Publicly available health information is increasingly important for patients and their families. While the average US citizen reads at an 8th-grade level, electronic educational materials for patients and families are often advanced. We assessed the quality and readability of publicly available resources regarding hypoplastic left heart syndrome (HLHS).

We queried four search engines for “hypoplastic left heart syndrome”, “HLHS”, and “hypoplastic left ventricle”. The top 30 websites from searches on Google, Yahoo!, Bing, and Dogpile were combined into a single list. Duplicates, commercial websites, physician-oriented resources, disability websites, and broken links were removed. Websites were graded for accountability, content, interactivity, and structure using a two-reviewer system. Nonparametric analysis of variance was performed.

Fifty-two websites were analysed. Inter-rater agreement was high (Kappa = 0.874). Website types included 35 hospital/healthcare organisation (67.3%), 12 open access (23.1%), 4 governmental agency (7.7%), and 1 professional medical society (1.9%). Median total score was 19 of 39 (interquartile range = 15.8–25.3): accountability 5.5 of 17 (interquartile range = 2.0–9.3), content 8 of 12 (interquartile range = 6.4–10.0), interactivity 2 of 6 (interquartile range = 2.0–3.0), and structure 3 of 4 (interquartile range = 2.8–4.0). Accountability was low with 32.7% (n = 17) of sites disclosing authorship and 26.9% (n = 14) citing sources. Forty-two percent (n = 22) of websites were available in Spanish. Total score varied by website type (p = 0.03), with open access sites scoring highest (median = 26.5; interquartile range = 20.5–28.6) and hospital/healthcare organisation websites scoring lowest (median = 17.5; interquartile range = 13.5–21.5). Score differences were driven by differences in accountability (p = 0.001) – content scores were similar between groups (p = 0.25). Overall readability was low, with median Flesch–Kincaid Grade Level of 11th grade (interquartile range = 10th–12th grade).

Our evaluation of popular websites about HLHS identifies multiple opportunities for improvement, including increasing accountability by disclosing authorship and citing sources, enhancing readability by providing material that is understandable to readers with the full spectrum of educational background, and providing information in languages besides English, all of which would enhance health equity.

CHDs correspond to 28% of all congenital anomalies, being the leading cause of infant mortality in the first year of life. Thus, it is essential to explore risk factors for CHDs presentation, allowing the detection of probable cases within a population.

We identified newborns with CHDs within a cohort from the Program for the Prevention and Monitoring of Congenital Defects in Bogota and Cali, 2002–2020. Cases were classified as isolated, complex isolated, polymalformed, and syndromic. Variables were analysed by comparing case and control averages with Student’s t test using a 95% confidence level.

Prevalence obtained was 19.36 per 10 000 live births; non-specified CHD, ventricular septal defect, and atrial septal defect were the most prevalent. As risk factors were found: paternal and maternal age above 45 years, pregestational diabetes, mother’s body mass index above 25, low educational level, and socio-economic status. As protective factors: folic acid consumption within the first trimester and pregestational period.

Different risk and protective factors associated with the presentation of CHDs have been described. We consider that public health strategies should be aimed to reduce risk factors exposure. Also, improving diagnosis and prognosis by having a close monitoring on high-risk patients.

To define the incidence of definitive necrotising enterocolitis in term infants with CHD and identify risk factors for morbidity/mortality.

We performed a 20-year (2000–2020) single-institution retrospective cohort study of term infants with CHD admitted to the Boston Children’s Hospital cardiac ICU with necrotising enterocolitis (Bell’s stage ≥ II). The primary outcome was a composite of in-hospital mortality and post-necrotising enterocolitis morbidity (need for extracorporeal membrane oxygenation, multisystem organ failure based on the paediatric sequential organ failure assessment score, and/or need for acute gastrointestinal intervention). Predictors included patient characteristics, cardiac diagnosis/interventions, feeding regimen, and severity measures.

Of 3933 term infants with CHD, 2.1% (n = 82) developed necrotising enterocolitis, with 67% diagnosed post-cardiac intervention. Thirty (37%) met criteria for the primary outcome. In-hospital mortality occurred in 14 infants (17%), of which nine (11%) deaths were attributable to necrotising enterocolitis. Independent predictors of the primary outcome included moderate to severe systolic ventricular dysfunction (odds ratio 13.4,confidence intervals 1.13–159) and central line infections pre-necrotising enterocolitis diagnosis (odds ratio 17.7, confidence intervals 3.21–97.0) and mechanical ventilation post-necrotising enterocolitis diagnosis (odds ratio 13.5, confidence intervals 3.34–54.4). Single ventricle, ductal dependency, and feeding related factors were not independently associated with the primary outcome.

The incidence of necrotising enterocolitis was 2.1% in term infants with CHD. Adverse outcomes occurred in greater than 30% of patients. Presence of systolic dysfunction and central line infections prior to diagnosis and need for mechanical ventilation after diagnosis of necrotising enterocolitis can inform risk triage and prognostic counseling for families.

Parents of children with CHD face several barriers when trying to access the services needed to support their child’s development. In fact, current developmental follow-up practices may not identify developmental challenges in a timely manner and important opportunities for interventions may be lost. This study aimed to explore the perspectives of parents of children and adolescents with CHD with respect to developmental follow-up in Canada.

Interpretive description was used as a methodological approach for this qualitative study. Parents of children aged 5–15 years with complex CHD were eligible. Semi-structured interviews that aimed to explore their perspectives regarding their child’s developmental follow-up were conducted.

Fifteen parents of children with CHD were recruited for this study. They expressed that the lack of systematic and responsive developmental follow-up services and limited access to resources to support their child’s development placed an undue burden on their families, and as a result, they needed to assume new roles as case managers or advocates to address these limitations. This additional burden resulted in a high level of parental stress, which, in turn, affected the parent–child relationship and siblings.

The limitations of the current Canadian developmental follow-up practices put undue pressure on the parents of children with complex CHD. The parents stressed the importance of implementing a universal and systematic approach to developmental follow-up to allow for the timely identification of challenges, enabling the initiation of interventions and supports and promoting more positive parent–child relationships.

Idiopathic dilatation of the right atrium is a rare congenital anomaly that presents as an isolated enlargement of the right atrium. Thrombus formation and atrial arrhythmias are the major complications and management with antiplatelet therapy is recommended. Reduction atrial plasty is reserved for specific patients. We report a case of idiopathic dilatation of the right atrium diagnosed prenatally with a 10-year follow-up.

The Residual Lesion Score is a novel tool for assessing the achievement of surgical objectives in congenital heart surgery based on widely available clinical and echocardiographic characteristics. This article describes the methodology used to develop the Residual Lesion Score from the previously developed Technical Performance Score for five common congenital cardiac procedures using the RAND Delphi methodology.

A panel of 11 experts from the field of paediatric and congenital cardiology and cardiac surgery, 2 co-chairs, and a consultant were assembled to review and comment on validity and feasibility of measuring the sub-components of intraoperative and discharge Residual Lesion Score for five congenital cardiac procedures. In the first email round, the panel reviewed and commented on the Residual Lesion Score and provided validity and feasibility scores for sub-components of each of the five procedures. In the second in-person round, email comments and scores were reviewed and the Residual Lesion Score revised. The modified Residual Lesion Score was scored independently by each panellist for validity and feasibility and used to develop the “final” Residual Lesion Score.

The Residual Lesion Score sub-components with a median validity score of ≥7 and median feasibility score of ≥4 that were scored without disagreement and with low absolute deviation from the median were included in the “final” Residual Lesion Score.

Using the RAND Delphi methodology, we were able to develop Residual Lesion Score modules for five important congenital cardiac procedures for the Pediatric Heart Network’s Residual Lesion Score study.

The present study focuses on assessing the physical activity level of children with Fontan circulation for Hypoplastic Left Heart Syndrome and identifying potential barriers and facilitators toward their participation in physical activity.

Seven children aged 5–16 years (mean (SD) 8.8 (3.7) years) with a Fontan procedure for hypoplastic left heart syndrome, their parents (n = 7), and siblings (n = 1) were recruited. Data were collected using a mixed-methods approach: (i) children wore an activity monitor for 7 days to record physical activity, with sedentary time and level of activity calculated from accelerometer data; (ii) children completed a bespoke questionnaire recording limitations in physical activity; (iii) parents completed a semi-structured interview discussing perceptions about their child’s physical activity participation. Qualitative data were analysed using thematic analysis.

Activity monitors data recorded highly active children with a mean (SD) of 153(36) minutes/day spent in moderate-to-vigorous physical activity. Time spent in sedentary behaviour was also high (57.5% of total accelerometer wearing-time). Four key themes relating to parental perceptions of physical activity were identified: (i) A new lease of life –post-Fontan; (ii) Setting limits – managing limitations; (iii) The wider world – how others set limits; and (iv) “I fear the future” – parental concerns.

Following completion of the Fontan circulation, children engaged in higher levels of physical activity in comparison to the national average. However, more than half their time was spent in sedentary behaviour. Fears and anxiety from parents and teachers may act as a barrier toward physical activity participation.

To describe the epidemiology of severe bleeding in the immediate post-operative period in children who undergo cardiopulmonary bypass surgery using the Bleeding Assessment Scale for critically Ill Children (BASIC).

Retrospective cohort study in a paediatric ICU from 2015 to 2020.

356 children were enrolled; 59% were male with median (IQR) age 2.1 (0.5–8) years. Fifty-seven patients (16%) had severe bleeding in the first 24 hours post-operatively. Severe bleeding was observed more frequently in younger and smaller children with longer bypass and cross-clamp times (p-values <0.001), in addition to higher surgical complexity (p = 0.048). Those with severe bleeding received significantly more red blood cells, platelets, plasma, and cryoprecipitate in the paediatric ICU following surgery (all p-values <0.001). No laboratory values obtained on paediatric ICU admission were able to predict severe post-operative bleeding. Those with severe bleeding had significantly less paediatric ICU-free days (p = 0.010) and mechanical ventilation-free days (p = 0.013) as compared to those without severe bleeding.

Applying the BASIC definition to our cohort, severe bleeding occurred in 16% of children in the first day following cardiopulmonary bypass. Severe bleeding was associated with worse clinical outcomes. Standard laboratory assays do not predict bleeding warranting further study of available laboratory tests.

Right ventricular outflow tract intervention spans transcatheter, surgical, or hybrid pulmonary valve replacement methodologies. Standardised pre-procedure workup includes cardiac MRI to identify an intended valve site (landing zone). Our institutional practice includes measurement of the right ventricular outflow tract perimeter (circumference) of this site in end-systole. Our primary aim was to compare patients by their perimeter values to the palliative interventions performed (transcatheter versus surgical/hybrid methodologies).

Retrospective review of patients undergoing pulmonary valve replacement from January 2017 to 2021. We performed perimeter measurements at the intended valve site on advanced imaging; the outcomes of interventions were outlined via descriptive and statistical analyses.

A total of 37 patients underwent pulmonary valve replacement that met study criteria – 21 transcatheter, 7 surgical, and 9 hybrid. Median age at intervention was 26 years (range 8–70). The mean end-systolic perimeter of the transcatheter cohort was 88.9 ± 8.7 mm and in the surgical/hybrid cohort measured 106.6 ± 7.5 mm. For the transcatheter cohort, the median “circularised” diameter derived from the perimeter measurement (divided by π) was 27.7 mm (range 24.3–32.4). Notably, this correlated (r = 0.93, p < 0.01) with the median diameter of the narrowest region during actual transcatheter right ventricular outflow tract balloon sizing (lateral imaging) of 27.1 mm (range 23.2–30.1).

Right ventricular outflow tract perimeter measurement to determine circularised diameter is useful in planning pulmonary valve replacement in terms of candidacy of transcatheter versus the need for a surgical/hybrid approach. The circularised diameter correlates with transcatheter right ventricular outflow tract balloon sizing.