To examine the prevalence of anxiety symptoms and associated functional impairment to adaptive skills among elementary-aged children with CHD and to determine the need for anxiety screening in this high-risk population.

In a single-centre retrospective, cohort design, caregivers reported anxiety symptoms using Conner’s scales and functional impairment to adaptive skills using the Adaptive Behavior Assessment System. A total of 194 children were stratified across two cohorts: early elementary (ages 3–6 years) and late elementary (ages 6–14 years). Descriptive statistics summarised the frequency of anxiety symptoms and functional impairment. Spearman’s correlations compared anxiety symptoms to functional impairment of adaptive functioning. Univariable logistic regressions examined demographic and clinical characteristics associated with anxiety symptoms.

The majority of patients presented with anxiety, early elementary (63%), and late elementary cohorts (78%). Functional impairment was moderately correlated with anxiety symptoms in the early elementary cohort (r s = −.42, 95% CI [−0.58, −0.21], p = <.001). Greater anxiety symptoms were associated with lower cardiac complexity at primary age of surgery in the late elementary cohort (OR = 12.15, p = 0.019). Lesser anxiety symptoms were associated with having private insurance (OR = 0.25, p = 0.014).

This study demonstrates anxiety symptoms are common and associated with functional impairment to adaptive functioning in younger children with CHD. No clear clinical predictors exist for anxiety symptoms or functional impairment; therefore, screening for anxiety symptoms may need to be added to standard clinical assessment of all children with CHD participating in neurodevelopmental follow-up.

The primary objective of this study was to determine whether Altmetric score, number of reads, and citations for paediatric cardiology manuscripts correlate with one another. A secondary objective was to determine the extent to which factors mediated citation number for paediatric cardiology manuscripts.

Data for this study came from manuscripts published in Cardiology in the Young (2010–2021). Data were extracted by using data shared on the journal website. Spearman’s correlation analyses were conducted between manuscript reads, citations, and Altmetric score. Regression analyses were conducted with number of citations as the dependent variable and year of publication, publication type, number of reads, and Altmetric score as independent variables.

A total of 2642 manuscripts were included in the final analyses. Reads and citations had poor correlation (r-value 0.32); reads and Altmetric score had negligible correlation (r-value 0.26); and Altmetric score and citations had negligible correlation (r-value 0.07). Year of publication was independently associated with number of citations (β –0.95, p-value <0.01). Manuscript type was independently associated with number of citations (β 1.04, p-value <0.01). Number of reads was independently associated with citations (β 0.01, p-value <0.01). Altmetric score was independently associated with number of citations (β 0.05, p-value <0.01).

This study describes the correlation of reads, citations, and Altmetric score in manuscripts published in Cardiology in the Young, demonstrating poor correlation, at best, between these metrics. Each bibliometric index seems to represent a different phenomenon of manuscript consumption. No single bibliometric index in isolation offers ample representation of manuscript consumption.

An early warning tool, the Congenital Heart Assessment Tool (CHAT), was designed in 2012 to support parental preparation before discharge, enhancing understanding of their infant’s complex CHD, the signs of deterioration to look out for and to support decision-making at home. Acceptability and feasibility of the tool were tested during 2013–2015 in a single centre.

To evaluate the wider implementation across four children’s cardiac centres of the CHAT for infants with complex CHD in the community setting.

A four-centre collaborative mixed-methods quality improvement project funded by The Health Foundation, during 2016–2018. A plan, do, study, act cycle of improvement was employed. This article reports on the planning phases, creation of a modified tool (CHATm); and the implementation phases, including retrospective case note review using CHATm; tabletop simulation exercise using CHATm and clinical implementation of CHATm.

Key findings included the benefit of using CHATm simulation for practitioners; the effectiveness of CHATm in predicting amber and red triggers, indicating parental actions and escalation of concerns to professionals. Parents using CHATm found it enhanced knowledge in preparation for going home, supported decision-making, and discussions with health care professionals.

Using the CHATm clinically identified the need to develop a structured model of assessment of parental suitability for home-monitoring programmes. A robust and nationally agreed training programme for all staff using CHATm was recommended to ensure successful and complete implementation in practice.

Joint programmes are an alternative model that may aid in improving congenital cardiac surgery outcomes while avoiding the potential resource and accessibility challenges that could result from regionalisation. This study aims to characterise current joint programmes, identify factors that are associated with joint programme success and failure, and gauge attitudes within the profession regarding joint programmes as an alternative.

A multiple choice survey with 23 standard questions for all programmes and additional 42 additional questions for each participant hospital in a joint programme was addressed to paediatric cardiac surgeons in the United States of America. Questions were designed to qualitatively and quantitatively characterise congenital cardiac surgery joint programmes.

Of the 34 unique congenital cardiac surgery programmes identified in this survey, 14 have participated in a joint programme and 50% of those joint programmes existed for more than 10 years. Most joint programmes (86%) participate or participated in a model where the hospital participants are engaged in a “mother–daughter” relationship in both perception and case volume distribution. In three out of four defunct joint programmes, there were case complexity limitations placed on partner institutions, but the now independent partner institutions operate with no limitation on complexity. Most (71%) hospital participants in a joint programme felt that the joint programme produced better outcomes than two separate programmes; however, among those who participate or have participated in a joint programme, only 18% felt that joint programmes were the optimal model for delivery of congenital cardiac surgical care.

Acute rheumatic fever is an autoimmune disease that develops due to streptococcal infection. The positive effect of breastfeeding on the development of the child’s immune system is well documented. In this study, we aimed to investigate the effect of breast milk intake period on the development of carditis.

Patients (n: 182) who were diagnosed with acute rheumatic fever between 2010 and 2019 were enrolled in the study. The patients were divided into groups according to carditis development. The demographic, socio-economic, and breastfeeding data were compared between groups.

The mean age of the patients was 10.5 ± 3.4, and 43.4 % (n: 79) of them were female. Independent predictors of the development of carditis in the first acute rheumatic fever episode were the number of children at home (OR: 1.773, CI 95%: 1.105, 2.845; p: 0.018) and breast milk intake less than 6 months (OR: 0.404, CI 95%: 0.174, 0.934; p: 0.034). Independent predictors of the development of carditis in any of the acute rheumatic fever episodes were the number of children at home (OR: 1.858, CI 95%: 1.100, 3.137; p: 0.021) and female gender (OR: 3.504, CI 95%: 1.227, 10.008; p: 0.019). The only independently predictor of the development of chorea during acute rheumatic fever was female gender (OR: 3.801, CI 95%: 1.463, 9.874; p: 0.006).

Although the occurrence of carditis is less common during the first acute rheumatic fever attack in patients with breast milk intake less than six months, this advantage is lost in recurrent attacks. This study showed that breast milk does not have a negative effect on acute rheumatic fever carditis.

This study aimed to analyse the results of paediatric aortic valve repairs in our institution.

The data of 57 patients under 18 years of age who underwent aortic valve repair between 2014 and 2019 were retrospectively analysed. Early postoperative reoperation and hospital mortality rates were evaluated based on the ages of the patients, their preoperative diagnoses, and the surgical techniques used. Survival curves for groups of patients were calculated by Kaplan–Meier analysis.

The rate of reoperation was 14% (n = 8), and there were no significant differences regarding valve pathologies and preoperative diagnoses (p > 0.05). Among the repair techniques, tricuspidisation was considered to be a risk factor for reoperation (p < 0.05). Augmentation, the material used (0.1 PTFE or pericardium), and the number of cusps were not found to have significant effects on reoperation or mortality. The mean follow-up period was 29.86 ± 21.30 months. The survival rates of the patients were 88%, 100%, and 88.2% for those with aortic stenosis, aortic insufficiency, and mixed disease, respectively, and no significant difference was found when these rates were evaluated with the log-rank test (p > 0.05). The mortality rate was 8.8% (n = 5), and undergoing surgery before the age of 1 year was found to be significant in terms of mortality (p = 0.032, p < 0.05). The bicuspid aortic valve group had the lowest mortality risk, while the Shone complex group had the highest.

With its acceptable reoperation and mortality rates, aortic valve repair should be the first choice of treatment in the paediatric age group. Early results were satisfactory in all groups.

Prolonged effusions post-Fontan procedure are associated with morbidity. Fontan patients have higher pro-inflammatory cytokines in chest tube drainage compared to controls. Colchicine, an anti-inflammatory medication, decreases effusions in adults after cardiac surgery. We hypothesised that patients post-Fontan treated with colchicine would have decreased pro-inflammatory cytokine levels and shorter duration of chest tube drainage.

This pilot clinical trial enrolled nine patients (intention to treat); five completed the protocol (per protocol). Post-operative Fontan patients 20 months to 5 years receiving colchicine were compared to a previously published control cohort (n = 25). Per protocol patients received 0.6 mg colchicine daily starting post-operative day 2, ending 1 day after chest tube removal. Chest tube samples were taken on days 1–4, 7 and 10, or until removal and analysed with a 17-cytokine Bio-Plex Assay. Descriptive statistics and basic univariate comparisons were made.

There was no difference in duration of chest tube drainage or length of stay between intention to treat patients and controls. Per protocol patients had shorter duration of chest tube drainage compared to controls (6 days [interquartile range 4.7–7], versus 10 days [7–11], p = 0.007) and shorter length of stay (7 days [5.5–9] versus 9 days [9–13], p = 0.005). Pro-inflammatory cytokines trended lower in per protocol patients.

In this pilot cohort, patients who completed the colchicine protocol post-Fontan procedure had shortened duration of chest tube drainage and length of stay. A decrease of pro-inflammatory cytokines may contribute to the mechanism of this change.

ClinicalTrials.gov: Colchicine in Postoperative Fontan Patients (CPFP); NCT03575572; https://clinicaltrials.gov/ct2/show/NCT03575572.

Prolonged pleural effusions are common post Fontan operation and are associated with morbidity. Fontan pleural effusions have elevated proinflammatory cytokines. Little is known about the chest tube drainage after a superior cavopulmonary connection. We examined the chest tube drainage and the inflammatory profiles in post-operative superior cavopulmonary connection patients.

This prospective cohort study enrolled 25 patients undergoing superior cavopulmonary connection and 10 age-similar controls. Data are also compared to 25 previously published Fontan patients and their 15 age-similar controls. Chest tube samples were analysed with a 17-cytokine BioPlex Assay. Descriptive statistics and univariate comparisons were made between groups.

Duration of chest tube drainage was significantly shorter in superior cavopulmonary connection patients (median 4 days, [interquartile range 3–5 days]) versus Fontan patients (10 days, [7–11 days], p < 0.0001). Cytokine concentrations were higher on post-operative day 1 in superior cavopulmonary connection patients versus Fontan patients (all p ≤ 0.01), however levels were comparable to age-similar controls. While proinflammatory IL 8, MIP-1β, and TNF-α concentrations increased in chest tube drainage of Fontan patients from post-operative day 1 to last chest tube day (all p < 0.0001), there was no change in these biomarkers in superior cavopulmonary connection patients, their controls, or Fontan controls.

Our study demonstrates that after superior cavopulmonary connection, proinflammatory cytokines in the chest tube drainage remain similar to biventricular controls of both age groups, unlike the significant rise over time observed in Fontan patients. Inflammation within the chest tube drainage is likely not innate to single ventricle patients.

Fetal arterial duct aneurysm, saccular, or fusiform enlargement of the arterial duct affect > 8% of pregnancies. It is uncommonly associated with serious sequelae postnatally, including thromboembolic events such as stroke and left pulmonary artery obstruction, rupture with demise, and vocal cord compression. Risk factors include maternal diabetes, late maternal age, maternal blood type A, large size for gestational age, and connective tissue disorders. The clinical importance remains unknown, making it difficult to determine how to monitor this finding postnatally.

This is a retrospective echocardiogram study assessing the outcomes of fetally diagnosed arterial duct aneurysm. Images and records were reviewed to confirm the diagnosis and assess risk factors and outcomes. Descriptive statistics were performed.

Fifty-three affected fetuses were identified. The median gestational age at diagnosis was 34.9 weeks (IQR 32.6, 36.6). The median maternal age was 31 years (IQR 27.3–34.1). Eight (15%) had maternal diabetes. The most common blood type was type O. The median maximal dimension of the aneurysm was 7.6 mm (IQR 6.1, 8.7). The aortic end was the maximal dimension in 67.9%. Median postnatal follow-up period was 76 days (IQR 7.5, 368). No patients sustained postnatal demise related to the duct, rupture of the ductal aneurysm, cerebral infarction, or other sequelae. No newborn had associated connective tissue disorders. No patients underwent ductal intervention.

In our experience, no adverse outcomes related to the ductal aneurysm were identified. This should be considered when counselling families about the need for postnatal follow-up.

Congenital heart defects (CHD) are the most frequent group of congenital anomalies representing a significant burden of mortality and morbidity and health service load.

In the Northern Ireland population, served by a single paediatric cardiology centre, we determine the prevalence and trends of CHD among live births.

This is a descriptive cross-sectional population-based study, using the paediatric cardiology database. The study included a total of 245,120 live births representing all children born in Northern Ireland 2005-2014.

A total of 11,410 children (4.65% of live births in Northern Ireland) received an echocardiogram for suspected CHD, and 3,059 children were subsequently diagnosed with a major CHD (prevalence = 12.48 per 1,000 live births (95% CI 12.04–12.93)) of whom 490 (16.02%) had genetic or chromosomal disorders including Down syndrome. The prevalence of non-genetic or chromosomal cases was 10.48 per 1,000 live births (95% CI 10.08–10.89) and did not change significantly over time (p = 0.91). The prevalence of CHD diagnosed in the first year of life was 8.46 per 1,000 live births (95% CI 8.10–8.83), which increased over time (p < 0.01). The prevalence of severe CHD was 2.02 per 1,000 live births (95% CI 1.85–2.21).

Northern Ireland has a high prevalence of CHD among European countries, which may be associated with complete ascertainment of both early and late diagnosed cases recorded in the paediatric cardiology database, as well as being one of the few European countries where terminations of pregnancy for foetal anomaly was illegal during the study period.

Chest pain, palpitations, and syncope are among the most common referrals to paediatric cardiology. These symptoms generally have a non-cardiac aetiology in children and adolescents. The aim of this study was to investigate the rate of common psychiatric disorders in children and adolescents referred to the paediatric cardiology clinic with chest pain, palpitations, and syncope and the relationship between cardiological symptoms and psychiatric disorders.

Children and adolescents aged 8–16 years who presented at the paediatric cardiology clinic with primary complaints of chest pain, palpitation, or syncope were included in the study. After a detailed cardiology examination, psychiatric disorders were assessed using the DSM IV-TR diagnostic criteria and a semi-structured interview scale (KSADS-PL). The Child Depression Inventory and Spielberger’s State-Trait Anxiety Inventory for Children were also applied to assess the severity of anxiety and depression.

The study participants comprised 73 (68.90%) girls and 33 (31.10%) boys with a mean age of 12.5 ± 2.4 years. Psychiatric disorders were determined in a total of 48 (45.3%) participants; 24 (38.7%) in the chest pain group, 12 (48.0%) in the palpitation group, and 12 (63.2%) in the syncope group. Cardiological disease was detected in 17% of the cases, and the total frequencies of psychiatric disorders (p = 0.045) were higher in patients with cardiological disease.

It is clinically important to know that the frequency of psychiatric disorders is high in patients presenting at paediatric cardiology with chest pain, palpitations, and syncope. Physicians should be aware of patients’ psychiatric problems and take a biopsychosocial approach in the evaluation of somatic symptoms.

CHDs are the most common type of birth defect. One in four newborns with a heart defect has a critical CHD. In Mexico, there is a lack of data available to determine its prevalence. Pulse oximetry screening programmes have been implemented worldwide, reporting opportunity areas in algorithm interpretation and data management. Our study aims to share preliminary results of a 3-year experience of a multicentre pulse oximetry screening programme that addresses critical challenges.

This retrospective study examined the reports of newborns screened from February 2016 to July 2019 from five hospitals. Two algorithms –the New Jersey and the American Academy of Pediatrics– were implemented over consecutive periods. The algorithms’ impact was assessed through the calculation of the false-positive rate in an eligible population.

A total of 8960 newborns were eligible for the study; from it, 32.27% were screened under the New Jersey and 67.72% under the American Academy of Pediatrics algorithm – false-positive rate: 1% (CI 95: ± 0.36%) and 0.71% (CI 95: ± 0.21%), respectively. Seventy-nine newborns were referred, six were diagnosed with critical CHD, and six with CHD. The critical CHD estimated prevalence was 6.69:10,000 newborns (CI 95: ± 5.36). Our results showed that the algorithm was not related to the observable false-positive rate reduction.

Other factors may play a role in decreasing the false-positive rate. Our experience implementing this programme was that a systematic screening process led to more confident results, newborn’s report interpretation, and follow-up.

Anomalous origin of pulmonary artery branch from ascending aorta (APA) in the presence of two separate semilunar valves is an uncommon entity necessitating early diagnosis and surgery to prevent development of irreversible pulmonary vascular disease. We evaluated our experience with the technique and outcome of 11 patients with this condition.

Between January 2000 and December 2019, 11 patients were diagnosed with APA. Echocardiographic data were collected from the records, including the site of origin of the anomalous pulmonary artery, additional defects, pulmonary artery pressures, and biventricular function. Intraoperative charts were reviewed for the details of the surgical procedure and cardiopulmonary bypass. Post-operative data included survival, ventilatory support, and duration of hospital stay. After discharge, children were reviewed at 1 month, 3 months, 6 months, and then at yearly intervals.

Of the 11 patients, females were more than males (7:4) with a median age of 6 months (15 days–28 years) and median weight of 5.7 kg (1.8–40 kg). Nine patients underwent direct re-implantation of anomalous pulmonary artery branch to main pulmonary artery. The survival rate was 88.8% in our series. On follow-up, no re-operations or re-interventions were required and all surgically corrected patients were in stable clinical condition.

Early- and midterm outcomes of children who underwent surgery for APA is convincing. Early direct re-implantation of the anomalous branch pulmonary artery to main pulmonary artery without any graft material is the optimal surgical strategy for these patients.

Congenitally corrected transposition of the great arteries is a complex pathology characterised by atrioventricular and ventriculo-arterial discordance. Optimal surgical approaches are still a matter of debate.

To evaluate the outcomes of different surgical treatments in a single centre.

Between 1998 and 2020, 89 patients were studied. The cohort was divided into three groups: physiologic, anatomic, and univentricular repair.

Physiologic correction (56.18%) was associated with significant tricuspid valve regurgitation progress (42%) and complete AV block (30%) compared to anatomic repair. Right ventricular systolic dysfunction was developed in 14%. Instead, anatomic correction (30.34%) (double switch 59% and Rastelli type 40.7%) presented moderate to severe aortic regurgitation (4%) and left ventricular systolic dysfunction (11%). Complete AV block was developed in 14.8%. Rate of reintervention was 34% for physiologic and 26% for anatomic. Univentricular palliation (13.8%) presented no complications or late mortality during the follow-up. The overall survival at 5 and 10 years, respectively, was 80% (95% CI 69, 87) and 75% (95% CI 62, 84). There was no statistically significant difference in mortality between the groups (p log-rank = 0.5752).

Management of congenitally corrected transposition of the great arteries remains a challenge. In this cohort, outcomes after physiologic repair were satisfactory in spite of the progression of tricuspid regurgitation and the high incidence of AV block. Anatomic repair improved tricuspid regurgitation but increased the risk of aortic regurgitation and left ventricular systolic dysfunction. The Fontan group showed the lowest incidence of complications.

Patients with univentricular heart defects require lifelong imaging surveillance. Recent advances in non-invasive imaging have enabled replacing these patients’ routine catheterisation. Our objective was to describe the safety and cost savings of transition of a tertiary care children’s hospital from routine invasive to routine non-invasive imaging of low-risk patients with univentricular heart defects.

This single-centre cohort study consists of 1) a retrospective analysis of the transition from cardiac catheterisation (n = 21) to CT angiography (n = 20) before bidirectional Glenn operation and 2) a prospective study (n = 89) describing cardiac magnetic resonance before and after the total cavopulmonary connection in low-risk patients with univentricular heart defects.

Pre-Glenn: The total length of CT angiography was markedly shorter compared to the catheterisation: 30 min (range: 20–60) and 125 min (range: 70–220), respectively (p < 0.001). Catheterisation used more iodine contrast agents than CT angiography, 19 ± 3.9 ml, and 10 ± 2.4 ml, respectively (p < 0.001). Controlled ventilation was used for all catheterised and 3 (15%) CT angiography patients (p < 0.001). No complications occurred during CT angiography, while they emerged in 19% (4/21) catheterisation cases (p < 0.001). CT angiography and catheterisation showed no significant difference in the radiation exposure. Pre-/post-total cavopulmonary connection: All cardiac magnetic resonance studies were successful, and no complications occurred. In 60% of the cardiac magnetic resonance (53/89), no sedation was performed, and peripheral venous pressure was measured in all cases. Cost analysis suggests that moving to non-invasive imaging yielded cost savings of at least €2500–4000 per patient.

Transition from routine invasive to routine non-invasive pre-and post-operative imaging is safely achievable with cost savings.