To evaluate the safety and efficacy of two-stage arterial switch operation using bidirectional Glenn anastomosis combined with pulmonary artery banding in late-diagnosed dextro-transposition of the great arteries with intact ventricular septum.

This single-centre retrospective study included 24 children with late-diagnosed dextro-transposition of the great arteries with intact ventricular septum treated between 2020 and 2025, all with left ventricular mass index <35 g/m2 and banana-shaped geometry. Demographics, interventions, imaging, postoperative course, and follow-up data were collected. Haemodynamics, left ventricular retraining, complications, and survival were evaluated.

The cohort included 9 boys (37.5%) and 15 girls (62.5%), with a median age at first-stage surgery of 5 months (4–9.75). Patent ductus arteriosus was more common in patients without prior interventions (61.54% vs. 0%, p = 0.002). Additional surgical interventions performed concurrently with the first-stage BDG + pulmonary artery banding differed by prior-intervention status (p = 0.016). One mortality (4.17%) occurred due to sepsis in the no-prior group. Twenty-three patients proceeded to second-stage arterial switch (median interval 15 months, median age 22 months). In the prior-intervention group, post-second-stage ICU stay (median 5 vs. 4 days, p = 0.007) and inotrope use (median 4 vs. 3 days, p = 0.027) were longer. Two patients (8.7%) required ECMO (both prior group, resolved). At follow-up (1–4 years), all survivors were NYHA class I, with mild neo-aortic insufficiency in 52.17% and mild neo-pulmonary insufficiency in 13.04%.

Bidirectional Glenn anastomosis combined with pulmonary artery banding is a safe and effective strategy for gradual left ventricular retraining in late-diagnosed dextro-transposition of the great arteries with intact ventricular septum. It reduces perioperative morbidity and provides good survival and functional outcomes, which is valuable in resource-limited settings.

The choice between transcatheter and surgical pulmonary valve replacement for young adults with repaired tetralogy of Fallot who develop significant pulmonary valve insufficiency is challenging. Decision analytic modelling may be used to simulate long-term outcomes and suggest influential clinical thresholds for decision-making. A Markov model was constructed to compare the 5-year outcomes for a hypothetical cohort of 18-year-old patients.

A Markov model was constructed to simulate 10,000 hypothetical patients undergoing either transcatheter pulmonary valve replacement or surgical pulmonary valve replacement. Model inputs were abstracted from contemporary literature on the 5-year horizon. Outputs were used to derive an incremental cost-effectiveness ratio. Sensitivity and threshold analyses were performed to identify factors that would hypothetically change management.

From modelling, surgical pulmonary valve replacement had superior survival, lower incidence of endocarditis, and lower reintervention rate compared to transcatheter pulmonary valve replacement at 5 years. Surgical pulmonary valve replacement yielded lower cumulative postprocedural costs ($10,767 versus $14,528) and greater quality-adjusted life years (3.16 versus 3.12 QALYs) than transcatheter pulmonary valve replacement. The calculated incremental cost-effectiveness ratio (−88,743$/QALY) identified surgical pulmonary valve replacement as the preferred strategy at baseline. Sensitivity analysis demonstrated that transcatheter pulmonary valve replacement would be the preferred strategy if either the post-transcatheter pulmonary valve replacement endocarditis rate or the post-transcatheter pulmonary valve replacement surgical reintervention rate were reduced to 0%/month.

Comprehensive modelling of diverse outcomes showed that surgical pulmonary valve replacement had superior mid-term cost-effectiveness outcomes compared to transcatheter pulmonary valve replacement for young adults with repaired tetralogy of Fallot and pulmonary valve regurgitation. Sensitivity analysis found that the prevalence of post-transcatheter pulmonary valve replacement endocarditis and post-transcatheter pulmonary valve replacement surgical reintervention were influential outcomes for centres to consider when choosing between these strategies.

Systemic inflammation plays an important role in the pathophysiology of hypertension, contributing to endothelial dysfunction and target organ damage. This study aimed to evaluate the diagnostic and predictive value of the systemic immune inflammation index, neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, and C-reactive protein-albumin ratio in children with primary hypertension by comparing them with healthy controls, assessing their association with disease severity, and examining changes after treatment.

This retrospective study included 49 paediatric patients with newly diagnosed primary hypertension and 50 age- and gender-matched healthy controls. Complete blood count derived indices and biochemical markers were analysed. Patients were stratified by hypertension stage, and post-treatment changes in inflammatory indices were evaluated.

The mean age was 16.3 ± 1.9 years in the hypertension group and 15.8 ± 1.3 years in the control group. Systemic immune inflammation index, neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, and C-reactive protein-albumin ratio were significantly higher in children with hypertension than in controls (p < 0.05). Systemic immune inflammation index was markedly higher in stage 2 compared with stage 1 hypertension (p = 0.003) and decreased significantly after treatment (p < 0.001). Neutrophil-to-lymphocyte ratio and C-reactive protein-albumin ratio emerged as strong diagnostic markers, while systemic immune inflammation index, although less discriminative, was associated with disease severity and decreased significantly after treatment.

Neutrophil-to-lymphocyte ratio and C-reactive protein-albumin ratio provide robust diagnostic value in paediatric hypertension. Systemic immune-inflammation index, despite modest diagnostic accuracy, has prognostic significance by indicating disease severity and monitoring treatment effects. Collectively, these markers may serve as cheap and accessible tools for early diagnosis, risk stratification, and follow-up in children with hypertension.

Childhood-onset cardiomyopathies are rare and not well characterised. This study aimed to describe the clinical features of a paediatric cohort with primary cardiomyopathies, with a particular focus on aetiology and both short- and long-term outcomes.

A retrospective descriptive study was conducted, including patients diagnosed with primary cardiomyopathies before the age of 18. Clinical presentation, aetiology, and outcomes were analysed for each morphological subtype of cardiomyopathy.

A total of 76 patients met the inclusion criteria. Dilated cardiomyopathy was the most common subtype (48.6%), followed by hypertrophic (31.5%), left ventricular non-compaction (10.5%), restrictive (5.2%), and arrhythmogenic cardiomyopathy (3.9%). The mean age at diagnosis was 6.3 ± 5.6 years, with a slight female predominance (56.6%). The rate of genetic diagnosis was 25.6%; the most commonly identified pathogenic or likely pathogenic variants were in MYH7, FLNC, TTN, and MYBPC3, across different morphological subtypes. A total of 94.7% of patients received at least one cardiovascular medication, and 9.2% received intracardiac devices. The overall mortality rate was 22.3%, and the heart transplant rate was 15.7%.

These findings highlight the heterogeneous aetiology of paediatric cardiomyopathies and the variability in outcomes according to morphological, genetic, and clinical subtypes. The results underscore the importance of individualised evaluation and management for affected patients.

Interatrial stenting has become an essential intervention for children with CHD requiring sustained interatrial communication to optimise systemic and pulmonary haemodynamics. This study aimed to evaluate procedural success, clinical outcomes, and factors influencing haemodynamic improvement in a single-centre paediatric cohort.

A retrospective study was conducted on 16 patients who underwent interatrial stenting between 2018 and 2024. Patients were categorised by age as Group 1 (≤12 months, n = 10) and Group 2 (>12 months, n = 6). Demographic, procedural parameters, and follow-up data were analysed.

Sixteen patients (median age 10.5 months, median weight 5.0 kg) underwent interatrial stenting. The most frequent diagnosis was borderline left ventricle (37.5%), followed by hypoplastic left heart syndrome (25%) and myocarditis (12.5%). Procedural success was achieved in 15 of 16 patients (93.8%), with one acute failure due to stent embolisation. The median radiation exposure was 244.5 mGy (interquartile range 123.8–447.0). Radiation exposure correlated positively with patient weight (rs = 0.596, p = 0.015). During a median follow-up of 17.5 months, six patients (37.5%) died, and two required redilation. No stent thrombosis occurred.

Interatrial stenting is a safe and effective palliative intervention for paediatric CHD, providing significant haemodynamic improvement and bridging to definitive surgical management.

Aortopulmonary window is an uncommon CHD that often coexists with additional cardiovascular anomalies. Criss-cross heart, defined by rotation of the ventricular mass with crossed atrioventricular inflow streams, is exceedingly rare. The coexistence of aortopulmonary window and criss-cross heart is exceptional and markedly increases diagnostic and therapeutic complexity.

We report a full-term neonate with prenatal suspicion of aortopulmonary window. Postnatal echocardiography and CT demonstrated situs solitus, concordant atrioventricular and ventriculoarterial connections, rotational malalignment of the ventricles consistent with criss-cross heart, an inferior sinus venosus atrial septal defect, and a large type III aortopulmonary window. There was marked right-sided dilation and evolving right ventricular dysfunction, although no interrupted aortic arch was identified. At 17 days of life, the patient underwent early surgical repair with reconstruction of the aortic pathway. Despite anatomically successful correction, the immediate postoperative course was complicated by severe ventricular dysfunction and refractory low cardiac output syndrome, requiring venoarterial extracorporeal membrane oxygenation for haemodynamic stabilisation. Extracorporeal membrane oxygenation was discontinued on day nine, and follow-up imaging showed preserved biventricular systolic function and no residual aortopulmonary window.

This case illustrates an exceptionally rare association between aortopulmonary window and criss-cross heart, underscoring the value of prenatal detection and detailed postnatal imaging to delineate complex anatomical relationships. Early surgical intervention was mandatory, and the need for extracorporeal membrane oxygenation highlights the high intrinsic risk of this anatomical combination. Reporting such cases expands the limited literature on rare congenital cardiac malformations and may inform future refinements in diagnostic evaluation and perioperative management.

Shone complex is a rare congenital cardiovascular anomaly characterized by multiple left-sided obstructive lesions. Adult presentation is uncommon and may result in atypical clinical manifestations, particularly when complicated by infective endocarditis. Coarctation of the aorta–associated infective endocarditis is especially rare and carries significant clinical risk.

We report a 25-year-old male with partial Shone complex consisting of a parachute mitral valve, mitral stenosis, and coarctation of the aorta. The patient presented with persistent high-grade fever and was diagnosed with methicillin-sensitive Staphylococcus aureus infective endocarditis based on positive blood cultures. Transthoracic and transesophageal echocardiography revealed obstructive left-sided lesions, mitral valve pathology, and moderate aortic regurgitation. Positron emission tomography demonstrated intense hypermetabolic activity at the coarctation site, consistent with active infection and confirming a saccular aneurysm. The patient underwent prolonged intravenous cefazolin therapy followed by surgical repair. The early postoperative course was stable; however, seven months later he re-presented with recurrent fever. Repeat imaging demonstrated graft infection, vegetation on the prosthetic material, and a perforated mitral leaflet. A second course of intravenous antibiotics (cefazolin and rifampicin) was administered, followed by long-term suppressive oral therapy. Subsequent follow-up showed persistent moderate-to-severe mitral regurgitation with stable postoperative anatomy.

This case highlights a rare adult manifestation of partial Shone complex complicated by coarctation of the aorta-site methicillin-sensitive Staphylococcus aureus infection, saccular aneurysm formation, and recurrent graft-associated infective endocarditis. The clinical course underscores the importance of vigilant multimodality imaging, early multidisciplinary evaluation, and long-term follow-up in adults with complex CHD, particularly when infectious complications are present.

Sinus venosus atrial septal defect is an uncommon congenital cardiac anomaly frequently associated with partial anomalous pulmonary venous drainage. The coexistence of this defect with anomalous hepatic venous drainage, cor triatriatum, and two arterial ductal connections is extremely rare and may create diagnostic and surgical challenges.

An eleven-month-old female infant with tachypnoea and growth retardation was found to have sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage and anomalous hepatic venous drainage to the coronary sinus on CT angiography. Cardiac catheterisation demonstrated a moderate left-to-right shunt and mildly elevated pulmonary artery pressure. Intraoperative assessment unexpectedly revealed two arterial ductal connections and cor triatriatum. Surgical management consisted of closure of the atrial septal defect, rerouting of pulmonary veins, and ligation of both arterial ductal connections. Hepatic venous drainage was left unaltered due to low flow and lack of hemodynamic effect. Postoperative recovery was uneventful.

This rare constellation of anomalies highlights the importance of advanced imaging, careful preoperative planning, and intraoperative vigilance for unanticipated findings in CHD.