Arrhythmic mitral valve prolapse is a rare yet potentially fatal syndrome. Recently, high-risk markers for malignant arrhythmias have been identified in adults with mitral valve prolapse, and a risk stratification scheme has been proposed. Little is known about the prevalence of arrhythmic mitral valve prolapse, the characteristics of the syndrome, and risk markers in children and young adults.

A database of a high-volume, tertiary congenital heart defects department was searched to identify all patients with MVP between 2018 and 2025 (8 years). Patients’ characteristics, including arrhythmic risk factors, AMVP diagnosis, and last survival, were noted.

Of 12,955 hospitalised patients, 52 were diagnosed with mitral valve prolapse (0.4%; median age 14y [IQR 6], 35% male). Arrhythmic mitral valve prolapse was diagnosed in 13 patients (25% of the mitral valve prolapse cohort). Phenotypic risk factors were highly prevalent (88.5%), but in the crosstab analysis, only an enlarged left atrium was associated with high-risk arrhythmic events (p = 0.0432). Furthermore, patients with arrhythmic events were significantly older (median 16y) than those without (median 13y; p = 0.019). One patient (2.7%, with concomitant LQTS2) presented with unstable ventricular tachycardia and received an ICD. During a median follow-up of 3 years (IQR 1–4.5), all 52 patients were alive.

One in 250 patients admitted to a tertiary invasive paediatric cardiology department has mitral valve prolapse. Among those with mitral valve prolapse, 1 in 4 presents with arrhythmic mitral valve prolapse. Although risk factors are common, outcomes among young arrhythmic mitral valve prolapse patients appear good. Overlapping with primary electrical disease may flag a worse prognosis. Identification of new risk factors for children is warranted.

There is currently no consensus on the optimal mapping technique for atrioventricular nodal re-entry tachycardia ablation.

This, the first of its kind, prospective randomised trial compared procedural characteristics and ablation outcomes between an anatomic approach for atrioventricular nodal re-entry tachycardia ablation and an approach guided by low voltage signals, local activation time, and the propagation wave collision.

A randomised, prospective, multi-centre clinical trial was performed at 5 paediatric cardiac centres. 3D mapping was used on all patients. After atrioventricular nodal re-entry tachycardia confirmation, patients were randomly assigned to either an anatomic-based approach or to a 3D mapping technique that assessed for low voltage, activation time, and propagation wave collision to select the initial ablation site. Patient and procedural characteristics were collected with up to a 2-year post-procedure follow-up.

In all, 70 patients were randomised: 37 within the voltage-propagation wave approach and 33 in the anatomic approach group. There was no significant difference between patient demographics or follow-up duration between groups. No significant difference was seen between duration of procedure, success rate, complications, or recurrences between techniques. There was a trend toward fewer ablation applications to initial success with the voltage-propagation technique (median of 2 vs 5). Conversely, there were significantly more total lesions placed for the voltage-propagation group. Typical atrioventricular nodal re-entry tachycardia trended towards fewer lesions to success and fewer recurrences than ablations for atypical atrioventricular nodal re-entry tachycardia or jump/echo.

Both techniques demonstrated an excellent acute success rate and a low recurrence rate. Voltage-propagation mapping trended toward fewer ablations to initial success and did not prolong the procedure time. This paediatric study suggests that both a traditional anatomical technique and a voltage-propagation technique can provide excellent clinical outcomes, especially for typical atrioventricular nodal re-entry tachycardia.

CHDs, affecting 1.1% of newborns, are the most prevalent congenital anomalies. Improved survival rates expose children with CHD to long-term risks such as metabolic and acquired cardiovascular disorders. Despite physical activity’s benefits, participation is often limited by real and perceived safety concerns. This study evaluates awareness and practice of physical activity among Omani children with CHD.

To quantify physical activity levels and identify influencing factors in Omani children with CHD.

This cross-sectional study, conducted at major Omani paediatric cardiology centres, surveyed parents of children aged 5–18 years with CHD attending clinics from January 2019 to January 2023. A validated questionnaire assessed activity levels and influencing factors. Children with recent surgery (<3 months), single ventricle, cardiomyopathies, or without parental consent were excluded.

Among 412 children, mean weekly physical activity was 2.18 hours. Parental participation in sports (β = 0.42, p < 0.001) and cardiologist encouragement (β = 0.38, p < 0.001) significantly increased activity levels. Children in houses (64% participation) were more active than those in apartments (34%, p = 0.004). Acyanotic CHD was associated with higher participation (66%) than cyanotic CHD (45%). Gender, parental education, and surgical history were not significant predictors.

Omani children with CHD engage in insufficient physical activity. Parental involvement and cardiologist guidance are critical drivers. We recommend targeted educational programmes and routine exercise counselling to enhance participation.

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.

This study evaluated the safety and efficacy of individualised interventional strategies in paediatric pulmonary arterial hypertension, focusing on the outcomes of atrial septostomy, reverse Potts shunt, and partial ASD closure in patients unresponsive to medical therapy or with rapid right ventricular deterioration.

A retrospective analysis included 12 paediatric patients who underwent 18 interventional procedures between 2019 and 2024. Pulmonary arterial hypertension was confirmed by right heart catheterisation, and pre- and post-procedural clinical, echocardiographic, and haemodynamic data were compared.

Interventions included graded balloon atrial septostomy (n = 10), partial ASD closure (n = 2), and reverse Potts shunt via ductal stenting (n = 1). All surviving patients showed clinical improvement with significant reductions in heart rate (p < 0.01), NT-proBNP (p = 0.008), mean right atrial pressure (p < 0.001), and RV/LV end-diastolic diameter ratio (p = 0.014), along with improvements in six-minute walk distance (p = 0.002), WHO functional class (p < 0.001), and TAPSE (p = 0.028). One patient (8.3%) died within days following atrial septostomy due to nonadherence to medical therapy. Three patients required repeat atrial septostomy because of shunt restriction or spontaneous narrowing.

Individualised interventional strategies guided by comprehensive haemodynamic assessment can slow disease progression and improve quality of life in paediatric pulmonary arterial hypertension. Partial ASD closure, as a novel approach in this cohort, reduces excessive left-to-right volume load while preserving a controlled right-to-left shunt, thereby enhancing haemodynamic stability and optimising outcomes. Timely implementation of interventional strategies before disease progression may help reduce mortality.

Parental adverse childhood experiences have been associated with poorer health outcomes for children in the general population. This single-centre study examined the prevalence of parental adverse childhood experiences in a sample of young children with single ventricle CHD, the associations between parental adverse childhood experiences and child health outcomes, and the moderating effects of parental stress and social support on the relationship between parental adverse childhood experiences and child health outcomes.

Parents (N = 72) responded to questionnaires assessing demographic characteristics, parental adverse childhood experiences, social support, and stress. Child health outcomes (hospital admissions, length of stay, missed appointments) were assessed via parent-report and medical record review.

Half of parents (52.8%) endorsed exposure to at least one adverse childhood experience. In univariate analyses, children whose parents had a history of adverse childhood experiences were 1.78 times as likely to have more parent-reported hospital admissions (p = 0.002) and 2.22 times as likely to have more missed visits (p = 0.03) compared to children of parents without a history of adverse childhood experiences. Parental social support significantly moderated the relationship between parental adverse childhood experiences and total hospital length of stay (p = 0.03). Specifically, for each unit increase in parents’ social support total score, parents with a history of adverse childhood experiences were likely to have an 83% reduction in their child’s length of stay.

Parental adverse childhood experiences may be associated with poorer single ventricle CHD outcomes. Strengthening parental social support may improve health outcomes for children with single ventricle CHD in the setting of pre-existing psychosocial risk.

Exercise capacity (VO2peak) predicts mortality in adult patients with CHD. There is a lack of paediatric exercise capacity data based on specific CHD lesions, limiting the ability to contextualise interpretation based on expected performance during testing. The primary aim of this study was to establish VO2peak percentiles for paediatric patients with repaired CHD undergoing treadmill-based cardiopulmonary exercise testing (CPET).

Retrospective analysis of CPET data from 2004 to 2022. CPETs were analysed for patients with CHD aged 6–18 years. Patients with repaired CHD were categorised based on their most haemodynamically significant CHD lesion. Percentiles and age-based trends were plotted for each group.

A total of 887 patients were included. CHD patients were divided into ten diagnostic subgroups. The mean percent expected VO2peak for each of the subgroups were as follows: Atrial and ventricular septal defect (94.5 ± 25.1%), pulmonary valve repair (88.1 ± 18.4%), aortic valve repair (92.7 ± 16.4%), tricuspid and mitral valve repair (81.3 ± 20.4%), coarctation of the aorta (93.6 ± 18.8%), transposition of the great arteries (90.5 ± 19.4%), double outlet right ventricle and truncus arteriosus (80.5 ± 16.2%), tetralogy of Fallot (85.6 ± 20.9%), left ventricle dominant Fontan (74.7 ± 18.3%), and right ventricle dominant Fontan (75.7 ± 16.7%).

There is a varying degree of reduced exercise capacity in paediatric patients with repaired CHD. Univentricular hearts and tricuspid and mitral valve repair have the lowest VO2peak. These CHD-specific percentiles may help providers risk-stratify and counsel patients with CHD.

This study aimed to identify echocardiographic predictors of successful weaning from extracorporeal membrane oxygenation in paediatric and congenital heart disease patients.

We retrospectively analyzed pediatric patients who underwent venoarterial extracorporeal membrane oxygenation for cardiogenic shock or postoperative support between March 2018 and September 2023. Clinical and echocardiographic variables assessed at the time of weaning evaluation were compared between patients who were successfully weaned and those who were not.

Among the 46 enrolled patients, 31 were successfully weaned from extracorporeal membrane oxygenation. The mean age at extracorporeal membrane oxygenation initiation was 9.6 ± 13.9 years, and the mean duration of support was 12.3 ± 12.1 days. Patients in the successfully weaned group had significantly higher left ventricular ejection fraction (50.9 ± 16.4% vs. 27.3 ± 18.7%, p < 0.001) and higher velocity time integral at the left ventricular outflow tract (12.3 ± 8.0 cm vs. 4.1 ± 3.6 cm, p = 0.001) compared with the unsuccessfully weaned group. The cutoff values for predicting successful weaning were a left ventricular ejection fraction of 43.03% (sensitivity, 74.2%; specificity, 86.7%) and a velocity time integral of 4.45 cm (sensitivity, 92.0%; specificity, 66.7%).

Left ventricular ejection fraction and velocity time integral provide valuable echocardiographic information for predicting successful weaning from extracorporeal membrane oxygenation in pediatric patients and may support clinical decision-making during weaning assessments.

There is currently no established normative data for cardiopulmonary exercise parameters in the semi-supine position. There is conflicting data regarding the impact of a semi-supine body position on the semi-supine recumbent ergometer on cardiopulmonary exercise parameters. The goal of the current study was to match semi-supine recumbent ergometer tests completed in children/adolescents with those completed on the cycle ergometer and treadmill to identify differences in cardiopulmonary exercise parameters between devices.

Maximal semi-supine recumbent ergometer tests were matched by demographics (age, race/ethnicity, sex, height, weight, and body mass index) to tests completed on the cycle ergometer and treadmill. Groups were compared with two-sample T-tests for numeric variables and Fisher’s exact tests for categorical variables.

There was no difference in demographics between groups. Peak cardiopulmonary exercise parameters (watts, oxygen consumption, heart rate, blood pressure, oxygen saturation, minute ventilation, respiratory exchange ratio, respiratory rate, and anaerobic threshold) were unchanged between semi-supine recumbent ergometer and cycle ergometer, but the ventilatory equivalent of carbon dioxide was higher on the cycle ergometer versus semi-supine recumbent ergometer. Anaerobic threshold, peak oxygen consumption, and peak minute ventilation were lower on the semi-supine recumbent ergometer than on the treadmill.

The uniformity in nearly all cardiopulmonary exercise parameters between the semi-supine recumbent ergometer and cycle ergometer suggests that normative data for the cycle ergometer are a reasonable surrogate for normative data on the semi-supine recumbent ergometer until semi-supine recumbent ergometer-specific normative data are developed.

This study aimed at investigating the clinical, individual, and systemic factors influencing paediatricians’ and family physicians’ clinical decision-making process in the vaccination of children during infection from the physician’s perspective.

A qualitative study through semi-structured in-depth interviews was conducted among 10 paediatricians and 10 family physicians working in Ankara, Türkiye. The audio-recorded interviews were translated into written texts, and the obtained data was analysed using the thematic analysis method proposed by Braun and Clarke.

Four main themes were identified in of thematic analysis: (I) Impact of clinical conditions on vaccination decisions, (II) attitudes of families and their communication processes with physicians, (III) impact of practice settings and institutional factors, and (IV) vaccine postponement and compensation approaches. It was observed that the decision-making processes of the paediatricians were mainly based on the clinical evaluation criteria, while family physicians considered the expectations of the families and institutional conditions. Also, the importance of establishing effective communication with vaccine-hesitant families has been emphasized by both groups of physicians.

In the immunization of infected children, decision-making is shaped in addition to medical facts in relation to the parental attitude, organizational factors within health institutions, and personal experiences of medical staff. Decisions of paediatricians are largely grounded in medical facts, whereas family practitioners assess that social and organizational factors are of higher importance. Improved adherence to medical guidelines and communication competencies of medical professionals can contribute towards medical practice consistency.

Infective endocarditis is a leading cause of morbidity and mortality in children and adolescents with underlying CHD. Appropriate diagnostic workup and management in the inpatient setting can be challenging in this patient population due to the spectrum of disease complexity and the dynamic nature of the field. Therefore, the Paediatric Acute Care Cardiology Collaborative has undertaken the creation of this clinical practice guideline.

A panel of paediatric cardiologists, infectious disease specialists, intensivists, advanced practice practitioners, pharmacists, cardiothoracic surgeons, and a dentist was convened. The literature was systematically reviewed for relevant articles on the management of infective endocarditis in patients with CHD. Using the modified Delphi technique, recommendations were generated and put through iterative Delphi rounds to achieve consensus for inclusion.

Based on 127 articles that met the inclusion criteria, 82 recommendations were generated, 50 of which achieved consensus for inclusion and are included in this guideline. They address risk factors specific to CHD lesion type and prior interventions including implanted material, diagnostic considerations, management strategies, and recommendations on counselling other healthcare providers, patients, and families. Of the 50 consensus recommendations, 36 are strong recommendations, though 20 have low or very low quality of evidence.

A central theme in this guideline is that an individual’s specific CHD lesion and prior interventions must be carefully considered for risk stratification, diagnostic approach, and management. While most are strong recommendations, many are supported by low quality of evidence, emphasising the need for further research in this subject.

To map the scope, methods and focus areas of qualitative research in paediatric otolaryngology.

A Preferred Reporting Items for Systematic Reviews and Meta-Analyses-compliant systematic mapping review searched MEDLINE, Embase, CENTRAL and PsycInfo (August 2025) for qualitative or mixed-methods studies with a qualitative component related to paediatric otolaryngology. Two reviewers independently applied the inclusion criteria. Key study characteristics were extracted; no formal risk-of-bias assessment was performed, in line with the aims of a mapping review.

Eighty-nine studies were included. Publications rose sharply after 2015, with nearly three-quarters from the USA, Canada and the UK. Otology (49 per cent) and laryngology (40 per cent) predominated; common topics were hearing loss, tonsillectomy and tracheostomy. Interviews, mainly semi-structured (73 per cent), were the dominant method, and caregivers were the most frequent participants (62 per cent).

Qualitative research in paediatric otolaryngology is growing but remains geographically and methodologically narrow. Broader stakeholder inclusion and methodological diversity are needed to deepen understanding and support patient-centred care.

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.

Tonsillectomy is a common paediatric procedure with potential morbidity, notably post-operative pain and bleeding. One proposed factor influencing these outcomes is the surgeon’s level of training.

To evaluate whether surgical training level affects short-term outcomes in paediatric tonsillectomy.

In a prospective randomised study (2019–2022), outcomes were compared in children undergoing cold dissection tonsillectomy performed by either an attending or a supervised resident. Measured outcomes included duration of analgesia use, time to resume oral intake, length of hospital stay and post-operative bleeding.

A total of 115 children were included in the study, of which 60 (52.2 per cent) were operated on by residents and 55 (47.8 per cent) were operated on by attending surgeons. Baseline characteristics were similar. Operations by attending surgeons were shorter (20.4 vs 29.1 minutes), but no significant differences were found in post-operative pain, diet resumption, length of stay (1.1 days) or bleeding.

Supervised residents achieve comparable outcomes to attending surgeons. Surgical training level does not impact tonsillectomy outcomes under supervision.

To determine the feasibility of developing and implementing a multidisciplinary bootcamp for early-career Paediatric Cardiac Critical Care practitioners.

A one-day pre-conference bootcamp at the Pediatric Cardiac Intensive Care Society Annual Meeting in December 2022.

Physicians, fellows, nurses, advanced practice registered nurses, and respiratory therapists who work primarily in paediatric cardiac critical care units.

A modified Delphi needs assessment with interprofessional content experts for the development of a mixed didactic and simulation-based bootcamp at the Pediatric Cardiac Intensive Care Society Annual Meeting in December 2022, with pre- and post-testing to evaluate knowledge gain and additional surveys to assess perceived value.

Eighty-three course participants came from a variety of professions and represented institutions nationally and internationally. Most participants (77%) had two or more years of professional experience, aligning with the bootcamp’s focus on advanced learners. The bootcamp received strong participant evaluations: 84.1% (37/44) strongly agreed that the bootcamp improved their clinical knowledge. Ninety-seven percent (43/44) reported increased confidence in the ability to care for paediatric cardiac critical care patients. The bootcamp demonstrated a significant improvement in participant knowledge, with pre-test scores averaging 54.9% (95% CI: 49.9–59.9) compared to post-test scores of 64.5% (95% CI: 59.7–69.2), achieving statistical significance (p < 0.05).

Our pilot bootcamp has shown an improvement in immediate knowledge retention with valuable insights gathered to enhance future bootcamps. The results of this advanced interprofessional bootcamp evaluation will inform future iterations for providers in paediatric cardiac critical care.