative peak-gradient. A low postoperative peak gradient may be important in avoiding recurrence.

Low neuromuscular fitness is documented in adolescents with CHD and may be associated with clinical and morphological factors, indicating the need to assess strength in this population.

To evaluate neuromuscular fitness with a multifactorial approach and its associations with other clinical and morphological factors in adolescents with CHD.

This is an observational, cross-sectional study with adolescents with CHD, aged between 10 and 18 years. Neuromuscular fitness was calculated by the sum of the z-scores of four strength tests. Clinical factors of CHD were assessed by medical records and questionnaire. The morphological factors assessed were waist-to-height ratio, sum of skinfolds, and upper arm muscle area. Descriptive statistics, analysis of covariance, and linear regressions were performed.

Sixty adolescents with CHD participated, aged 12,7 ± 2,1 years, 55% girls. Maximum isometric strength was inadequate in 33%, jump height (power) in 33%, abdominal muscle strength resistance in 78%, and upper limb muscle strength resistance in 27%. Neuromuscular fitness was inadequate in 89% (n = 53) of adolescents with CHD. In the unadjusted regression, neuromuscular fitness was associated with arm muscle area (β = 0,12; p = 0,02; R2adj = 0,08) and in the unadjusted and adjusted regression it was lower in cyanotic (vs. acyanotic) CHDs (β = −1,76; p = 0,03 R2adj = 0,24).

The findings reveal deficits in different presentations of musculoskeletal strength in a large proportion of adolescents with CHD, reinforcing the need to measure fitness from a broader perspective. Low muscle mass and the presence of cyanotic CHD may imply in reduced neuromuscular fitness in adolescents with CHD.

Chronic pulmonary regurgitation following tetralogy of Fallot repair burdens the right ventricle. This study evaluated early outcomes of pulmonary valve reconstruction using right atrial tissue versus standard transannular patch repair.

A retrospective analysis of 412 tetralogy of Fallot patients (2014–2024) was conducted: Atrial Valve Group (n = 205) underwent valve reconstruction; No-Valve Group (n = 207) received standard repair. Patients were followed for 1 year with echocardiographic assessments. Outcomes included right ventricular insufficiency, ventilation duration, and ICU stay.

Atrial Valve Group had lower right ventricular insufficiency at 12 months (9.3% vs. 19.8%, p = 0.004, OR = 2.39, 95% CI: 1.32–4.33), shorter ventilation times (6.1 vs. 18.0 hours, p < 0.001, Cohen’s d = 3.54), and reduced ICU stays (3.0 vs. 5.7 days, p < 0.001, Cohen’s d = 1.87), despite longer CPB durations (47.1 vs. 40.5 minutes, p = 0.02).

Right atrial tissue reconstruction reduces early and intermediate-term right ventricular dysfunction post-tetralogy of Fallot repair. Long-term studies are needed.

Alternate electrocardiogram acquisition with fewer leads lacks systematic evaluation in children. This study aims to determine if electrocardiograms with fewer leads maintain diagnostic accuracy in paediatrics.

This is a single-centre review of 200 randomly selected standard 12-lead electrocardiograms from our hospital database (2017–2020) for patients aged 2 weeks to 21 years. An overlay technique generated 8-lead (limb + V1/V6) and 6-lead (limb only) variations of the 12-lead tracings, resulting in a total of 600 electrocardiograms, which were then interpreted by two independent paediatric electrophysiologists.

In total, 18% (35/200) of the baseline electrocardiograms were abnormal. Intervals were measured in lead II for all electrocardiograms. Comparing 12-lead to 6- and 8-lead electrocardiograms, there was almost perfect agreement for specific rhythm identification (97.5–100%, κ 0.85-1). The 8-lead showed substantial agreement with 12-lead electrocardiograms when identifying specific electrocardiogram patterns (97.5–100%, κ 0.66–1). A similar degree of agreement was not demonstrated with the 6-lead variant. Utilising the 12-lead electrocardiogram as the gold standard, sensitivity and specificity of the 8- and 6-lead electrocardiogram were > 89% for specific rhythm identification. Specificity for specific pattern recognition was > 99% while sensitivity was < 90% for certain variables for both 6- and 8-lead electrocardiogram, likely due to smaller sample size and fewer abnormal electrocardiograms. There was high percent reader agreement (92.5–100%).

8-lead electrocardiograms provide comparable diagnostic accuracy to 12-lead electrocardiograms for children. This information holds potential for future technological advancements in electrocardiogram acquisition tailored specifically for paediatrics. Additional studies are required to further refine conventional electrocardiogram acquisition.

Giant coronary artery aneurysms and myocardial fibrosis after Kawasaki disease may lead to devastating cardiovascular outcomes. We characterised the vascular and myocardial outcomes in five selected Kawasaki disease patients with a history of giant coronary artery aneurysms that completely regressed.

Five patients were selected who had giant coronary artery aneurysm in early childhood that regressed when studied 12–33 years after Kawasaki disease onset. Coronary arteries were imaged by coronary CT angiography, and coronary artery calcium volume scores were determined. We used endocardial strain measurements from CT imaging to assess myocardial regional wall function. Calprotectin and galectin-3 (gal-3) as biomarkers of inflammation and myocardial fibrosis were measured by enzyme-linked immunosorbent assay.

The five selected patients with regressed giant coronary artery aneurysms had calcium scores of zero, normal levels of calprotectin and gal-3, and normal appearance of the coronary arteries by coronary computed tomography angiography. CT strain demonstrated normal peak systolic and diastolic strain patterns in four of five patients. In one patient with a myocardial infarction at the time of Kawasaki disease diagnosis at the age of 10 months, CT strain showed altered global longitudinal strain, reduced segmental peak strain, and reduced diastolic relaxation patterns in multiple left ventricle segments.

These patients illustrate that regression of giant aneurysms after Kawasaki disease is possible with no detectable calcium, normal biomarkers of inflammation and fibrosis, and normal myocardial function. Individuals with regressed giant coronary artery aneurysm still require longitudinal surveillance to assess the durability of this favourable outcome.

Glenn procedure carries low morbidity and mortality within stages of single-ventricle palliation. However, some patients with Glenn failure need a stage reversal, while others require unanticipated surgical interventions. Our understanding of perioperative factors and outcomes associated with such unexpected interventions is extremely limited.

Patients who underwent unexpected surgery after the Glenn procedure between January 2010 and December 2019 within the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) were identified with a subgroup analysis of those reverting to stage I physiology. Patient’s requiring reversal to stage I palliation were matched 1:5 with controls. Multivariable logistic regression analysis was performed to evaluate risk factors for reintervention.

A total of 16,913 patients underwent Glenn procedure with 1221 (7.2%) requiring unexpected cardiac surgical intervention and 95 (0.56%) patients required takedown to a stage I. Significant clinical and operative risk factors were identified for such unexpected interventions.

The overall mortality after Glenn procedure was 1.2%, while mortality after unexpected reintervention was 6.6% at 30 days and after Glenn takedown was 27.5% at last follow-up. Unexpected surgical intervention and right ventricular dominance were significant risk factors for mortality.

Unexpected reinterventions, including need for takedown after the Glenn procedure, are associated with significantly higher mortality. Further studies should focus on improving our patient selection, understanding the risk factors mechanistically, including impact of the right ventricle as systemic ventricle in order to avoid need for unexpected surgical interventions.

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.

Specialised training opportunities in paediatric cardiology are rare for advanced practice providers, creating an educational gap for novice practitioners. Standardised curricula have been cited as a beneficial intervention to optimally prepare these providers for highly specialised fields. We sought to understand the current onboarding practices of advanced practice providers in paediatric acute care cardiology to identify opportunities for curricular improvement.

A survey developed by a task force by the Pediatric Acute Care Cardiology Collaborative (PAC3) was distributed across PAC3 programmes in May 2023 to evaluate the onboarding practices of advanced practice providers at paediatric heart centres nationwide.

Survey responses reflected orienting practices at 19 paediatric heart centres representing varying programme and team sizes. Of the respondents, 32% felt their current model does not meet all the needs of the new team member. Key successful onboarding elements included a structured curriculum with goals and objectives, dedicated education time and materials, standardised assessments, and individualised learning in the presence of a supportive team. All respondents agreed that an online curriculum would be beneficial.

There is no national standardised educational pathway for advanced practice providers entering paediatric acute care cardiology practice. There are opportunities to develop a formalised curriculum with structured learner assessment at a national level, which could be modified at the institution or learner level to enhance current onboarding practices.

Individuals with 22q11 deletion syndrome have a mutation in the TBX1 gene. This is associated with reduced left pulmonary artery/right pulmonary artery ratio in animal models and in humans with structurally normal hearts.

A retrospective analysis was undertaken of patients who underwent surgical repair of Tetralogy of Fallot, truncus arteriosus, and interrupted aortic arch between 01/2007 and 12/2022. The left pulmonary artery/right pulmonary artery ratio on initial and most recent echocardiogram and initial and subsequent intervention on the left pulmonary artery were compared between patients with and without 22q11 deletion.

There were 134 included patients; 19 patients had the deletion (22q11 positive), and 115 patients did not have the deletion (22q11 negative). Tetralogy of Fallot was present in 8/19 and 101/115 patients, truncus arteriosus in 7/19 and 7/115 patients, and interrupted aortic arch in 4/19 and 7/115 patients. Patients who were 22q11 positive had a reduced left pulmonary artery/right pulmonary artery ratio on both the initial echocardiogram [0.88 (interquartile range 0.71, 0.97) versus 1.02 (interquartile range 0.92, 1.12); p < 0.001] and most recent echocardiogram [0.66 (interquartile range 0.62, 0.91) versus 1.01 (interquartile range 0.89, 1.16); p < 0.001] and were more likely to have intervention on the left pulmonary artery at their initial surgery (36% versus 8.7%; p = 0.003).

Patients who were 22q11 positive trended towards reduced left pulmonary artery/right pulmonary artery ratios and need for early surgical intervention on the left pulmonary artery in comparison to patients without 22q11 deletion negative patients.

Outcomes of single ventricle heart defects may be influenced by the morphological type of the hypoplastic ventricle. Recent multi-centre studies have targeted on outcomes of hypoplastic left heart syndrome with limited focus on outcomes of hypoplastic right heart syndrome. We aimed at studying the clinical outcomes of hypoplastic right heart syndrome in the recent era.

We performed a retrospective analysis of all hypoplastic right heart syndrome patients (n = 153) born between January 2010 and January 2023. Five-year transplant-free survival was compared with hypoplastic left heart syndrome patients without heterotaxy (n = 144) born during the same time.

Double-inlet left ventricle was the most common anatomic hypoplastic right heart syndrome subtype (n = 39, 25%). Twenty-six (17%) patients with hypoplastic right heart had associated heterotaxy. Five-year transplant-free survival was high for most groups (double inlet left ventricle: 100%, pulmonary atresia: 94%, Ebstein’s anomaly: 92%, tricuspid atresia: 90% respectively). The heterotaxy group had worse early outcomes with 3 deaths and 3 heart transplants, before Fontan completion. Heterotaxy was a significant risk factor for death/transplant prior to Fontan completion in hypoplastic right heart syndrome patients (p = 0.03). Patients with hypoplastic right heart syndrome with heterotaxy had significantly worse 5-year transplant-free survival (71%) when compared to hypoplastic right heart syndrome without heterotaxy (95%) and hypoplastic left heart syndrome without heterotaxy (75%), p < 0.001.

Hypoplastic right heart syndrome patients have excellent clinical outcomes and better early childhood survival than hypoplastic left heart syndrome in the current era, except when associated with heterotaxy. The survival advantage conferred by a single left ventricle appears to be negated by heterotaxy syndrome and should be strongly considered during caregiver counselling and medical decision-making.

Adult Kawasaki patients may require intervention for occlusive coronary artery disease. Some adverse effects of first-generation drug-eluting stent implantation with sirolimus have been reported in this population.

A total of nine lesions in eight (seven males, one female) patients who underwent stent implantations in this population between 2000 and 2021 were reviewed.

The age at stent implantation ranged from 31 to 47 years, with a median of 37 years. There were six lesions treated by primary percutaneous transluminal coronary interventions, and three by elective procedures. A coronary aneurysm was found in two lesions, and coronary artery calcification was found in all culprit lesions. The numbers of everolimus-eluting stents, sirolimus-eluting stents and bare metal stents were six, two, and one, respectively. As anti- thrombotic therapy, aspirin, clopidogrel, and prasugrel were given to four, three, and one, respectively. Warfarin was given to five patients. The follow-up ranged from 2 to 12 years, with a median of 4 years. Follow-up angiograms were performed for eight lesions, at 2 to 38 months, with a median of 11 months. The patency of the target vessel was confirmed in all eight vessels. Slight malapposition, and peri-stent contrast staining were found in two lesions each.

Acute coronary syndrome due to coronary artery lesions caused by Kawasaki disease occurred, even in lesions without any apparent coronary artery aneurysms. In our study, we show safe and efficacious placement of second-generation stent without adverse effects during the short-term follow-up, but long-term follow-up is needed to determine the efficacy and complication.

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.