Haemolysis is developing prominence in the setting of supporting increasingly complex children with heart failure with a ventricular assist device. The goal of this study is to better characterise haemolysis and its implications in children supported with pulsatile ventricular assist devices.

This is a single-centre retrospective review of 44 children who were supported by Berlin Heart EXCOR between January 2006 and June 2020. Patients were divided into major haemolysers and non-major haemolysers. Major haemolysers were defined as patients with lactate dehydrogenase > 500U/L (2.5x the upper limits of normal) with either total bilirubin > 2mg/dL (with predominantly indirect hyperbilirubinemia) or anaemia out of proportion to the clinical scenario more than three days following implantation of the ventricular assist device(s). Patient demographics, ventricular assist device factors, and outcomes, including end-organ function and mortality, were compared between major haemolysers and non-major haemolysers.

Forty-four patients supported by the Berlin EXCOR were included in the analytic cohort of the study: 27 major haemolysers and 17 non-major haemolysers. Major haemolysis was more common in those supported with single-ventricle ventricular assist device (i.e., VAD in the context of functionally univentricular anatomy) compared to those with biventricular hearts, p = 0.01. There were no patients with an isolated left ventricular assist device or isolated right ventricular assist device in our analytic cohort of 44 patients. Of the 19 patients with single-ventricle ventricular assist device, 84% (16/19) were major haemolysers. Of the 25 patients with a biventricular assist device, 44% (11/25) were major haemolysers. Major haemolysers and non-major haemolysers had a body surface area of 0.28 and 0.40, respectively (p = 0.01). Overall, survival to discharge from the hospital was 66% (n = 29/44). Survival to discharge from the hospital was 52% (14/27) in major haemolysers versus 88% (15/17) in non-major haemolysers, p = 0.02. Only 3 of the 27 with major haemolysis had severe haemolysis, that is, lactate dehydrogenase > 2000 and bilirubin above 10. Non-major haemolysers had a better improvement in creatinine clearance during ventricular assist device support, p < 0.0001. (During the same era of this study, 22 patients who were supported with Berlin Heart were excluded from the analytic cohort because they did not have any recorded measurement of lactate dehydrogenase. Seventeen of these 22 patients had no clinical evidence of haemolysis. Survival to discharge from the hospital in this excluded cohort was 86% [19/22].)

Major haemolysis in patients with pulsatile ventricular assist device is more likely with single-ventricle ventricular assist device support and smaller body surface area.

The delivery of paediatric cardiac care across the world occurs in settings with significant variability in available resources. Irrespective of the resources locally available, we must always strive to improve the quality of care we provide to our patients and simultaneously deliver such care in the most efficient and cost-effective manner. The development of cardiac networks is used widely to achieve these aims.

This paper reports three talks presented during the 56th meeting of the Association for European Paediatric and Congenital Cardiology held in Dublin in April 2023.

The three talks describe how centres of congenital cardiac excellence can be developed in low-income countries, middle-income countries, and well-resourced environments, and also reports how centres across different countries can come together to collaborate and deliver high-quality care. It is a fact that barriers to creating effective networks may arise from competition that may exist among programmes in unregulated and especially privatised health care environments. Nevertheless, reflecting on the creation of networks has important implications because collaboration between different centres can facilitate the maintenance of sustainable programmes of paediatric and congenital cardiac care.

This article examines the delivery of paediatric and congenital cardiac care in resource limited environments, well-resourced environments, and within collaborative networks, with the hope that the lessons learned from these examples can be helpful to other institutions across the world. It is important to emphasise that irrespective of the differences in resources across different continents, the critical principles underlying provision of excellent care in different environments remain the same.

High takeoff of the right coronary artery suspected by echocardiography is widely considered a normal variant. However, in our experience, some patients initially thought to have a high takeoff of the coronary artery were later found to have an anomalous coronary origin with high-risk features. The aim of this study was to test the hypothesis that high takeoff of the right coronary artery suspected by echocardiography may indicate the presence of an anomalous coronary artery lesion with an intramural course requiring further investigation.

A retrospective chart review was performed of patients evaluated at the University of Florida Congenital Heart Center from January 2010 through September 2015. Charts of all 62 patients who were noted to have an anomalous coronary artery or concern for an anomalous coronary artery were reviewed to identify those who were initially identified as having simply a high takeoff of the right coronary artery by initial echocardiogram. A total of 24 patients met these criteria.

Out of 24 patients identified as having high takeoff of the right coronary artery on their initial echocardiogram, 20 had confirmatory computerized tomographic angiography. On review of these patients, 9 had a right coronary origin from the left. This included 3 patients with an anomalous right coronary artery from the left coronary sinus and 6 with an anomalous right coronary artery origin just above the left coronary sinus. Six of these 9 patients had an intramural course. The remaining patients had high takeoff above the right coronary sinus or normal coronary origins.

Additionally, on review of all patients with computerized tomographic angiographic confirmation of high takeoff of the coronary artery, those with high takeoff above the left coronary sinus were more likely to have an intramural course (6 out of 9). Meanwhile, none of the 6 patients with high takeoff above the right coronary sinus confirmed by computerized tomographic angiography had an intramural course.

Accurate identification of the coronary origin and course of the anomalous coronary artery is difficult by echocardiogram. Correct diagnosis of origin and course is important for appropriate risk stratification and treatment decisions. Therefore, patients with high takeoff of the right coronary artery suspected by echocardiography should undergo additional evaluation to assess for the presence of a potentially malignant course.

The study objective was to develop and validate a clinical decision support system (CDSS) to guide clinicians through the diagnostic evaluation of hospitalized individuals with suspected pulmonary tuberculosis (TB) in low-prevalence settings.

The “TBorNotTB” CDSS was developed using a modified Delphi method. The CDSS assigns points based on epidemiologic risk factors, TB history, symptoms, chest imaging, and sputum/bronchoscopy results. Below a set point threshold, airborne isolation precautions are automatically discontinued; otherwise, additional evaluation, including infection control review, is recommended. The model was validated through retrospective application of the CDSS to all individuals hospitalized in the Mass General Brigham system from July 2016 to December 2022 with culture-confirmed pulmonary TB (cases) and equal numbers of age and date of testing-matched controls with three negative respiratory mycobacterial cultures.

104 individuals with TB (cases) and 104 controls were identified. Prior residence in a highly endemic country, positive interferon release assay, weight loss, absence of symptom resolution with treatment for alternative diagnoses, and findings concerning for TB on chest imaging were significant predictors of TB (all P < 0.05). CDSS contents and scoring were refined based on the case–control analysis. The final CDSS demonstrated 100% sensitivity and 27% specificity for TB with an AUC of 0.87.

The TBorNotTB CDSS demonstrated modest specificity and high sensitivity to detect TB even when AFB smears were negative. This CDSS, embedded into the electronic medical record system, could help reduce risks of nosocomial TB transmission, patient-time in airborne isolation, and person-time spent reviewing individuals with suspected TB.

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.

Prior studies evaluating the impact of discontinuation of contact precautions (DcCP) on methicillin-resistant Staphylococcus aureus (MRSA) outcomes have characterized all healthcare-associated infections (HAIs) rather than those likely preventable by contact precautions. We aimed to analyze the impact of DcCP on the rate of MRSA HAI including transmission events identified through whole genome sequencing (WGS) surveillance.

Quasi experimental interrupted time series.

Acute care medical center.

Inpatients.

The effect of DcCP (use of gowns and gloves) for encounters among patients with MRSA carriage was evaluated using time series analysis of MRSA HAI rates from January 2019 through December 2022, compared to WGS-defined attributable transmission events before and after DcCP in December 2020.

The MRSA HAI rate was 4.22/10,000 patient days before and 2.98/10,000 patient days after DcCP (incidence rate ratio [IRR] 0.71 [95% confidence interval 0.56–0.89]) with a significant immediate decrease (P = .001). There were 7 WGS-defined attributable transmission events before and 11 events after DcCP (incident rate ratio 0.90 [95% confidence interval 0.30–2.55]).

DcCP did not result in an increase in MRSA HAI or, in WGS-defined attributable transmission events. Comprehensive analyses of the effect of transmission prevention measures should include outcomes specifically measuring transmission-associated HAI.

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.

The aim of this study was to determine the impact of the COVID-19 pandemic on test requests for the diagnosis and routine care of patients with various non-communicable diseases (NCD) across South Africa (SA).

A retrospective audit of laboratory test requests received from hospital outpatient departments and primary healthcare facilities across SA was performed. The following analytes were studied: glycated hemoglobin (HbA1c), lipids profiles, thyroid-stimulating hormone (TSH), and thyroxine (fT4), as well as triiodothyronine (fT3), serum protein electrophoresis (SPE), serum free light chains (SFLC), and prostate specific antigen (PSA); these tests were used as a proxy of NCD detection and follow-up. Requests received during the 3 waves of the pandemic were compared to requests received within the same period during 2017 - 2019.

During the first wave, requests for all analytes were reduced, with the biggest reduction observed for SPE (− 37%); TSH (− 29%); fT4 (− 28%); and HbA1c (− 25%). Requests received from urban facilities showed a larger decrease compared to those from rural facilities. During the third wave there was an increase in requests for all analytes; the biggest increase observed was for fT3 (21%) and HbA1c (18%).

The COVID-19 pandemic had a significant impact on the South African population receiving care in the public healthcare sector.