Describing our institution’s off-label use of gabapentin to treat irritability after superior cavopulmonary connection surgery and its impact on subsequent opiate and benzodiazepine requirements.

This is a single-center retrospective cohort study including infants who underwent superior cavopulmonary connection operation between 2011 and 2019.

Gabapentin was administered in 74 subjects (74/323, 22.9%) during the observation period, with a median (IQR) starting dose of 5.7 (3.3, 15.0) mg/kg/day and a maximum dose of 10.7 (5.5, 23.4) mg/kg/day. Infants who underwent surgery in 2015–19 were more likely to receive gabapentin compared with those who underwent surgery in 2011–14 (p < 0.0001). Infants prescribed gabapentin were younger at surgery (137 versus 146 days, p = 0.007) and had longer chest tube durations (1.8 versus 0.9 days, p < 0.001), as well as longer postoperative intensive care (5.8 versus 3.1 days, p < 0.0001) and hospital (11.5 versus 7.0 days, p < 0.0001) lengths of stays. The year of surgery was the only predisposing factor associated with gabapentin administration in multivariate analysis. In adjusted linear regression, infants prescribed gabapentin on postoperative day 0–4 (n = 64) had reduced benzodiazepine exposure in the following 3 days (−0.29 mg/kg, 95% CI −0.52 – −0.06, p = 0.01) compared with those not prescribed gabapentin, while no difference was seen in opioid exposure (p = 0.59).

Gabapentin was used with increasing frequency during the study period. There was a modest reduction in benzodiazepine requirements associated with gabapentin administration and no reduction in opioid requirements. A randomised controlled trial could better assess gabapentin’s benefits postoperatively in children with congenital heart disease.

We reviewed outcomes in all 36 consecutive children <5 kg supported with the Berlin Heart pulsatile ventricular assist device at the University of Florida, comparing those with acquired heart disease (n = 8) to those with congenital heart disease (CHD) (n = 28).

The primary outcome was mortality. The Kaplan-Meier method and log-rank tests were used to assess group differences in long-term survival after ventricular assist device insertion. T-tests using estimated survival proportions were used to compare groups at specific time points.

Of 82 patients supported with the Berlin Heart at our institution, 49 (49/82 = 59.76%) weighed <10 kg and 36 (36/82 = 43.90%) weighed <5 kg. Of 36 patients <5 kg, 26 (26/36 = 72.22%) were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 36 patients <5 kg was [days]: median = 109, range = 4–305.) Eight out of 36 patients <5 kg had acquired heart disease, and all eight [8/8 = 100%] were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 8 patients <5 kg with acquired heart disease was [days]: median = 50, range = 9–130.) Twenty-eight of 36 patients <5 kg had congenital heart disease. Eighteen of these 28 [64.3%] were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 28 patients <5 kg with congenital heart disease was [days]: median = 136, range = 4–305.) For all 36 patients who weighed <5 kg: 1-year survival estimate after ventricular assist device insertion = 62.7% (95% confidence interval = 48.5–81.2%) and 5-year survival estimate after ventricular assist device insertion = 58.5% (95% confidence interval = 43.8–78.3%). One-year survival after ventricular assist device insertion = 87.5% (95% confidence interval = 67.3–99.9%) in acquired heart disease and 55.6% (95% confidence interval = 39.5–78.2%) in CHD, P = 0.036. Five-year survival after ventricular assist device insertion = 87.5% (95% confidence interval = 67.3–99.9%) in acquired heart disease and 48.6% (95% confidence interval = 31.6–74.8%) in CHD, P = 0.014.

Pulsatile ventricular assist device facilitates bridge to transplantation in neonates and infants weighing <5 kg; however, survival after ventricular assist device insertion in these small patients is less in those with CHD in comparison to those with acquired heart disease.

Cardiac arrhythmias are a major concern in patients with CHD. The purpose of this study was to evaluate the long-term outcomes in patients with CHD submitted to catheter ablation.

Observational retrospective study of patients with CHD referred for catheter ablation from January 2016 to December 2021 in a tertiary referral centre. Acute procedural endpoints and long-term outcomes were assessed.

A total of 44 ablation procedures were performed in 36 CHD patients (55% male, mean age 43 ±3 years). Fifty-four arrhythmias were ablated: 23 cavotricuspid isthmus atrial flutters, 10 atrial re-entrant tachycardias, eight focal atrial tachycardias, eight atrial fibrillations, three atrioventricular re-entrant tachycardias, and two ventricular tachycardias. During a median follow-up time of 37 months (interquartile range 12–51), freedom from arrhythmia recurrence was achieved in 93%, with 1.2 procedures per patient (18% with anti-arrhythmic drugs). There were no adverse events related to catheter ablation. No predictors of recurrence were identified.

In patients with CHD, catheter ablation presents a high mid-term efficacy while maintaining a safe profile.

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.