Diastolic heart failure may be noted in paediatric patients with CHD, cardiomyopathy, or malignancies requiring chemotherapy, but the available data are scarce, and often derived from adult trials or based on theoretic or anecdotal evidence.

Data between 2016 and 2021 were obtained from Pediatric Health Information System database. Patients <18 years of age with isolated diastolic heart failure admitted to ICU at some point during admission were included. They were divided into patients with and without inpatient mortality. Patients’ demographics, comorbidities using ICD-10 codes, and pharmacologic interventions were also recorded. Univariate analysis was done in demographics, comorbidities, pharmacologic interventions, and mechanical interventions between admissions with and without mortality. Multivariable logistic regression was done for inpatient mortality and multivariable linear regression was done for total hospital length of stay in survivors.

Isolated diastolic heart failure comprised 0.5% of critically ill paediatric patients. A total of 121 (5%) experienced mortality among the 2,273 admissions in the final analyses. Milrinone and angiotensin converting enzyme inhibitor were found to be associated with decreased mortality. Increasing age and diuretics were associated with decreased total hospital length of stay in survivors.

In the cohort studied, isolated diastolic left heart failure has a 5% mortality. Several comorbidities and interventions are associated with increased mortality with milrinone and angiotensin converting enzyme inhibitors being associated with decreased risk of mortality. When only admissions with survival to discharge are considered, older age and diuretics are associated with lower total hospital length of stay.

Lymphatic abnormalities have been documented on magnetic resonance lymphangiography in patients with functionally univentricular circulation.

The aim of this study is to evaluate associations between findings on magnetic resonance lymphangiography, cardiac catheterisation and functional phenotype in patients with Fontan-Kreutzer circulation.

Between January 2017 and October 2019, seven patients with Fontan-Kreutzer circulation (16.57 ± 7.10 years) were enrolled following ethical committee approval and attainment of informed consent. Clinical data, consecutive magnetic resonance imaging and cardiac catheterisation findings were reviewed. Qualitative lymphatic abnormality types were defined as: 1 – Little or none; 2 – Localised to the supraclavicular region; 3 – Extending into the mediastinum; and 4 – Extending into the lung. Pulmonary hypertension was defined as a transpulmonary gradient > 6 mmHg. Quantitative data were assessed using Mann–Whitney U tests, and p < 0.05 was considered statistically significant.

Patients with lymphatic abnormalities categorised type 1 or 2 and transpulmonary gradient ≤ 6 mmHg had a normal functional phenotype, while those with type 3 or 4 and transpulmonary gradient > 6 mmHg had a failing functional phenotype.

Magnetic resonance lymphangiography in patients with Fontan-Kreutzer circulation and pulmonary hypertension revealed lymphatic abnormalities types 3–4, indicative of a failing functional phenotype compared to patients without pulmonary hypertension. If validated in larger studies, these results suggest the potential utility of this extracardiac biomarker for improving risk stratification. Integration of lymphangiography and transpulmonary gradient with functional class, atrioventricular valve function, and ejection fraction allowed for the categorisation of three distinct functional phenotypes, which may be valuable for future analyses.

Kawasaki disease is an acute febrile disease causing systemic vasculitis that is common in infants and young children. This study was conducted to explore the relationships of the rs1412125, and rs2249825 single nucleotide polymorphisms of the high mobility group box 1 gene to Kawasaki disease and its complication of coronary artery injury.

In total, 200 children with Kawasaki disease (49 with coronary artery injury) and 200 healthy controls were enrolled in this study. Polymerase chain reaction was used to amplify the target gene, and direct sequencing was performed to determine distributions at the rs1412125 T/C and rs2249825 C/G loci in the HMGB1 gene. The chi-squared test was used to compare data between groups. Linkage disequilibrium coefficients and single nucleotide polymorphism haplotype analysis were conducted, and a false-positive report probability analysis was used to assess significant associations. Expression quantitative trait loci analysis was performed to determine if single nucleotide polymorphisms affected mRNA levels via the GTEx portal.

Significant differences in the genotype TT, TC, and CC distributions (χ2 = 7.918, P = 0.019) and allele T and C frequencies (χ2 = 6.125, P = 0.013) of rs1412125 T/C locus were found between the Kawasaki disease and healthy control groups. The genotype CC was associated with a greater Kawasaki disease risk [odds ratio = 3.205, 95% confidence interval = 1.352–7.595, χ2 = 7.560, P = 0.006]. C allele carriers had a higher Kawasaki disease risk than did T allele carriers (odds ratio = 1.469, 95% confidence interval = 1.083–1.993, χ2 = 6.125, P = 0.013). The rs1412125 genotype T/C distribution (χ2 = 10.906, P = 0.004) and allele frequencies (χ2 = 8.813, P = 0.003) differed significantly between patients with and without coronary artery injury. In the dominant model, the coronary artery injury risk was 3.006 times greater for patients with the TT genotype than for those with the other genotypes (odds ratio = 3.006, 95% confidence interval = 1.540–5.867, χ2 = 10.875, P = 0.001). No significant difference in the rs2249825 genotype C/G distribution or allele frequencies was found between the Kawasaki disease and control groups, or between the coronary artery injury and without coronary artery injury groups.

The rs1412125 polymorphism of the HMGB1 gene is associated with Kawasaki disease and its coronary artery injury complication. The CC genotype may be a risk factor for Kawasaki disease onset, and the TT genotype may be a risk factor for coronary artery injury in Kawasaki disease.

Lack of psychological safety within surgical teams and fear of damaging one’s career or reputation may counteract the effect of formal reporting as a tool for preventing bullying. Also, personality traits of cardiothoracic surgeons may promote bullying. In the current study, we evaluate these factors and their association with bullying in cardiothoracic surgical teams.

Forty-four cardiothoracic surgeons filled in the Negative Acts Questionnaire-Revised, Safety Attitude Questionnaire, and Big Five Inventory.

Of the 44 participants, 18 (40.90%) experienced bullying during their careers. Psychological safety was negatively associated with work-related bullying and positively associated with the agreeableness and openness personality factors.

Bullying within the cardiothoracic surgical teams is a prominent problem that is negatively associated with psychological safety. Psychological safety is positively associated with agreeableness and openness, and interventions focusing on promoting an organizational culture based on such factors may improve the effectiveness of such interventions.

Interstage monitoring programs for single ventricle disease have been developed to reduce morbidity and mortality. There is increased use of telemedicine and mobile application monitoring. It is unknown if there are disparities in use based on patient socio-demographic factors.

We conducted a retrospective cohort study of patients enrolled in the single ventricle monitoring program and KidsHeart application at a single centre from 4/21/2021 to 12/31/2023. We investigated the association of socio-demographic factors with telemedicine usage, mobile application enrollment and usage. We assessed resource utilisation and weight changes by program era.

There were 94 children in the cohort. Patients with Norwood and ductal stent had higher mean telemedicine visits per month (1.8 visits, p = 0.004), without differences based on socio-demographic factors. There were differences in application enrollment with more Black patients enrolled compared to White patients (p = 0.016). There were less Hispanic patients enrolled than Non-Hispanic patients (p = 0.034). There were no Spaish speaking patient’s enrolled (p = 0.0015). There were no patients with maternal education of less than high school enrolled and all those with maternal education of advanced degree were enrolled (p = 0.0016). There was decreased mobile application use in those from neighbourhoods in the lowest income quartile. There were decreased emergency department visits with mobile application monitoring. Mean weight-for-age z-scores had increased from start to completion of the program in all eras.

Differences were seen in mobile application enrollment and usage based on socio-demographic factors. Further work is needed to ensure that all patients have access to mobile application usage.

Cardiac intensive care providers require a comprehensive understanding of cardiac output and oxygen delivery. The estimation of cardiac output in clinical practice often relies on thermodilution and the Fick principle. Central venous saturation and lactate levels are commonly used indicators for cardiac output assessment. However, the relationship between venous lactate levels and venous oxygen saturation in paediatric cardiac intensive care patients remains unclear.

This is a single-centre retrospective pilot study aimed to investigate the correlation between venous lactate and venous oxygen saturation in paediatric patients. Data collected included venous saturation, heart rate, mean arterial blood pressure, arterial saturation by pulse oximetry, cerebral and renal near-infra-red spectroscopy values, and the presence of a functionally univentricular heart. Statistical analyses included Bayesian Pearson correlation and regression analyses.

A total of 203 data points from 37 unique patients were included in the analysis. There was no significant correlation between serum lactate and venous saturation (correlation coefficient = –0.01; Bayes factor 10 = 0.06). Serum lactate also did not correlate with other haemodynamic metrics. Venous saturation showed correlations with arterial saturation and cerebral and renal near-infra-red spectroscopy. Regression analysis revealed that parallel circulation, arterial saturation, and cerebral near-infra-red spectroscopy were predictive of venous saturation. The following equation resulted from the regression analysis: 68.0 – (12.7 x parallel circulation) – (0.8 x arterial saturation) + (0.3 x cerebral near-infra-red spectroscopy). This model had a Bayes factor 10 of 0.03 and adjusted R-squared was 0.29.

In paediatric cardiac intensive care patients, there is no significant correlation between venous lactate and venous saturation, suggesting that lactate may not be a reliable marker for assessing the adequacy of oxygen delivery in this population. Only a weak correlation could be identified once the venous saturation was 70% or lower. Additional research is needed to explore alternative markers for monitoring oxygen delivery in critically ill paediatric patients.

CHD predisposes children to neurodevelopmental delays. Frequent, prolonged hospitalisations during infancy prevent children with heart disease from participating in recommended language and cognitive development programmes, such as outpatient early childhood literacy programmes, and contribute to caregiver stress, a risk factor for adverse developmental outcomes. This study aims to describe the implementation of a single-centre inpatient early childhood literacy programme for hospitalised infants with heart disease and assess its impact on reading practices and patient–family hospital experience.

Admitted infants ≤1 year old receive books, a calendar to track reading frequency, and reading guidance at regular intervals. Voluntary feedback is solicited from caregivers using an anonymous, QR-code survey on books. A prospective survey also assessed programme impact on hospital experience.

From February 2021 to November 2023, the Books@Heart programme provided 1,293 books to families of 840 infants, of whom 110 voluntarily submitted feedback. Caregivers reported a significant improvement in access to books (p < 0.001) and increased reading frequency after learning about Books@Heart (p = 0.003), with the proportion reading to their child daily increasing from 27% to 62%. Among 40 prospective survey responses, caregivers reported feeling a sense of personal fulfillment (60%), self-confidence (30%), connection (98%), and personal well-being (40%) while reading to their child.

An inpatient early childhood literacy programme is a well-received intervention for infants with heart disease that promotes development, improves book access, increases reading exposure, and engages families. Further studies are needed to assess its impact on sustained reading practices and neurodevelopmental outcomes.

Preoperative pneumonia in children with CHD may lead to longer stays in the ICU after surgery. However, research on the associated risk factors is limited. This study aims to evaluate the pre-, intra-, and postoperative risk factors contributing to extended ICU stays in these children.

This retrospective cohort study collected data from 496 children with CHD complicated by preoperative pneumonia who underwent cardiac surgery following medical treatment at a single centre from 2017 to 2022. We compared the clinical outcomes of patients with varying ICU stays and utilised multivariate logistic regression analysis and multiple linear regression analyses to evaluate the risk factors for prolonged ICU stays.

The median ICU stay for the 496 children was 7 days. Bacterial infection, severe pneumonia, and Risk Adjustment for Congenital Heart Surgery-1 were independent risk factors for prolonged ICU stays following cardiac surgery (P < 0.05).

CHD complicated by pneumonia presents a significant treatment challenge. Better identification of the risk factors associated with long-term postoperative ICU stays in these children, along with timely diagnosis and treatment of respiratory infections in high-risk populations, can effectively reduce ICU stays and improve resource utilisation.

Despite advances in treatment and outcomes for paediatric heart failure, both physical and psychosocial comorbidities remain notable among this patient population. We aimed to qualitatively describe the psychosocial experiences of adolescent and young adults with heart failure and their caregivers’ perceptions, with specific focus on personal challenges, worries, coping skills, and resilience.

Structured, in-depth interviews were performed with 16 adolescent and young adults with heart failure and 14 of their caregivers. Interviews were recorded and transcribed. Content analysis was performed, and themes were generated. Transcripts were coded by independent reviewers.

Ten (63%) adolescent and young adults with heart failure identified as male and six (37.5%) patients self-identified with a racial or ethnic minority group. Adolescent and young adults with heart failure generally perceived their overall illness experience more positively and less burdensome than their caregivers. Some adolescent and young adults noted specific worries related to surgeries, admissions, major complications, death, and prognostic/treatment uncertainty, while caregivers perceived their adolescent and young adult’s greatest worries to be around major complications and death. Adolescent and young adults and their caregivers were able to define and reflect on adolescent and young adult experiences of resilience, with many adolescent and young adults expressing a sense of optimism and gratitude as it relates to their medical journey.

This study is the first of its kind to qualitatively describe the psychosocial experiences of a racially and socioeconomically diverse sample of adolescent and young adults with heart failure, as well as their caregivers’ perceptions of patient experiences. Findings underscore the importance of identifying distress and fostering resilient processes and outcomes in young people with advanced heart disease.

Guidelines recommend screening for psychiatric co-morbidities in patients with congenital heart defects alongside cardiac outpatient follow-ups. These recommendations are not implemented in Denmark. This study aimed to investigate the psychiatric co-morbidities in children and adolescents with Fontan circulation in Denmark and to evaluate the feasibility of an online screening measure for psychiatric disorders.

Children, adolescents, and their families answered the Development and Well-Being Assessment questionnaire and a questionnaire about received help online. Development and Well-Being Assessment ratings present psychiatric diagnoses in accordance with ICD-10 and DSM-5. Parent-reported received psychiatric help is also presented. Feasibility data are reported as participation rate (completed Development and Well-Being Assessments) and parental/adolescent acceptability from the feasibility questionnaire.

The participation rate was 27%. Of the participating children and adolescents, 53% (ICD-10)/59% (DSM-5) met full diagnostic criteria for at least one psychiatric diagnosis. Of these, 50% had not received any psychiatric or psychological help. Only 12% of participants had an a priori psychiatric diagnosis.

We found that a large proportion of children and adolescents with Fontan circulation are underdiagnosed and undertreated for psychiatric disorders. The results from our study emphasise the need for psychiatric screening in this patient group. Development and Well-Being Assessment may be too comprehensive for online electronic screening in children and adolescents with CHD.

To determine the short- and medium-term cardiac outcomes in children admitted with multisystem inflammatory syndrome in children at a tertiary care centre in Pakistan.

Children fulfilling the criteria for multisystem inflammatory syndrome and admitted to the hospital between April 2020 and March 2022 were enrolled in this prospective longitudinal cohort study. From admission to discharge, laboratory and cardiac parameters were recorded for all patients, who were subsequently followed up in clinics at various intervals. Data analysis was conducted using STATA version 15.0.

A total of 51 children were included, with viral myocarditis (41.2%) and toxic shock syndrome (33.3%) being the most common phenotypes. The cardiovascular system was most commonly affected in 27 children (53%) with laboratory evidence of inflammation and myocardial injury with median and interquartile levels of ferritin 1169 (534-1704), C-reactive protein 83 (24-175), lactate dehydrogenase 468 (365-1270), N-terminal pro-B-type natriuretic peptide 8,656 (2,538-31,166), and troponin 0.16 (0.02-2.0).

On admission, decreased left ventricular ejection fraction was observed in 58.8% of patients and impaired global longitudinal strain in 33.3%. At discharge, left ventricular ejection fraction had normalised in 83% of patients. Pericardial effusion resolved in all patients, and valvulitis resolved in 86% by 12 months. Paediatric ICU admission was required in 42 (82%) of patients with an overall mortality of 12% (n = 6).

Our study finds high hospital mortality for multisystem inflammatory syndrome in children compared to 1-2% from previous studies. Yet, in Pakistan, surviving children with multisystem inflammatory syndrome show favourable short- to medium-term cardiac outcomes

Rastelli surgery is used for the correction of several CHDs. Although late-onset cardiac arrhythmias have emerged as a major complication after corrective surgeries, there is a paucity of data on arrhythmias after Rastelli surgery.

This retrospective cohort study was conducted on patients who had undergone Rastelli surgery and have been followed at the adult CHD clinic at our hospital.

A total of 55 patients (36.4% female, age 22.2 ± 6.4 years) were followed for a median period of 24.2 (20.6–31.0) years. Tachyarrhythmias occurred in 21 (38.4 %) patients (n = 15 for atrial tachycardia, 5 for ventricular tachycardia, and 1 for both atrial and ventricular tachycardia). Older age at surgery was significantly associated with the risk of tachyarrhythmias (P = 0.022). Bradyarrhythmia occurred in 12 (21.8%) patients and consisted of perioperative AV block (n = 5), late AV block (n = 1), and sinus node dysfunction (n = 6). Nine (16.4%) patients underwent catheter ablation. The mechanisms of atrial arrhythmias include cavotricuspid isthmus-dependent and surgical scar-dependent intra-atrial reentrant tachycardias. Among the three patients who underwent ablation for ventricular tachycardia, all circuits were dependent on the scar at the base of the right ventricle to pulmonary artery conduit. Median survival free from any event (arrhythmia, death, or heart failure) was 31.6 (28.1–35.1) years after Rastelli surgery.

The prevalence of arrhythmias late after Rastelli surgery is substantial and increases in the second decade after surgery. Older age at surgery is associated with a higher prevalence of arrhythmias.

Complexity stratification for CHD is an integral part of clinical research due to its heterogenous clinical presentation and outcomes. To support our ongoing research efforts into CHD requiring disease severity stratifications, a simplified CHD severity classification system was developed and verified, with potential utility for clinical researchers without specialist CHD knowledge or access to clinical/medical records.

A two-tiered analysis approach was undertaken. First-tier analysis included the audit of a comprehensive system based on: i) timing of intervention, ii) cardiac morphology, and iii) cardiovascular physiology using real patient data (n = 30), across 10 common CHD lesions. Second-tier analysis allowed for a simplified version of the classification system using morphology as a stand-alone predictor. Twelve clinicians of varying specialities involved in CHD care ranked 10 common lesions from least to most severe based on typical presentation and clinical course.

First-tier analysis identified that cardiac morphology was the principal driver of complexity. Second-tier analysis largely confirmed the ranking and classification of the lesions into the broad CHD severity groups, although some variation was noted, specifically among non-cardiac specialists. This simplified version of the classicisation system, with morphology as a stand-alone predictor of severity, allowed for effective stratification for the purposes of analysis.

The findings presented here support this comprehensive and simple CHD severity classification system with broad utility in CHD research, particularly among clinicians and researchers with limited knowledge of CHD. The model may be applied to produce locally relevant research tools.

Our objective was to assess the predictive value of physiologic dead space fraction for mortality in patients undergoing the comprehensive stage 2 operation.

This was a single-centre retrospective observational study conducted at a quaternary free-standing children’s hospital specialising in hybrid palliation of single ventricle cardiac disease. 180 patients underwent the comprehensive stage 2 operation. 76 patients (42%) underwent early extubation, 59 (33%) standard extubation, and 45 (25%) delayed extubation. We measured time to extubation, post-operative outcomes, length of stay and utilised Fine gray models, Youden’s J statistic, cumulative incidence function, and logistic regression to analyse outcomes.

Delayed extubation group suffered significantly higher rates of mortality (31.1% vs. 6.8%), cardiac arrest (40.0% vs. 10.2%), stroke (37.8% vs. 11.9%), and need for catheter (28.9% vs. 5.1%) and surgical intervention (24.4% vs. 8.5%) (P < 0.001). Physiologic dead space fraction was significantly higher in the delayed extubation group and in non-survivors with a value of 0.3, which was found to be the discriminatory point by Youden’s J statistic. For a 0.1 unit increase in physiologic dead space fraction on post-operative day 1, the odds of a patient expiring increase by a factor of 2.26 (95% CI 1.41–3.97, p < 0.001) and by a factor of 3.79 (95% CI 1.65–11.7, p 0.01) on post-operative day 3.

Delayed extubation impacts morbidity and mortality in patients undergoing the comprehensive stage 2 operation. Increased physiologic dead space fraction in the first 60 hours after arrival to the ICU is associated with higher mortality.