Hypoplastic left heart syndrome is considered one of the most severe CHDs and occurs in approximately 2-3% of all CHD cases. Hybrid procedures have been introduced as an alternative to traditional surgical techniques, such as the Norwood procedure, particularly for neonates at high risk. Although hybrid approaches claim to reduce surgical risks and improve recovery, little is known regarding their comparative effectiveness and safety. This review aims to describe the contribution of hybrid procedures in hypoplastic left heart syndrome management regarding survival rates, postoperative complications, and quality of life, through recovery outcomes and long-term results, in light of conventional surgical techniques. A systematic review was carried out following the PRISMA guidelines. Data were retrieved from PubMed, Scopus, Web of Science, and Cochrane Library. The search articles were published from January 2014 to November 2024. Inclusion was focused on paediatric patients diagnosed with hypoplastic left heart syndrome and studies comparing hybrid procedures versus traditional surgical methods. All screening, data extraction, and quality assessment were done by two independent reviewers. This review analysed data from 11 studies comprising 934 neonates with hypoplastic left heart syndrome, of whom 371underwent hybrid procedures (HP) and 563 received the Norwood procedure. The findingsdemonstrated that both approaches achieved comparable survival rates at 1, 3, and 5years. Hybrid procedures were linked to slightly earlier interventions (standardised mean difference=-0.10, 95% confidence interval: -0.61 to 0.41, p = 0.77) and were favored for high-risk neonates due to reduced invasiveness. However, hybrid procedures showed a higher rate of interstage events (risk ratio = 0.81), 95% confidence interval: -0.62 to 2.25, p = 0.09) and higher rates of pulmonary artery stenosis requiring reinterventions (30% vs. 18% for Norwood). Norwood procedures were associated with fewer reinterventions and lower interstage event rates, highlighting their effectiveness for stable patients. Hybrid procedures offer a practical alternative to the Norwood procedure, especially for high-risk neonates. Although both approaches show similar long-term survival rates, hybrid procedures are associated with a higher risk of complications, including increased interstage mortality. These challenges highlight the need for continued advancements to refine hybrid techniques and to improve long-term outcomes. This review emphasises the critical role of tailored patient selection and calls for further research to enhance hybrid procedure protocols and optimise their effectiveness for specific patient populations.

A case of a 3-year-old asymptomatic male patient, who previously lost two brothers and was found to have a homozygote ryanodine receptor 2 gene mutation, is presented. The patient's initial electrocardiogram showed right bundle branch block, first-degree AV block, and left anterior hemiblock (trifascicular block), and echocardiographic examination revealed increased trabeculation in the left ventricle. Since the patient's mother and father were consanguineous, a homozygous missense mutation was detected in the ryanodine receptor type 2 gene within the scope of clinical exome sequencing in the genetic test performed. Considering the family history, a dual-chamber epicardial implantable cardioverter-defibrillator implantation was performed. This case highlights the treatment process of a patient with a significant family history and a ryanodine receptor type 2 gene mutation, commonly associated with catecholaminergic polymorphic ventricular tachycardia, aiming to raise awareness of the disease.

This case illustrates a rare presentation of Wilson's disease, as ventricular arrhythmia-induced syncope was the initial and only manifestation in a young child before the development of classic hepatic or neurological signs. It underscores the importance of considering metabolic and genetic aetiologies in paediatric arrhythmias, especially when accompanied by abnormal liver function. Moreover, early recognition and initiation of chelation therapy can prevent disease progression and enable timely screening and management of affected family members.

This case underscores an uncommon presentation of Pulmonary artery hypertension which, to our knowledge, has limited neonatal data. Its atypical course reveals diagnostic pitfalls that, if unrecognized, may delay effective therapy. This report highlights options for early recognition and management. It serves as a reminder that clinicians should maintain vigilance for a simple respiratory distress, even in settings where it is not expected. Ultimately, this case adds to the limited literature and could influence practice by expanding differential diagnoses, refining diagnostic algorithms, or prompting guideline modifications in similar clinical scenarios.

Atrial septal defect is commonly considered a minor CHD, but morbidity and mortality are higher compared to the background population. Maternal pre-eclampsia is associated with CHD in the offspring in large registry-based studies. However, the association between pre-eclampsia and atrial septal defects might be subject to detection bias, as many atrial septal defects are asymptomatic or might remain undiagnosed until late in life. We investigated the association between maternal pre-eclampsia and the risk of atrial septal defects in a population-based cohort of neonates examined with echocardiography. Neonates included in the Copenhagen Baby Heart Study, who were examined using transthoracic echocardiography within 30 days of birth, were systematically assessed for atrial septal defects and patent foramen ovale using a standardised algorithm. Using log-linear binomial regression and polytomous logistic regression, we compared the risk of atrial septal defects in neonates exposed to maternal pre-eclampsia with the risk in neonates not exposed to pre-eclampsia. Our study cohort included 12,354 neonates (mean age, 11 days), including 462 exposed to maternal pre-eclampsia. Atrial septal defect was found in 5.9% (n = 732) of the study cohort and compared with unexposed neonates, neonates exposed to maternal pre-eclampsia had a modestly increased risk of atrial septal defects (adjusted risk ratio 1.19, 95% confidence interval 0.83, 1.64). Estimates were robust to various exclusions in sensitivity analyses. There appears to be an association between maternal pre-eclampsia and atrial septal defect in the neonate in a population-based cohort of neonates.

Clinicians in paediatric cardiology have unique exposure to different pathologies and treatment based on geographic location and resource availability. "Cardiology Across Continents" is a virtual, case-based discussion series aimed at knowledge sharing across a variety of practice and resource settings. We report on a recent case discussion highlighting a rare cause of new-onset heart failure in a teenager, which is more frequently seen among African and Asian populations.

Beta blocker non-adherence is a key factor in precipitating cardiac events in patients affected by congenital long QT syndrome. Adherence has been examined in adults affected by congenital long QT syndrome but not in a large paediatric group. Patients with congenital long QT syndrome types 1 and 2 were identified using a database curated by the Inherited Cardiac Conditions team in Northern Ireland. Medication adherence was reviewed by contacting the patient's GP. A medication possession ratio was then calculated for the year. Adequate adherence was defined as a ratio of ≥0.8, and ideal adherence was defined as a ratio of ≥1.0. Risk factor analysis for poor adherence was performed using multivariable binary logistic regression. 99 patients' data was suitable for analysis, 71 had LQT1 (78%) and 28 had LQT2 (28%). The median age of the children involved was ten years old. Over 36,135 days the median medication possession ratio of this patient group was 0.92. 56 patients (57%) had at least adequate adherence, of these 44 patients (44%) had ideal adherence. In contrast 43 patients (43%) had less than adequate adherence and of these six patients (6%) were completely non-adherent. Increased deprivation was significantly associated with "less than" ideal adherence Odds Ratio (OR) 1.2 95% confidence intervals CI (1.1-1.4). Adherence in the paediatric cohort was mostly in the "adequate range." Increased deprivation is a risk factor for "less than" ideal adherence. A small minority of patients can be identified as completely non-adherent by checking prescription records. Future studies should focus on elucidating barriers and enablers to ideal adherence in this population.

Ventricular septal defect is the most common CHD and is complicated by pulmonary hypertension in about 5% of cases. Although long-term outcomes after repair are generally good, persistent pulmonary hypertension has been reported, especially before the widespread treat-and-repair strategy. We retrospectively analysed patients aged ≥13 years who underwent repair for ventricular septal defect with pulmonary hypertension at our centre (1970-2024). Pre- and post-operative hemodynamics were assessed. Patients were grouped by preoperative pulmonary vascular resistance (≥3 vs. <3 Wood units). Residual pulmonary hypertension was evaluated by echocardiography. We studied 115 patients (43 male, 72 female). Follow-up reached 47 years (median 18.5). Residual pulmonary hypertension occurred in 3/115 (2.6%). Median age at intracardiac repair was 5 months. No significant differences were seen between patients with and without residual pulmonary hypertension in age at surgery, preoperative estimated right ventricular pressure, mean pulmonary artery pressure, or postoperative estimated right ventricular pressure. Mean pulmonary artery pressure was higher in the pulmonary vascular resistance ≥3 group than in the <3 group (47 ± 13 vs. 38 ± 11 mmHg, p = 0.019), while postoperative estimated right ventricular pressure did not differ. Among the 3 with residual pulmonary hypertension, 2 underwent intracardiac repair after 1 year of age. Most patients with ventricular septal defect and pulmonary hypertension had resolution after intracardiac repair; however, residual pulmonary hypertension occurred in about 2.6% during long-term follow-up. Preoperative hemodynamics did not predict persistence. Long-term periodic follow-up remains important after surgery.

Patients with CHD are at risk for developing necrotising enterocolitis. Currently, no standardised approaches for identification, diagnosis, and treatment of necrotising enterocolitis exists, and there are varying rates and management strategies of necrotising enterocolitis across centres. We used the Paediatric Cardiac Critical Care Consortium to identify high- and low-performing centres based on necrotising enterocolitis rates and convened a necrotising enterocolitis working group. The aims of the group were to understand why variability exists, identify risk factors, and create a foundation for a prospective improvement project. Nine centres participated, and collaborative learning sessions were held with multidisciplinary input. REDCap surveys were disseminated to centres to create consensus among site practices and recommendations. The following topics were discussed: diagnosis, risk factors, and management. Diagnosis consensus suggests (1) Diagnosis would benefit from a comprehensive scoring tool, and (2) ultrasound may serve as a highly sensitive diagnostic tool for those at high risk with the absence of other radiologic findings of necrotising enterocolitis. Risk factor consensus suggests (1) those with ductal-dependent systemic blood flow are the highest risk, and (2) vasopressors with splanchnic constriction should be used with caution. Management consensus suggests (1) breastmilk be used first-line for feeding, 2) resume feeds 24-48 hours after a necrotising enterocolitis rule-out, and 3) surgical deference to physical examination and laboratory evaluation above radiographic findings. Variability exists in diagnosing necrotising enterocolitis and feeding approaches for at-risk patients. Opportunities exist for collaboration to standardise definitions, compare outcomes, identify risk factors, and create consensus on the management of necrotising enterocolitis.

Transcatheter interventions are becoming increasingly used to address postoperative residual lesions after Norwood procedure with Sano shunt. This is a single-centre retrospective review of the outcome of all cases with Sano shunt at our institution over a 6-years period (2017-2023) who underwent transcatheter interventions. Thirteen out of the total 34 patients (38%) needed transcatheter interventions. The most common interventions were left pulmonary artery balloon angioplasty (n = 6), balloon angioplasty of aortic recoarctation (n = 6), and Sano shunt stenting (n = 5). Left pulmonary artery size improved from 3 [IQR; 2-5] mm to 4.9 [IQR; 2.7-7.3] mm post-intervention (p-value = 0.068), and gradient from 28 [IQR; 25-33] mmHg to 11 [IQR; 10-13] mmHg (p-value = 0.109). Balloon angioplasty of aortic recoarctation improved vessel size from 6.7 [IQR; 4-9] mm to 9.5 [IQR; 7-13] mm (p-value = 0.066), and reduced peak-to-peak gradient from 22.3 [IQR; 10-39] mmHg to 7.6 [IQR; 4-14] mmHg (p-value = 0.109). Finally, Stenting of Sano shunt resulted in increased shunt size from 3.4 [IQR; 3.1-3.6] mm to 5.5 [IQR; 4.2-6] mm (p-value = 0.066), and improvement of the oxygen saturation from 71.3 [IQR; 69-74] % to 85.3 [IQR; 83-89] % (p-value = 0.066). There was one procedure-related death. Transcatheter intervention for patients post Sano shunt is feasible with good results, improving haemodynamics and oxygenation of the patients and thereby allowing them to come on the proper time for the second stage palliation.