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 371 underwent hybrid procedures (HP) and 563 received the Norwood procedure. The findings demonstrated that both approaches achieved comparable survival rates at 1, 3, and 5 years. 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.

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.

Clinical outcomes of a standardised enhanced recovery after surgery protocol, including thoracic epidural analgesia, were studied in children undergoing trans-atrial cardiac surgery via the right mid-axillary thoracotomy approach.

This single-centre retrospective study examined 42 paediatric patients who underwent trans-atrial cardiac surgeries via the mid-axillary approach (2018 to 2020), of whom 30 received epidural catheters. The standardised enhanced recovery after surgery protocol included transesophageal echo-guided thoracic epidural catheter placement, multimodal analgesia, reduced opioid use, and planned early extubation and discharge. Clinical outcomes assessed included extubation times, postoperative analgesic requirements, ICU pain scores, hospital length of stay, and any complications related to regional anaesthesia or surgery.

Thirty patients received an epidural placed between the third and sixth thoracic interspace levels under transesophageal ultrasonography guidance. The median age was 42 months (range 3–156), and the median weight was 15.7 kg (range 4.9–61 kg). Epidural infusions were continued for a median of 52 hours postoperatively. The intraoperative fentanyl usage was a median of 4.5 mcg/kg (interquartile range (IQR) 2–9). Intraoperative extubation was achieved in 28 of the 30 patients. Median post-extubation pain scores in the first 6 and 12 hours were 0 (IQR 0), and postoperative rescue opioid requirements were low in epidural patients. There were no instances of tracheal reintubation, neuraxial blockade-related complications, or other anaesthesia-related adverse events.

Through our standardised and reproducible anaesthetic protocol, we achieved excellent and nearly pain-free recovery in paediatric patients undergoing trans-atrial cardiac surgeries via the mid-axillary approach.

This retrospective study aimed to investigate the prevalence of arrhythmia in patients presenting with palpitation to the paediatric emergency department of our hospital, which serves as an arrhythmia centre and to share the principles of their management.

Patients presenting with palpitations were retrospectively reviewed. Those diagnosed with arrhythmias received appropriate emergency interventions. Cardiac electrophysiological studies and ablation were performed when indicated.

Among 534 paediatric patients evaluated for palpitations, 140 (26.2%) were diagnosed with arrhythmias requiring antiarrhythmic treatment (Group 1). Within this group, 61 patients described palpitations lasting longer than one hour and/or heart rates too rapid to count, compared to only 35 patients in the not requiring antiarrhythmic treatment group (Group 2) (p < 0.001). Group 1 also demonstrated significantly higher rates of isolated palpitations (a single episode without accompanying symptoms or recurrent occurrences), recurrent palpitations, and palpitations ongoing at the time of paediatric emergency department admission (all p < 0.001).

Our study supports that, as in adults, the probability of arrhythmia increases in children when palpitations persist for more than an hour, occur at an uncountable rapid rate, present as isolated or recurrent episodes, or continue at the time of admission. This data highlights the importance of taking a detailed medical history once again. To our knowledge, this is one of the few studies to comprehensively examine both the acute management and long-term outcomes of arrhythmia in children, including the role of ablation therapy, making it a potentially valuable contribution to the existing literature.

Single ventricle pulmonary arteriovenous malformations are poorly understood and variably assessed in published literature. To improve our understanding of single ventricle pulmonary arteriovenous malformations and facilitate multi-centre studies, it will be necessary to have uniform clinical practice patterns among paediatric heart institutions.

The aim of this study was to assess paediatric interventional cardiologists’ clinical perspectives and practice patterns for diagnosing single ventricle pulmonary arteriovenous malformations.

We surveyed paediatric interventional cardiologists using the Congenital Cardiovascular Interventional Consortium listserv. A single survey was distributed electronically with two subsequent reminder emails. Voluntary participants completed the anonymous survey electronically via RedCap.

Among 253 Congenital Cardiovascular Interventional Consortium members, a total of 55 (21.7%) paediatric cardiology interventional attending physicians completed the survey. There was near unanimity (98%) that pulmonary arteriovenous malformations develop due to lack of hepatic vein blood flow to the lungs; however, there was wide variation among practice patterns. A minority (20%) of respondents perform bubble contrast echocardiograms (bubble studies) more than half the time pre-Fontan, whereas many (31%) almost never (< 5% of cases) perform bubble studies pre-Fontan. Most respondents reported that they did not perform bubble studies because results do not impact clinical decision making pre-Fontan (56%) or post-Fontan (60%). Many respondents (49%) do not have a typical volume of agitated saline that they inject for bubble studies.

Clinical practice patterns vary widely among paediatric cardiology interventionalists. A standardised clinical approach, new diagnostic tools, or both are needed to standardise our field’s approach to diagnosing, studying, and treating single ventricle pulmonary arteriovenous malformations.

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.

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.

Severe obesity in adolescents is a public health crisis of increasing concern. Cardiac maladaptation, such as left ventricular hypertrophy, increases the risk of future cardiovascular disease. This study aims to evaluate the impact of weight loss surgery on cardiac geometry in morbidly obese adolescents.

A single-centre, randomised controlled trial titled comparing laparoscopic adjustable gastric banding with multidisciplinary lifestyle intervention against a control group with multidisciplinary lifestyle intervention alone. The trial included severely obese adolescents aged 14–16 who had not responded to a minimum of 12 months of multidisciplinary lifestyle intervention. The primary outcome measured was the percentage change in total body weight one year after surgery. Left ventricular geometry was evaluated ultrasonographically before and one year after bariatric surgery.

In the intervention and control group, a total of 10 and 8 patients received both the baseline and follow-up ultrasonographic examination. BMI significantly decreased in the intervention group compared to the control group (−6.75; 95% CI: -10.28 – −3.23). Moreover, left ventricular mass index (−8.21; 95% CI: −15.43 – −0.98) and relative left ventricular wall thickness (−0.04; 95% CI: −0.07 – 0.00) significantly decreased within the intervention group, reflecting amelioration of adverse remodelling, albeit without statistically significant intervention effect when compared to the control group.

Significant weight loss through bariatric surgery may improve left ventricular geometry in severely obese adolescents. These findings support the potential of bariatric surgery not only for reducing weight loss but also for improving cardiac health.

The impact of arch anatomy on the prognosis of aortic coarctation of the aorta (COA) is not well established. We aim to assess the relationship between arch anatomy and the short- and long-term prognosis after surgical repair.

Patients with COA operated on the period between 11/2007 and 03/2016 were retrospectively recruited. Anatomical analysis of the aortic arch was done using Multidetector CT with measurement of the inter-branch distances between left common carotid artery, innominate artery, and left subclavian. We classified patients into group I, whose distance ratio (LCCA- IA)/(LSCA-IA) is short and less than 50%, and group II with such ratio ≥ 50%.

Seventy-three patients were recruited. The distance (LCCA-IA) had a range of Zero (common origin) to 22.3 mm. The distance ratio (LCCA-IA)/(LSCA-IA) ranged between Zero and 89%. Group I had a significantly higher incidence of adverse outcomes, including recoarctation, re-elevation of blood pressure, and re-elevation of pressure gradient, compared to Group II (p = 0.0001, 0.011, and 0.014, respectively). A positive correlation exists between the distance ratio and the residual SPG across the repaired segment (P = 0.0001). Only the anatomical distance ratio (LCCA-IA)/(LSCA-IA) can independently predict recoarctation in the long term.

There is a strong association between the anatomical distance LCCA-IA and recoarctation. This novel parameter is the only anatomical independent predictor of recoarctation.

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.

Medical advances have extended the lives of adults with CHD, but transitioning to adult healthcare remains challenging. Identifying factors related to lapses in care is crucial for adult females due to their unique healthcare needs.

We examined the relationships among length of lapses in primary care and cardiology services with quality of life and perceived health status for adult females with CHD.

A convergent mixed-methods design was used to examine thirty adult females with CHD, aged 21–30. Descriptive statistics and correlational analysis were used to examine perceived health status and quality of life based on lapse of care durations. Thematic analysis was used for qualitative data.

A 12-month or greater lapse in care occurred in 46.7% of participants for cardiology and 60% for primary care, and 30.0% lacked an established adult primary care provider. Participants cited healthcare system barriers, provider shortages, and difficult transitions from paediatric providers as contributing factors to lapses in care. Despite lapses, most participants reported similar or better physical health scores than the general population. However, those with either no or prolonged lapse in cardiology care had lower mental health scores. Participants with primary care lapses greater than 6 months reported better quality-of-life scores than those with uninterrupted care.

Significant gaps, which contribute to lapses of care, persist in transition care for females with CHD. Findings highlight the need for structured transition planning, improved primary care access, and an integrated primary-specialty care model to improve transition.

The timing for intervention in patients with significant chronic aortic regurgitation is based on adult guidelines and criteria which may not apply to children. There is limited data on the use of cardiac MRI parameters to guide surgical decision-making in paediatrics. We examined associations between MRI quantification of aortic regurgitation and left ventricular volumetric function and the need for surgical intervention.

Forty children and young adults with aortic regurgitation who had undergone cardiac MRI were divided into two groups based on aortic valve surgery (n = 20) or no surgery (n = 20). Ventricular volumetric functional parameters and aortic regurgitant volume and fraction were collected. Differences in MRI parameters between the groups were compared using unpaired t-tests. Receiver operating characteristic analysis identified MRI cut-off values with discriminatory ability towards primary end point of surgery (area under the curve > 0.7).

Patients who underwent surgery had significantly larger ventricular volumes and aortic regurgitant fraction than those without surgery. Aortic regurgitant fraction and volume had the highest discriminatory power (0.93 and 0.92, respectively) between the two groups, followed by indexed left ventricular volumes (end-diastolic volume 0.85 and end-systolic volume 0.89).

Current guidelines for surgical intervention in children with chronic aortic regurgitation are limited. Our findings suggest potential MRI-based threshold values that may aid in surgical decision-making and highlight the need future research for aortic valve surgery in children with chronic aortic regurgitation.

Scimitar syndrome is an uncommon congenital malformation of pulmonary venous drainage to the junction of the right atrium and inferior caval vein. Treatment is usually surgical, depending on the morphological variant. When there is dual drainage of the anomalous veins to the inferior caval vein and the left atrium, a transcatheter procedure may represent an alternative to surgery.

We report a series of four patients with the scimitar variant with dual pulmonary venous drainage treated with a transcatheter approach.

All four patients (three children, one adult) had dual drainage of right pulmonary veins into the inferior caval vein and to the left atrium via a connecting vein. All patients underwent a successful catheter occlusion of the anomalous connection to the systemic vein without complication. Vascular plug devices were used in two patients: a left atrial appendage closure device in one patient and a ventricular septal defect closure device in one patient. All the procedures resulted in complete occlusion of the anomalous venous drainage to the inferior caval vein and unobstructed drainage to the left atrium.

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.

Dexmedetomidine is frequently used in paediatric anaesthesia. This includes use in patients with CHD, but detailed analysis of haemodynamics after administration in these patients has not yet been published. We performed a systematic review and meta-analyses examining haemodynamic changes immediately after dexmedetomidine administration in patients with CHD.

We conducted a systematic review of PubMed, Embase, and Medline from inception until May 31, 2024. Inclusion criteria were studies that contained children with CHD who received dexmedetomidine for a cardiac procedure and reported at least one haemodynamic variable before and after administration of dexmedetomidine. Exclusion criteria were studies of noncardiac procedures. We performed a meta-analysis on each haemodynamic variable that was reported by at least four studies.

We screened 5383 abstracts. We included 85 studies for review, and 16 studies were accepted for four meta-analyses (heart rate, 16 studies, n = 408; systolic blood pressure, 11 studies, n = 280; diastolic blood pressure, 10 studies, n = 276; mean arterial pressure, 5 studies, n = 130). Analysis of heart rate, systolic blood pressure, and diastolic blood pressure showed a statistically significant reduction (p < 0.001), while there was no significant change in mean arterial pressure. The clinical difference was minimal with a decrease in heart rate of 11.3 beats per minute, and a decrease in systolic blood pressure/diastolic blood pressure of 5.9 and 6.2 mmHg, respectively. Heterogeneity was high in all analyses.

Dexmedetomidine is associated with small changes in heart rate, systolic blood pressure, and diastolic blood pressure in children with CHD. Further study is warranted.