Physiologic data streaming and aggregation platforms such as Sickbay® and Etiometry are becoming increasingly used in the paediatric acute care setting. As these platforms gain popularity in clinical settings, there has been a parallel growth in scholarly interest. The primary aim of this study is to characterise research productivity utilising high-fidelity physiologic streaming data with Sickbay® or Etiometry in the acute care paediatric setting.

A systematic review of the literature was conducted to identify paediatric publications using data from Sickbay® or Etiometry. The resulting publications were reviewed to characterise them and identify trends in these publications.

A total of 41 papers have been published over 9 years using either platform. This involved 179 authors across 21 institutions. Most studies utilised Sickbay®, involved cardiac patients, were single-centre, and did not utilise machine learning or artificial intelligence methods. The number of publications has been significantly increasing over the past 9 years, and the average number of citations for each publication was 7.9.

A total of 41 papers have been published over 9 years using Sickbay® or Etiometry data in the paediatric setting. Although the majority of these are single-centre and pertain to cardiac patients, growth in publication volume suggests growing utilisation of high-fidelity physiologic data beyond clinical applications. Multicentre efforts may help increase the number of centres that can do such work and help drive improvements in clinical care.

Chronic pulmonary regurgitation following tetralogy of Fallot repair burdens the right ventricle. This study evaluated early outcomes of pulmonary valve reconstruction using right atrial tissue versus standard transannular patch repair.

A retrospective analysis of 412 tetralogy of Fallot patients (2014–2024) was conducted: Atrial Valve Group (n = 205) underwent valve reconstruction; No-Valve Group (n = 207) received standard repair. Patients were followed for 1 year with echocardiographic assessments. Outcomes included right ventricular insufficiency, ventilation duration, and ICU stay.

Atrial Valve Group had lower right ventricular insufficiency at 12 months (9.3% vs. 19.8%, p = 0.004, OR = 2.39, 95% CI: 1.32–4.33), shorter ventilation times (6.1 vs. 18.0 hours, p < 0.001, Cohen’s d = 3.54), and reduced ICU stays (3.0 vs. 5.7 days, p < 0.001, Cohen’s d = 1.87), despite longer CPB durations (47.1 vs. 40.5 minutes, p = 0.02).

Right atrial tissue reconstruction reduces early and intermediate-term right ventricular dysfunction post-tetralogy of Fallot repair. Long-term studies are needed.

Danon disease is an X-linked disorder caused by variants in the lysosome-associated membrane protein-2 (LAMP2) gene located on Xq24. Due to its inheritance in an X-chromosome dominant manner, males typically experience more severe manifestations than females.

The whole exome sequencing was conducted on a cohort of 218 children diagnosed with hypertrophic cardiomyopathy; four children with hypertrophic cardiomyopathy carrying the LAMP2 variants were diagnosed at our centre. Variants in the LAMP2 gene were summarised, and their pathogenicity and conservation were analysed using bioinformatics methods. A retrospective analysis of genotype-phenotype associations was also conducted in conjunction with previously reported cases.

Four patients with Danon disease were diagnosed in our single centre by gene sequencing; they all presented with myocardial hypertrophy as the initial manifestation. Both male patients manifested symptoms from infancy, while disease onset in the two female cases occurred below the average age reported for females. Through gene sequencing, a total of four variants were identified in these four patients, including one splicing variant: c.865-1G>C, one loss of heterozygosity variant: loss1 exon:4-9), one frameshift variant: c.973delG(p.(L325Wfs×21)), and one stop codon variant: c.467T>G(p.(L156*)).

This study identified four patients with LAMP2 gene variants, thereby enriching the documented genetic landscape of LAMP2-associated disorders. Bioinformatics analyses corroborated the pathogenicity of these variants. Additionally, we emphasised that women with suspected Danon disease should be thoroughly evaluated, and the possibility of implantable cardioverter defibrillator implantation and heart transplantation should be considered and discussed as early as possible.

The limited efficacy of monotherapy and the insufficient clinical experience with triple therapy (levosimendan, dapagliflozin, and sacubitril/valsartan) warrant further investigation. The aim of this study was to evaluate the effects of triple therapy on left ventricular function in children with advanced heart failure whose left ventricular function had not improved despite classical heart failure treatment and who remained dependent on inotropes.

The study included children who were admitted to the hospital with advanced heart failure and who were still inotrope-dependent at a mean of 42 days after the start of classical heart failure treatment and then started triple therapy at our hospital.

The study included 18 patients, 8 (44%) males, with a median age of 4 years (2–7 years). Before and after classical treatment and after triple treatment, statistically significant improvement in two-dimensional left ventricular ejection fraction (%) (median values 30; 38; 55, respectively), left ventricular end-diastolic diameter (median values 44; 45; 40 mm), left ventricular end-systolic diameter (median values 38; 36; 29 mm), left ventricular end-diastolic diameter (z score) (median values 4.2; 3.2; 2.7), left ventricular end-systolic diameter (z score) (median values 5.8; 4.8; 3.2), Simpson left ventricular ejection fraction (%) (median values 29; 36.5; 55), Simpson left ventricular end-diastolic volume (median values 60; 55; 43 ml), left ventricular end-systolic volume (median values 43; 40; 18. 5 ml), left ventricular global longitudinal strain four-chamber (median values -8.1;-10;-19), left ventricular global longitudinal strain three-chamber (median values -5.9;-8.9;-14), and left ventricular global longitudinal strain mean (median values -6.9;-9.7;-19) values was observed (all values p < 0.05).

In children admitted to the hospital with advanced heart failure whose left ventricular function has not improved with classical therapy, it seems likely that both left ventricular systolic and diastolic function will improve, inotrope dependency will resolve, and patients can be discharged with the new triple drug therapy.

Describe the hemodynamic implications of anaesthetic choice among children with heart disease undergoing cardiac catheterisation.

Study 1 was a secondary analysis of data obtained during catheterisation-based hemodynamic assessment of infants with hypoplastic left heart syndrome following Stage 1 palliation, randomised in the Single Ventricle Reconstruction trial. Measured and calculated hemodynamics including pulmonary and systemic vascular resistance indexed to body surface area (PVRi and SVRi respectively) and pulmonary/systemic blood flow (Qp/Qs) were analysed with respect to anaesthetic employed during catheterisation, classified as moderate sedation or general anaesthesia. Study 2 consisted of a single centre, prospective analysis of patients requiring percutaneous closure of a patent ductus arteriosus or endomyocardial biopsy after orthotopic heart transplant. Participants underwent hemodynamic assessment first using inhaled volatile anaesthesia (IA), and then transitioned to total intravenous anaesthesia, comparing hemodynamic measures with respect to anaesthetic approach.

In Study 1, independent of shunt type, PVRi, and patient size, moderate sedation was associated with a greater than two-fold odds of a Qp/Qs >1 (OR 2.12, 95%CI 1.18–3.87, p = 0.013). In Study 2, while PVRi was similar, SVRi was significantly higher using total intravenous anaesthesia. Among the patent ductus arteriosus subgroup, Qp/Qs increased significantly with a total intravenous anaesthesia relative to IA (p = 0.003); additionally, among the orthotopic heart transplant subgroup, left ventricular end diastolic pressure increased following a transition to total intravenous anaesthesia (p = 0.002).

Analyses of hemodynamics during catheterisation support a significant impact of anaesthetic type on hemodynamic values including SVRi, left ventricular end diastolic pressure, and Qp/Qs. Anaesthesia choice and intraprocedural management of SVRi are important considerations when making clinical decisions based on hemodynamic data.

Implantable haemodynamic monitors allow remote monitoring of Fontan circulation. We report unique opportunities and challenges related to device use in rural, high-altitude regions.

Assess the performance of implantable haemodynamic monitor in Fontan circulation and identify potential sources of measurement discrepancy defined as non-physiological, negative, or significantly lower reading than baseline.

We performed a retrospective review of patients who underwent implantable haemodynamic monitor implantation from September 2021 to April 2024 (n = 17) at our centre (∼1,000 feet above sea level; ASL) and identified those with sensor discrepancies.

During a mean follow-up duration of 26 months (range 13–44 months), there were no procedure-related complications, thromboembolism, or device displacement. Ten patients lived in rural, higher-altitude regions (average altitude 5100 feet above sea level, average distance from centre ∼160 miles, range = 100–400 miles). Challenges in remote monitoring included unreliable home-internet connection, non-compliance, and difficulty performing device recalibration at patient’s home altitude. Sensor discrepancies were noted in 7 patients (41%), of whom 6 (86%) lived remotely. Manual review of the waveforms identified sources of discrepancy, including misinterpretation of the non-pulsatile pressure waveform (n = 3), offset due to change in hospital-interrogation unit (n = 4), and sensor drift (n = 1). Altitude change did not directly affect sensor performance. We were able to apply corrective interventions in 4/7 sensors, including Fontan-specific settings (overriding pulsatility), and back-end recalibration, which were effective in improving device accuracy.

Implantable haemodynamic monitors are a promising tool for monitoring Fontan circulation but may require modified settings and careful attention to potential interpretation errors. Home monitoring remains challenging for rural, high-altitude residents with limited resources.

There is geographic disparity in the provision of Pediatric and Congenital Heart Disease (PCHD) services; Africa accounts for only 1% of global cardiothoracic surgical capacity. Methods: We conducted a survey of PCHD services in Africa, to investigate institution and national-level resources for pediatric cardiology and cardiothoracic surgery. Results were compared with international guidelines for PCHD services and institutions were ranked by a composite score for low- and middle-income PCHD services. Results: There were 124 respondents from 96 institutions in 45 countries. Eighteen (40%) countries provided a full PCHD service including interventional cardiology and cardiopulmonary bypass (CPB) cardiac surgery. Ten countries (22%) provided cardiac surgery services but no interventional cardiology service, 4 of which did not have CPB facilities. One provided interventional cardiology services but no cardiac surgery service. Ten countries (22%) had no PCHD service. There were 0.04 (interquartile range [IQR]: 0.00-0.13) pediatric cardiothoracic surgeons and 0.17 (IQR: 0.02-0.35) pediatric cardiologists per million population. No institution met all criteria for level 5 PCHD national referral centers, and 8/87 (9.2%) met the criteria for level 4 regional referral centers. Thirteen (29%) countries report both pediatric cardiology and cardiothoracic surgery fellowship training programs. Conclusions: Only 18 (40%) countries provided full PCHD services. The number of pediatric cardiologists and cardiothoracic surgeons is below international recommendations. Only Libya and Mauritius have the recommended 2 pediatric cardiologists per million population, and no country meets the recommended 1.25 cardiothoracic surgeons per million. There is a significant shortage of fellowship training programs which must be addressed if PCHD capacity is to be increased.

Although the importance of advance care planning (ACP) for individuals with adult congenital heart disease (ACHD) has been established, there is no consensus regarding the optimal age to initiate ACP discussions. We asked ACHD patients their opinions about the timing of the first ACP discussion.

Adult patients seen in an outpatient ACHD clinic from April to August 2018 completed a self-administered questionnaire that evaluated opinions regarding the content and timing of ACP discussions, end-of-life communication preferences, and anticipated emotional responses to ACP discussions.

Ninety-five patients participated. Median age was 34.8 years (Q1 – Q3: 28.4 - 47.1 years), 53% (n = 50) were female, and 91% (n = 86) had great or moderate disease complexity. Although 75% (n/N = 69/92) thought ACP was important, only 37% (n/N = 35/94) had completed advance directives. Most (79%, n/N = 72/91) preferred ACP conversations early, either before getting sick (44%, n = 40/91) or when first diagnosed with a life-threatening illness (35%, n = 32/91). Responses varied regarding the appropriate age for first ACP conversations: 28% (n/N = 25/88) chose options ≤ 15 years, 23% (n/N = 20/88) 16–17 years, 32% (n/N = 28/88) 18–20 years, and 17% (n/N = 15/88) ≥ 21 years old.

ACHD patients value ACP discussions and think they should occur early in the disease course, before patients face a life-threatening disease complication, yet most think these conversations should wait until later adolescent or young adult years. ACP readiness should be assessed to determine the optimal timing of ACP discussions.