Venovenous collaterals are abnormal connections between the systemic and pulmonary venous systems. They are commonly seen in the Fontan circulation and may lead to significant hypoxaemia. Transcatheter closure of venovenous collaterals is a potential but controversial treatment as the long-term benefits and outcomes are not well understood.

This retrospective cohort study utilised data from the Australian and New Zealand Fontan Registry. Patients who underwent transcatheter venovenous collateral occlusion for hypoxemia from the year 2000 onwards were included. Atriopulmonary and Kawashima-type Fontan circulations were excluded to reflect a more contemporary Fontan cohort.

Nineteen patients (age 19.3 ± 7.8 years, 53% female) underwent transcatheter venovenous collateral occlusion. Compared to baseline, mean oxygen saturation was improved at latest follow-up (90.5% vs 87.0%; p = 0.003). Nine patients achieved a clinically significant response (defined as an increase of at least 5% to 90% or greater), and this was associated with lower baseline Fontan pressures (12.9 v 15.6 mmHg; p = 0.02). No heart failure hospitalisations, arrhythmia, transplant referrals, or mortality were observed during the median follow-up period of 4 years. Two patients experienced thromboembolic events and five patients underwent re-intervention.

Transcatheter occlusion of venovenous collaterals in Fontan patients with chronic hypoxaemia resulted in a modest increase in oxygenation over a median follow-up of 4 years and longer-term prognosis did not appear to be adversely affected. Lower Fontan pressures at baseline were associated with a greater improvement in oxygenation.

Developmental care for newborns with congenital heart disease (CHD) improves cardiac and respiratory patterns. According to the American Heart Association, developmental care in newborns with CHD is important for improving neurodevelopmental outcomes. This study aimed to evaluate the validity and reliability of the Turkish version of the Developmental Care Scale for Neonates with Congenital Heart Disease.

This was a methodological, descriptive study conducted with 169 nurses from a tertiary-level NICU. The Demographical Information Form and the Developmental Care Scale for Neonates with Congenital Heart Disease were used to collect the data. The scales’ language and content validity, construct validity, and internal consistency were also assessed.

The scale consists of 31 items and four subscales. Factor loadings ranged from 0.44 to 0.82 and explained 65% of the total variance. Fit indices indicate that the model is acceptable. Cronbach’s α was 0.95 for the entire instrument, 0.91 for developing the external environment subscale, 0.94 for assessing family well-being, 0.86 for the caregiver activities toward the neonate, and 0.82 for the basic need subscale. Item–total correlations ranged between 0.34 and 0.75, according to the item analysis results.

The Turkish version of the Developmental Care Scale for Neonates with Congenital Heart Disease is valid and reliable. The use of this scale could improve the performance of neonatal intensive care nurses in providing developmental care to newborns with CHD as well as the quality of care.

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 aim was to investigate all children admitted to paediatric intensive care units (ICU) in the Republic of Ireland between January 2020 and August 2022 with an admitting diagnosis of acute COVID-19 infection or paediatric inflammatory multi-system syndrome, temporally associated with SARS-CoV-2 (PIMS-TS) or associated illness. The patients were identified to catalogue the severity of illness, analyse cardiovascular manifestations of their disease, and short-term outcomes.

This is a retrospective multi-centre observational study.

127 children were admitted to paediatric ICU in Ireland with a COVID-19- related illness between January 2020 and August 2022. 87 (68.5%) of patients had acute COVID-19 infection, 39 (30.7%) had PIMS-TS and 1 (0.8%) patient had post-COVID vaccine-related myocarditis. Ventilatory support was required for 47/87 (54%) in the COVID-19 group comparative to 9/39 (23%) of patients with PIMS-TS. Inotropic support was required for 13/87 (14.9%) children with COVID-19 and 29/39 (74.3%) with PIMS-TS. Evidence of any cardiac disease on ECHO was identified in 23/38 (60.5%) of the PIMS-TS cohort comparative to only 5/36 (13.9%) of patients with COVID-19. 38/39 (97.4%) of patients with PIMS-TS-related cardiac disease and 100% with COVID-19 had a normal echo at the time of discharge from hospital. Overall survival of patients was 100%.

The burden of cardiac disease in children requiring paediatric ICU care for COVID-19-related disease was high in the acute phase; however, all children survived, and all cardiac investigations had normalised by short-term follow-up.

To investigate functional outcomes in children who survived extracorporeal life support at 12 months follow-up post-discharge.

Some patients who require extracorporeal life support acquire significant morbidity during their hospitalisation. The Functional Status Scale is a validated tool that allows quantification of paediatric function.

A retrospective study that included children placed on extracorporeal life support at a quaternary children’s hospital between March 2020 and October 2021 and had follow-up encounter within 12 months post-discharge.

Forty-two patients met inclusion criteria: 33% female, 93% veno-arterial extracorporeal membrane oxygenation (VA ECMO), and 12% with single ventricle anatomy. Median age was 1.7 years (interquartile range 10 days–11.9 years). Median hospital stay was 51 days (interquartile range 34–91 days), and median extracorporeal life support duration was 94 hours (interquartile range 56–142 hours). The median Functional Status Scale at discharge was 8.0 (interquartile range 6.3–8.8). The mean change in Functional Status Scale from discharge to follow-up at 9 months (n = 37) was −0.8 [95% confidence interval (CI) −1.3 to −0.4, p < 0.001] and at 12 months (n = 34) was −1 (95% confidence interval −1.5 to −0.4, p < 0.001); the most improvement was in the feeding score. New morbidity (Functional Status Scale increase of ≥3) occurred in 10 children (24%) from admission to discharge. Children with new morbidity were more likely to be younger (p = 0.01), have an underlying genetic syndrome (p = 0.02), and demonstrate evidence of neurologic injury by electroencephalogram or imaging (p = 0.05).

In survivors of extracorporeal life support, the Functional Status Scale improved from discharge to 12-month follow-up, with the most improvement demonstrated in the feeding score.

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.