The increased survival rate among individuals with CHD has sparked interest in their transition to adult healthcare. Although there is a general agreement on the importance of transition interventions, the empirical evidence supporting them is insufficient. Therefore, this study aimed to conduct a systematic review and meta-analysis of transition interventions for adult healthcare in adolescents and young adults.

A literature search was conducted for studies comparing the quantitative effects of transition interventions with control groups, published up to March 15, 2023, in major databases (CENTRAL, Embase, PubMed, Web of Science, CINAHL, KISS, and KMbase), major clinical trial registries, academic journal sites related to the topic, and grey literature databases. Ten studies involving a total of 1,297 participants were identified. Transition interventions proved effective in enhancing disease-related knowledge (Hedge’s g = 0.89, 95% CI = 0.29−1.48) and self-management (Hedge’s g = 0.67, 95% CI = 0.38−0.95), as well as reducing loss to follow-up (OR = 0.41, 95% CI = 0.22−0.77). The certainty of evidence for the estimated values of each major outcome was low or very low.

This study supports the implementation of transition interventions by demonstrating that they can improve patients’ disease knowledge and self-management, while also promoting treatment continuity. However, since the available data on transition interventions for adolescents and young adults with CHD remain limited, the widespread adoption of structured transition interventions in the future may alter the conclusions of this study.

URL: https://www.crd.york.ac.uk/PROSPERO. Unique identifier: CRD42023399026.

Paediatric early warning score systems are used for early detection of clinical deterioration of patients in paediatric wards. Several paediatric early warning scores have been developed, but most of them are not suitable for children with cyanotic CHD who are adapted to lower arterial oxygen saturation.

The present study compared the original paediatric early warning system of the Royal College of Physicians of Ireland with a modification for children with cyanotic CHD.

Retrospective single-centre study in a paediatric cardiology intermediate care unit at a German university hospital.

The distribution of recorded values showed a significant shift towards higher score values in patients with cyanotic CHD (p < 0.001) using the original score, but not with the modification. An analysis of sensitivity and specificity for the factor “requirement of action” showed an area under the receiver operating characteristic for non-cyanotic patients of 0.908 (95% CI 0.862–0.954). For patients with cyanotic CHD, using the original score, the area under the receiver operating characteristic was reduced to 0.731 (95% CI 0.637–0.824, p = 0.001) compared to 0.862 (95% CI 0.809–0.915, p = 0.207), when the modified score was used. Using the critical threshold of scores ≥ 4 in patients with cyanotic CHD, sensitivity and specificity for the modified score was higher than for the original (sensitivity 78.8 versus 72.7%, specificity 78.2 versus 58.4%).

The modified score is a uniform scoring system for identifying clinical deterioration, which can be used in children with and without cyanotic CHD.

CHD refers to structural cardiac abnormalities which comprise the commonest group of congenital malformations. Malta is a small island in the central Mediterranean with excellent diagnostic and therapeutic facilities. It is unique in the European population as termination of pregnancy is illegal. This study was carried out to ascertain patterns in CHD prevalence in comparison with EUROCAT data (European Surveillance of Congenital Anomalies).

Anonymised data were obtained from the EUROCAT website for 1993–2020.

There were a total of 22,833,032 births from all EUROCAT Registries, of which 121,697 were from Malta. The prevalence rate for Malta CHD was 32.38/10,000 births (at the higher end of the range). Malta had a significant excess of commoner, comparatively non-severe CHDs. For most of the severe lesions analysed rates reported were higher than EUROCAT average, however, apart from Ebstein’s anomaly, they all fell within the ranges reported from the different registries.

Wide variations in reported CHD prevalence are known, and the Malta rates may be higher for milder defects due to quicker pickup prior to spontaneous resolution. There may also be a higher pickup of milder forms of more severe conditions. For the more severe conditions, lack of termination may be the explanation. These factors may result in the higher neonatal mortality observed in Malta.

Lactating parents of infants hospitalised for critical congenital heart disease (CHD) face significant barriers to direct breastfeeding. While experiences of directly breastfeeding other hospitalised neonates have been described, studies including infants with critical CHD are scarce. There is no evidence-based standard of direct breastfeeding care for these infants, and substantial practice variation exists.

To explain how direct breastfeeding is established with an infant hospitalised for critical CHD, from lactating parents’ perspectives.

This study is a qualitative grounded dimensional analysis of interviews with 30 lactating parents of infants with critical CHD who directly breastfed within 3 years. Infants received care from 26 United States cardiac centres; 57% had single ventricle physiology. Analysis included open, axial, and selective coding; memoing; member checking; and explanatory matrices.

Findings were represented by a conceptual model, “Wayfinding through the ‘ocean of the great unknown’.” The core process of Wayfinding involved a nonlinear trajectory requiring immense persistence in navigating obstacles, occurring in a context of life-and-death consequences for the infant. Wayfinding was characterised by three subprocesses: navigating the relationship with the healthcare team; protecting the direct breastfeeding relationship; and doing the long, hard work. Primary influencing conditions included relentless concern about weight gain, the infant’s clinical course, and the parent’s previous direct breastfeeding experience

For parents, engaging in the Wayfinding process to establish direct breastfeeding was feasible and meaningful – though challenging. The conceptual model of Wayfinding explains how direct breastfeeding can be established and provides a framework for research and practice.

Feeding difficulty is common in infants with CHD.

The purpose of this study was to describe the feeding skills and behaviours of infants with CHD at 6–12 months of age and explore relationships between feeding, gastrointestinal distress, and gastroesophageal reflux.

Parents of 30 infants with CHD completed online surveys when their infant was 6, 8, 10, and 12 months old. Surveys included parent-report measures of feeding skills (Child Oral and Motor Proficiency Scale), feeding behaviours (Pediatric Eating Assessment Tool), symptoms of gastrointestinal distress (Infant Gastrointestinal Symptoms Questionnaire), and gastroesophageal reflux (Infant Gastroesophageal Reflux Questionnaire-Revised).

We found that 95, 32, 67, and 30% of infants were delayed in their feeding skill development at 6, 8, 10, and 12 months, respectively. Symptoms of problematic feeding behaviours were similarly high, with 90, 62, 29, and 38% of infants meeting criteria for problematic feeding at 6, 8, 10, and 12 months, respectively. Feeding skills and behaviours were related but unique contributors. Feeding behaviours were related to both gastrointestinal distress and gastroesophageal reflux, but feeding skills were rarely related to either.

Delayed acquisition of feeding skills and problematic feeding were common in infants with CHD. Infants with more gastrointestinal and gastroesophageal reflux symptoms had more symptoms of problematic feeding behaviours.

Comprehensive assessment, targeted interventions, and early involvement of feeding specialists are important to support feeding in infants with CHD through at least the first year of life.

Various electrocardiogram (ECG)-based devices are available for home monitoring, but the reliability in adults with CHD is unknown. Therefore, we determined the accuracy of different ECG-based devices compared to the standard 12-lead ECG in adult CHD.

This is a single-centre, prospective, cross-sectional study in 176 consecutive adults with CHD (54% male, age 40 ± 16.6 years, 24% severe CHD, 84% previous surgery, 3% atrial fibrillation (AF), 24% right bundle branch block). Diagnostic accuracy of the Withings Scanwatch (lead I), Eko DUO (precordial lead), and Kardia 6L (six leads) was determined in comparison to the standard 12-lead ECG on several tasks: 1) AF classification (percentage correct), 2) QRS-morphology classification (percentage correct), and 3) ECG intervals calculation (QTc time ≤ 40 ms difference). Both tested AF algorithms had high accuracy (Withings: 100%, Kardia 6L: 97%) in ECGs that were classified. However, the Withings algorithm classified fewer ECGs as inconclusive (5%) compared to 31% of Kardia (p < 0.001). Physician evaluation of Kardia correctly classified QRS morphology more frequently (90% accuracy) compared to Eko DUO (84% accuracy) (p = 0.03). QTc was underestimated on all ECG-based devices (p < 0.01). QTc duration accuracy was acceptable in only 51% of Withings versus 70% Eko and 74% Kardia (p < 0.001 for both comparisons).

Although all devices demonstrated high accuracy in AF detection, the Withings automatic algorithm had fewest uninterpretable results. Kardia 6L was most accurate in overall evaluation such as QRS morphology and QTc duration. These findings can inform both patients and caregivers for optimal choice of home monitoring.

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.

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.

Heterotaxy syndrome, being right atrial isomerism (RAI) or left atrial isomerism (LAI), often presents with Congenital Heart Disease (CHD). Intestinal abnormalities, including malrotation are common. We assessed the spectrum of gut abnormalities and their impact on medium-term outcome in a cohort of patients with fetal and postnatal diagnoses of heterotaxy syndrome.

We reviewed the cardiology records of heterotaxy syndrome patients from two centres, regarding the presence of CHD, time for cardiac intervention, presence of gastrointestinal abnormalities, and type/time of surgery. A questionnaire about gastrointestinal status was sent to patients <18 years old. Kaplan–Meier curves were derived for survival data and freedom from intervention.

Data were included for 182 patients (49 RAI and 133 LAI) of 247 identified. Questionnaires were sent to 77 families and 47 replied. CHD was present in all RAI and 61.7% of LAI cases. Thirty-eight patients had abdominal surgery (20.9%), similar for RAI and LAI (20.4% versus 21%, p> 0.99): Ladd procedure in 17 (44.7%), non-Ladd in 12 (31.5%), and both procedures in 9 (23.7%). Ten-year freedom from Ladd procedure for all was 86% for the whole cohort (RAI = 87%; LAI = 85%, p = 0.98). Freedom from any gastrointestinal surgery at 10 years was 79% for the whole cohort (RAI = 77%; LAI = 80%, p = 0.54). Ten-year freedom from cardiac surgery was 31% for the whole cohort (RAI = 6%; LAI = 43%, p < 0.0001).

In our cohort, one in five patients required abdominal surgery, mostly in their first year of life, similar for RAI and LAI. Between 1 and 10 years of follow-up, the impact of gastrointestinal abnormalities on outcome was minimal. Medium term survival was related to CHD.

To investigate the risk factors associated with prolonged ventilation after Fontan surgery.

Retrospective case series.

Tertiary childrens hospital.

We included 123 children who underwent Fontan surgery without delayed sternal closure or extracorporeal membrane oxygenation between 2011 and 2017.

Fontan surgery.

Prolonged ventilation was defined as intubation for more than 24 hours after surgery. Preoperative, intraoperative, and perioperative data were collected retrospectively from medical records. Multivariate logistic regression analysis was used to identify risk factors for prolonged ventilation. The median age and weight of patients were 2.2 years and 10.0 kg, respectively. Seventeen per cent of the patients (n = 21) received prolonged mechanical ventilation, and the median intubation period was 2.9 days. There were no 90-day or in-hospital deaths. The independent predictors of prolonged ventilation identified were fenestration (p < 0.01), low pulmonary artery index (p = 0.02), and advanced atrioventricular regurgitation (p < 0.01). The duration of ICU stay was significantly longer in the prolonged ventilation group than in the early extubation group (10 days versus 6 days, p < 0.01).

Fenestration, low pulmonary artery index, and significant atrioventricular regurgitation are risk factors for prolonged ventilation after Fontan surgery. Careful preoperative and perioperative management that considers the risk factors for prolonged ventilation in each individual is important.

Cyanotic CHD is one of many disorders in paediatrics that influence the health of children in different clinical aspects. One of the fundamental aspects that may be affected is bone mineral density.

The aim of our study is to assess bone mineral density in children with congenital cyanotic heart disease of different anatomical diagnoses.

Cross-sectional, observational study included 39 patients (20 males) with congenital cyanotic heart disease of different anatomical diagnoses following with the cardiology clinic in Mansoura University children’s hospital. All patients were subjected to anthropometric measures, oxygen saturation assessment, and lumber bone mineral density using dual-energy X-ray absorptiometry.

Six patients (15.4%) out of the 39 included patients showed bone mineral density reduction, 13 patients (33.3%) showed bone mineral density with Z-score between −1 and −2, while 20 patients (51.3%) showed bone mineral density with Z-score more than −1.

Low bone mineral density can be found in children with cyanotic CHD, making it important to consider bone mineral density assessment and early treatment if needed to avoid further complications.

The prevalence of congenital heart disease (CHD) in adults is rising necessitating a greater understanding of acquired diseases such as community-acquired pneumonia, which remains a leading cause of age-related mortality and morbidity in the general population. We hypothesise that the CHD population, given cardiopulmonary mechanics and altered immune function, bears a uniquely high risk for pneumonia-related hospitalisations and mortality.

A countrywide cohort study was performed to calculate the relative risk and cumulative incidence of pneumonia hospitalisations and resultant 30-day mortality amongst the adult CHD population, matched 1:10 with non-CHD persons by gender, age, and adjusted for comorbidities. Cox proportional hazard regression quantified the impact of CHD severity and extracardiac defects.

The CHD cohort includes 17,162 adults. The majority demonstrate mild/moderate CHD complexity. The cumulative incidence of pneumonia hospitalisation was higher for adults with CHD (hazard ratio 1.90; 95% confidence interval: 1.74–2.06) than the comparison cohort. This risk was increased for those with extracardiac defects or a syndrome (hazard ratio: 4.34; 95% confidence interval: 3.39–5.54). Additionally, CHD individuals with severe/univentricular subtypes demonstrate a heightened risk compared to the non-CHD cohort (hazard ratio: 2.35; 95% confidence interval: 1.94–2.84), as well as compared to those with mild/moderate CHD (hazard ratio: 1.28; 95% confidence interval: 1.07–1.53). In addition, pneumonia hospitalisation mortality was elevated above the comparison population with a 30-day mortality rate ratio of 1.31 (95% confidence interval: 1.00–1.73).

Adults with CHD are at elevated risk of pneumonia hospitalisations and pneumonia-associated mortality. This risk is further elevated in those with severe CHD and extracardiac defects.

Cardiopulmonary exercise testing performance has been shown to be a predictor of morbidity, mortality, and quality of life in patients with Fontan physiology; however, the role of exercise performance along with other diagnostics is not fully understood. We evaluated the hypothesis that reduced exercise performance correlates with poorer quality of life in Fontan patients as they continue to age.

Chart review was performed on patients 12 years and older with Fontan who had completed cardiopulmonary exercise testing and age-appropriate quality of life surveys. Quality of life outcomes were analysed against exercise performance and other descriptive data.

For the younger cohort (n = 22), exercise performance predicted quality of life with different measures across domains and had a stronger correlation than echocardiographic parameters. For the older cohort (n = 34), exercise performance did not predict quality of life.

Objective exercise performance was a useful marker for general, physical, emotional, social, and school quality of life in a younger cohort but less helpful in older adults. This is perhaps due to older patients accommodating to their conditions over time. The role of exercise performance and objective data in predicting quality of life in patients with Fontan physiology is incompletely understood and additional prospective evaluation should be undertaken.