Paediatric heart failure is a common clinical syndrome that may be experienced by children with congenital heart disease (CHD) and/or cardiomyopathy. It is characterised by clinical signs/symptoms which reflect the underlying pathophysiology based on one of three main clinical states: Pulmonary over-circulation, pressure overload, and ventricular failure. Current diagnosis relies on clinical assessment and echocardiogram imaging as cardiac biomarkers has been predominantly scientific to date. This review provides a comprehensive overview of paediatric heart failure pathophysiology and considers the available evidence for cardiac biomarkers in this setting.

A literature review was completed using MEDLINE ALL, EMBASE, and PubMed on 10th November, 2022. Search terms included biomarkers, heart failure, heart defects, congenital heart disease, fontan circulation, single ventricle circulation, cardiomyopathy, and child. This allowed the identification of individual cardiac biomarkers which are the focus of this review. These included NT-proBNP, MR-proANP, MR-proADM, troponin, sST2, galectin 3, and growth differentiation factor-15.

Paediatric studies have established reference ranges for NT-proBNP and troponin for children with structurally normal hearts. Of all the biomarkers reviewed, NT-proBNP appears to correlate most closely with symptoms of heart failure and ventricular dysfunction on echocardiogram. However, there remains limited longitudinal data for NT-proBNP, and no validated reference ranges for patients with CHD and/or cardiomyopathy. None of the other biomarkers reviewed were consistently superior to NT-proBNP.

Further large paediatric studies of patients with heart failure are needed to validate NT-proBNP in CHD and to evaluate the role of novel biomarkers in specific types of CHD, e.g. single ventricle physiology.

Diversification of the medical and cardiothoracic surgical workforce represents an ongoing need. A congenital cardiac surgery shadowing programme for undergraduate students was implemented at the University of Florida Congenital Heart Center.

Students shadowing in the Congenital Heart Center from 17 December 2020 through 20 July 2021 were sent a survey through Qualtrics to evaluate the impact of their shadowing experience. The main objectives of the survey were to determine the personal relationship(s) of the students to physicians prior to shadowing, how the presence or absence of physicians in the family of a given student related to the exposure of the student to a medical setting prior to shadowing, and the interest of the students in medicine and cardiothoracic surgery prior to and after the shadowing experience. Survey responses included “Yes/No” questions, scaled responses using a Likert scale, selection lists, and free text responses. When applicable, t-tests were utilised to assess differences between student groups.

Of the 37 students who shadowed during the study period, 26 (70%) responded. Most students were female (58%, n = 15), and the mean age was 20.9 ± 2.4 years. Students spent a mean duration of 95 ± 138 hours shadowing providers as part of the shadowing programme. Likert scale ratings of interest in the professions of medicine, surgery, and cardiothoracic surgery all increased after the shadowing experience (p < 0.01). Students with a family member in medicine had more clinical exposure prior to the shadowing programme (p < 0.01).

A surgical shadowing programme at a Congenital Heart Center may have an important formative impact on the views of undergraduate students regarding potential careers in surgery and medicine. Additionally, students without family members in medicine tend to have less prior exposure to medicine and could likely benefit more from this type of shadowing programme.

Cardiac manifestations of the coronavirus disease 2019 (COVID-19) have mainly been reported in adults. Therefore, we aimed to determine the electrocardiographic abnormalities in hospitalised paediatric patients with COVID-19 and multisystemic inflammatory syndrome in children.

We retrospectively evaluated hospitalised paediatric patients <18 years of age with a diagnosis of COVID-19 (n = 168) and multisystem inflammatory syndrome in children (n = 48) between March 2021 and December 2021. A daily electrocardiography was performed for the patients who had electrocardiographic abnormalities on admission or developed electrocardiographic abnormality on the follow-up. The characteristics of these patients, underlying predisposing conditions, and clinical course were also examined.

Two-hundred sixteen paediatric patients (55% were male) with a mean age of 10.7 ± 4.69 years were evaluated. There was an underlying disease in 84 (38.8%) patients and 51 (23.6%) required paediatric ICU admission. Electrocardiography abnormality was detected in 12 (5.5%) which were as follows: 7 (3.2%) had sinus bradycardia, 3 (1.4%) patients had transient ST elevation and concomitant T negativity, and 2 (0.9%) developed first-degree Atrioventricular (AV) block. The median time from the onset of disease symptoms to detecting electrocardiographic abnormality was 9 days. Electrocardiographic abnormalities returned to normal uneventfully 3 days later.

The prevalence of arrhythmia in paediatric patients with COVID-19 was detected in 5.5% of the patients. While two-thirds of the electrocardiography abnormalities were sinus bradycardia, ST elevation was remarkable (1.4%). Clinicians should be aware of electrocardiographic abnormalities and consider electrocardiographic monitoring in paediatric patients with COVID-19 and multisystemic inflammatory syndrome in children.

Ventricular septal defect is one of the commonest heart defect in children and closure of this defect with devices has seen a rapid progression over a period of time. The availability of new and safer devices has made the transcatheter closure of ventricular septal defect a suitable option even in young children.

The study was done to evaluate the feasibility and complications of device closure of ventricular septal defect in children weighing 10 kg or less with different types of devices.

The present study was undertaken in a newly established dedicated Paediatric Cardiac Unit at a Tertiary Care Hospital. Relevant data were obtained retrospectively from the case files and the catheterisation records and data were analysed for first 50 patients with ventricular septal defect weighing 10 kg or less between March 2018 and March 2021.

Among these 50 patients selected, device closure was successfully done in 45 (90%) cases while 5 (10%) attempts were unsuccessful for various reasons. The mean weight in this study was 7.46 ± 1.89 kg (2.3–10 kg), 21 (42%) cases were females while 29 (58%) were males; mean age was 19.4 ± 11.88 months (4–48 months). Right heart catheterisation study showed 21 (42%) patients with normal pulmonary artery pressures (no pulmonary artery hypertension). Among 29 patients with pulmonary arterial hypertension, 13 patients (22%) were having mild pulmonary arterial hypertension, 4 (8%) were with moderate pulmonary arterial hypertension, and 12 (24%) were with severe pulmonary arterial hypertension. Mean Qp/Qs was 2.73 ± 0.72 (2.5–4.5) and mean pulmonary vascular resistance was 1.5 ± 1.04 (0.6–4.6 WU). Amplatzer Duct Occluder (ADOI) was used in 15 (30%) cases, 27 (52%) cases were closed with Amplatzer Duct Occluder (ADOII), and the 3 (6%) cases closure was done with Amplatzer muscular ventricular septal defect occluder.

Transcatheter closure of ventricular septal defect in children 10 kg or less is feasible and safe alternative to surgical ventricular septal defect closure. The immediate and short-term outcomes have proven this method to be safe and valid.

In the absence of randomised trials for paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV2 (PIMS-TS), optimal management of PIMS-TS-patients remains somewhat uncertain. We aimed to evaluate the practicability of consensus diagnostic/therapeutic pathways in a real-life German hospital setting.

All children treated for PIMS-TS (February to November, 2021) at the Childrens’ Hospital Kassel were analysed retrospectively. Patients were treated according to local PIMS-TS standardised operating procedure based on the Swiss and UK consensus statements

Eleven patients treated for PIMS-TS were included in this study (female:male = 2.1:1). According to the categories of the Swiss and UK consensus statements, 36% were uncomplicated hyperinflammation, 36% Kawasaki-like and 27% shock-like disease. Local estimated incidence was 0.92/1000 Covid-19 cases in children aged 4–15 years. Significant inter-group differences in laboratory parameters were found: BNP was highest in shock-like presentation compared to Kawasaki-like and uncomplicated hyperinflammation (median 954 (668–1491) versus 213 (173–934) versus 80 (5–257) ng/l, p = 0.02), whereas troponin was highest in Kawasaki-like, followed by shock-like presentation and uncomplicated hyperinflammation (median 34.7 (27.5–58.4) versus 19.1 (14.1–23.4) versus 1.9 (1.9–16.4) ng/l, p = 0.02). Patients with shock-like presentation needed circulatory resuscitation in the paediatric ICU. All patients received standardised operating procedure-based therapy and were discharged home after a medium of 7.4 days.

The Swiss and UK consensus statements on the management of PIMS-TS proved very valuable in a real-life clinical setting, facilitated early categorisation, and initiation of specific therapy, possibly improving the outcome. Additional randomised trials are necessary to further improve the management of PIMS-TS

CHD was the most prevalent congenital anomaly (60.9 per 10,000, 95% CI 59.0–62.8) in England in 2018, with 1767 babies born with severe cardiac defects. The 30-day survival rates for complex procedures continue to improve; however despite care advances, the early post-operative period and first year of life remain a critical time for these infants. The Congenital Heart Assessment Tool was developed to support parental decision-making, standardise care provision, improve communication, and the safety and quality of care.

To further evaluate the Congenital Heart Assessment Tool.

A four centre collaborative mixed-methods quality improvement project funded by The Health Foundation, involving eight phases conducted during 2016–2018. Phases six to eight (clinical simulation exercise, parent workshop, and updated tool) are reported in this paper.

Four themes emerged from the clinical simulation exercise (phase six) including: improving documentation; preparation of parents; preparation of health care professionals; and communication. One main theme emerged from the parent workshop (phase seven): “what parents know versus what professionals know [about CHD]”.

These phases further validated the effectiveness of the CHATm in terms of triggering amber and red indicators and demonstrated parents’ ability to identify deterioration in their infant’s clinical condition. Recommendations arising from the quality improvement project enabled the project team to create an updated version of the Congenital Heart Assessment Tool, CHAT2.

Patients with single ventricle CHD have significant morbidity and healthcare utilisation throughout their lifetime, including non-cardiac hospital admissions. Respiratory viral infections are the main cause of hospitalisation in children, but few data exist for single ventricle patients. We sought to identify how respiratory viral infections impact patients with single ventricle CHD and potential differences between Glenn and Fontan circulation.

We conducted a retrospective study of patients seen from 01/01/2011–12/31/2020. We identified patients with a history of single ventricle CHD and Glenn palliation, and a normoxic control group with isolated atrial septal defect requiring surgical closure. We compared viral-related clinical presentations, admissions, and admission characteristics.

A total of 312 patients were included (182 single ventricle, 130 atrial septal defect). Single ventricle patients were more likely than children with isolated atrial septal defect to be admitted with a respiratory virus (odds ratio 4.15 [2.30–7.46]), but there was no difference in mechanical ventilation or hospital length of stay (p = 0.4709). Single ventricle patients with Glenn circulation were more likely than those with Fontan circulation to present and be admitted (odds ratio 3.25 [1.62–6.52]), but there was no difference in ICU admission, mechanical ventilation, or hospital length of stay (p = 0.1516).

Respiratory viral infections are prevalent but uncomplicated in patients with single ventricle CHD. Viral-related presentations and admissions are more prevalent during the period of Glenn circulation compared to Fontan circulation; however, rate of mechanical ventilation and hospital length of stay are similar.

Cardiovascular disease is one of the most important problems in long-term follow-up for Noonan syndrome. We examined cardiovascular issues and clinical manifestations, with a focus on the cardiovascular disease and prognosis of patients with Noonan syndrome.

This single-centre study evaluated patients who were clinically and genetically diagnosed with Noonan syndrome.

Forty-three patients diagnosed with Noonan syndrome were analysed. The most prevalent responsible mutation was found in PTPN11 (25/43). The second and third most prevalent causative genes were SOS1 (6/43) and RIT1 (5/43), respectively, and 67.4% of genetically diagnosed patients with Noonan syndrome had structural cardiovascular abnormalities. Pulmonary valve stenosis was prevalent in patients with mutations in PTPN11 (8/25), SOS1 (4/6), and RIT1 (4/5). Hypertrophic cardiomyopathy was found in two of three patients with mutations in RAF1. There was no difference in the cardiovascular events or cardiovascular disease prevalence in patients with or without PTPN11 mutations. The proportion of RIT1 mutation-positive patients who underwent intervention due to cardiovascular disease was significantly higher than that of patients with PTPN11 mutations. Patients who underwent any intervention for pulmonary valve stenosis exhibited significantly higher pulmonary flow velocity than patients who did not undergo intervention, when they visited our hospital for the first time. All patients who underwent intervention for pulmonary valve stenosis had a pulmonary flow velocity of more than 3.0 m/s at first visit.

These findings suggest that genetic information can provide a clinical prognosis for cardiovascular disease and may be part of genotype-based follow-up in Noonan syndrome.

Nutritional assessment appears to be an essential component of the evaluation of children with CHD undergoing surgery because nutritional status may impact corrective surgery-associated morbidity.

A prospective single-centre cohort study with children between 6 and 24 months of age. Patients who had genetic syndromes or those who were premature or low birthweight at birth were excluded. Pre-operative nutritional parameters included anthropometric measurements and serum concentrations of total protein, vitamin D, iron, and ferritin. Outcome measures included ICU length of stay, mechanical ventilation, vasoactive-inotropic score, and duration of inotropes. Linear regression analysis was performed to determine whether pre-operative variables were associated with outcomes.

Analysis was performed on 120 patients (median age of 8 months), of whom 67 were male. Prior to surgery, 50.8% of patients had reduced (z ≤ −2.0) weight-for-age z score, 23.3% had reduced length-for-age z score, and 59.2% had reduced mid-upper arm circumference z score. Pre-operative serum total protein levels were 59.36 ± 9.16 g/L. Multiple regression analysis showed that low serum protein was associated with longer ICU length of stay and length of mechanical ventilation, while mid-upper arm circumference z score ≤ −2 was associated with longer ICU length of stay and mechanical ventilation and inotropes duration.

Pre-operative assessment of nutritional status by performing anthropometric and biochemical measurements including mid-upper arm circumference z score and serum protein concentrations in children undergoing CHD surgery appears to be predictors of some post-operative short-term outcomes and could be used as a guide to highlight patients needing appropriate perioperative nutritional interventions.

This study aimed to evaluate the role of real-time three-dimensional (known as four-dimensional) echocardiography and three-dimensional speckle-tracking echocardiography for the early detection of left ventricular systolic dysfunction in asymptomatic children with type 1 diabetes mellitus.

This cross-sectional study included 38 patients (mean age 15.4 ± 2.9, 42.1% male) and 38 age, gender, and body measurements matched healthy children. Each patient underwent an interview about medical history, a detailed clinical examination, blood laboratory tests, conventional echocardiography, and tissue Doppler imaging. Left ventricular ejection fraction; global longitudinal, circumferential, radial strain; twist; and torsion were measured by real-time three-dimensional and speckle-tracking echocardiography.

Conventional echocardiography and tissue Doppler imaging showed normal left ventricular systolic function in the patients. Although left ventricular ejection fraction (61.6 ± 1.4%, 61.8 ± 1.1%, p = 0.386), global longitudinal [−26.6 (−27.7 to −26.1)%, −26.2 (−27.7 to −24.9)%, p = 0.224], and radial strain [44.4 (42.4–45.9)%, 43.9 (41–46.1)%, p = 0.513] were similar to the controls, circumferential strain (−27.3 ± 1.3%, −28.0 ± 1.6%, p = 0.048) and twist (9.5 ± 2.3°, 11.4 ± 3.0°, p = 0.003) were decreased in the patients compared with controls.

We found that even in children with asymptomatic type 1 diabetes mellitus with normal left ventricular ejection fraction, circumferential function was impaired and rotation pattern was changed. This novel echocardiographic method might be an important tool for detecting left ventricular systolic dysfunction in type 1 diabetes mellitus children before it becomes overt on conventional echocardiography and tissue Doppler imaging.

This study evaluates the retrograde approach compared to the antegrade approach in infants with PA-IVS who underwent transcatheter pulmonary valvotomy procedure at National Cardiovascular Center Harapan Kita, Jakarta, Indonesia.

This is a single-centre retrospective study conducted from January 2017 to June 2019 consisting of infants undergoing transcatheter pulmonary valvotomy procedures from our centre.

Among 3733 records of cardiac catheter procedure in paediatric patients during the last 3 years, there were 12 subjects with PA-IVS, where five subjects were done by antegrade approach and seven by retrograde approach. The retrograde approach is shown to excel the antegrade approach in terms of procedural time by 58.64 minutes (CI 95 % 32.97–84.29, p = 0.008) and PA-RV crossing time by 27 minutes (CI 95 % 14.01–39.99, p = 0.02). There was no significant difference in contrast used (120.23 ± 25.77 versus 150.27 ± 39.26 ml/BSA, p = 0.518), and right ventricle to pulmonary artery systolic pressure gradient after valvotomy (39.571 ± 5.814 versus 53.52 ± 29.15, p = 0.329) between the retrograde and the antegrade approach.

The retrograde approach offered shorter procedural time and comparably satisfying results than the antegrade approach. The shorter procedural time was preferred due to the shorter duration of general anaesthesia, which may decrease the risk of neurodevelopmental deficits in the patient.

The Etanercept as Adjunctive Treatment for Acute Kawasaki Disease, a phase-3 clinical trial, showed that etanercept reduced the prevalence of IVIg resistance in acute Kawasaki disease. In patients who presented with coronary artery involvement, it reduced the maximal size and short-term progression of coronary artery dilation. Following up with this patient group, we evaluated the potential long-term benefit of etanercept for coronary disease.

Patients were followed for at least 1 year after the trial. The size of dilated arteries (z-score ≥ 2.5) was measured at each follow-up visit. The z-score and size change from baseline were evaluated at each visit and compared between patients who received etanercept versus placebo at the initial trial.

Forty patients who received etanercept (22) or placebo (18) in the Etanercept as Adjunctive Treatment for Acute Kawasaki Disease trial were included. All patients showed a persistent decrease in coronary artery size measurement: 23.3 versus 5.9% at the 6-month visit, 24 versus 13.1% at the 1-year visit, and 20.8 versus 19.3% at the ≥ 2-year visit for etanercept or placebo, respectively, with similar results for decrease in coronary artery z-scores. In a multivariate analysis, correcting for patients’ growth, a greater size reduction for patients on the etanercept arm versus placebo was proved significant for the 6-month (p = 0.005) and the 1-year visits (p = 0.019) with a similar end outcome at the ≥ 2-year visit.

Primary adjunctive therapy with etanercept for children with acute Kawasaki disease does not change the end outcome of coronary artery disease but may promote earlier resolution of artery dilation.

Bronchiolitis obliterans organising pneumonia is a rare complication associated with calcineurin inhibitors and mammalian target of rapamycin inhibitors. While bronchiolitis obliterans organising pneumonia in adult transplant patients has been reported, it has not been well described in pediatric transplant patients.

We present a case of a 19-month-old male patient with dilated cardiomyopathy who underwent orthotropic heart transplantation at 14 months of life for heart failure refractory to medical therapy. Approximately 4 months post-transplant, he presented with diarrhea and vomiting with acute kidney injury secondary to dehydration. His tacrolimus level on admission and first week of hospitalisation was within target range of 10–12 ng/ml. He was diagnosed with esophagitis and prescribed proton pump inhibitors. Our patient subsequently developed significant respiratory distress with initial chest radiograph showing right lower lobe opacities. Repeat tacrolimus at the time of worsening respiratory status was 84.2 ng/dL and his tacrolimus was held. He required intubation due to significant hypoxia with progression of lung to disease and development of diffuse bilateral opacities consistent with acute respiratory distress syndrome. Despite initiation of steroids and aggressive ventilator management, he continued to be hypoxic on maximal respiratory support. After 28 days post admission, support was withdrawn. On autopsy, his lung biopsy findings were consistent with bronchiolitis obliterans organising pneumonia.

Life-threatening bronchiolitis obliterans organising pneumonia can be seen in pediatric transplant patients on tacrolimus or when transitioning from tacrolimus to sirolimus, highlighting the need for close monitoring of heart transplant patients on immunosuppressive medications presenting with hypoxia.