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.

This study aimed to analyse the influence of improved antenatal detection on the course, contemporary outcomes, and mortality risk factors of the complete atrioventricular block during fetal-neonatal and childhood periods in South Wales.

The clinical characteristics and outcomes of complete atrioventricular block in patients without structural heart disease at the University Hospital of Wales from January 1966 to April 2021 were studied. Patients were divided into two groups according to their age at diagnosis: I-fetal-neonatal and II-childhood. Contemporary outcomes during the post-2001 era were compared with historical data preceding fetal service development and hence earlier detection.

There were 64 patients: 26 were identified in the fetal-neonatal period and the remaining 38 in the childhood period. Maternal antibodies/systemic lupus erythematosus disease (anti-Ro/Sjögren’s-syndrome-related Antigen A and/or anti-La/Sjögren’s-syndrome-related Antigen B) were present in 15 (57.7%) of the fetal-neonatal. Fetal/neonatal and early diagnosis increased after 2001 with an incidence of 1:25000 pregnancies. Pacemaker implantation was required in 34 patients, of whom 13 were diagnosed in the fetal-neonatal group. Survival rates in cases identified before 2001 were at 96.3% (26/27), whereas it was 83.8% (31/37) in patients diagnosed after 2001 (P > 0.05). Other mortality risk factors comprised a lower gestational week at birth, maternal antibodies, and an average ventricular heart rate of < 55 bpm.

Fetal diagnosis of complete atrioventricular block is still portends high fetal and neonatal mortality and morbidity despite significantly improved antenatal detection after 2001. Pacemaker intervention is needed earlier in the fetal-neonatal group. Whether routine antenatal medical treatment might alter this outcome calls for further prospective multicentre studies.

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.

Invasive haemodynamics are often performed for initiating and guiding pulmonary artery hypertension therapy. Little is known about the predictive value of invasive haemodynamic indices for long-term outcomes in children with pulmonary artery hypertension. We aimed to evaluate invasive haemodynamic data to help predict outcomes in paediatric pulmonary artery hypertension.

Patients with pulmonary artery hypertension who underwent cardiac catheterisation (2006–2019) at a single centre were included. Invasive haemodynamic data from the first cardiac catheterisation and clinical outcomes were reviewed. The combined adverse outcome was defined as pericardial effusion (due to right ventricle failure), creation of a shunt for pulmonary artery hypertension (atrial septal defect or reverse Pott’s shunt), lung transplant, or death.

Among 46 patients with a median [interquartile range (IQR)] age of 13.2 [4.1–44.7] months, 76% had CHD. Median mean pulmonary artery pressure was 37 [28–52] mmHg and indexed pulmonary vascular resistance was 6.2 [3.6–10] Woods units × m2. Median pulmonary artery pulsatility index was 4.0 [3.0–4.7] and right ventricular stroke work index was 915 [715–1734] mmHg mL/m2. After a median follow-up of 2.4 years, nine patients had a combined adverse outcome (two had a pericardial effusion, one underwent atrial level shunt, one underwent reverse Pott’s shunt, and six died). Patients with an adverse outcome had higher systolic and mean pulmonary artery pressures, higher diastolic and transpulmonary pressure gradients, higher indexed pulmonary vascular resistance, higher pulmonary artery elastance, and higher right ventricular stroke work index (p < 0.05 each).

Invasive haemodynamics (especially mean pulmonary artery pressure and diastolic pressure gradient) obtained at first cardiac catheterisation in children with pulmonary artery hypertension predicts outcomes.

The diagnosis of Duchenne-linked cardiomyopathy may be challenging. Conventional echocardiographic measurements typically show deterioration beyond the second decade. Global longitudinal strain has been proposed as an earlier marker than left ventricular ejection fraction.

A prospective, observational, cross-sectional, case-control study was carried out. Both Duchenne patients and control subjects underwent transthoracic echocardiogram in order to assess left ventricle function. Bayesian linear regression was the main tool for inference. Age effects were parameterised by means of a spline function because of its simplicity to characterise continuous variables and smooth contributions. The posterior distribution of the marginal age effects was used to assess the earliest age of deterioration of each marker.

Sixteen Duchenne patients and twenty-two healthy male subjects were enrolled. On overage, cardiac function measures were found for ejection fraction and longitudinal strain among different groups. Age effects on global longitudinal strain are more reliably found at ages of 6 and above, while ejection fraction starts to deteriorate at an older age. Progressive left ventricular dysfunction in Duchenne patients is one of the key issues and starts at an early age with subtle symptoms.

This cross-sectional study provides supporting evidence that global longitudinal strain is an earlier marker of disease progression than ejection fraction in Duchenne patients.

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.

Systemic lupus erythematosus in children generally manifests more severely with a more aggressive disease course. Cardiac involvement in systemic lupus erythematosus often does not show specific signs and symptoms, but speckle-tracking echocardiography can detect cardiac dysfunction. This study aimed to determine the differences in left ventricular function as measured by speckle-tracking echocardiography in children with various severity of systemic lupus erythematosus activity.

A cross-sectional study of 49 children diagnosed with systemic lupus erythematosus are currently undergoing outpatient or inpatient care at Dr Hasan Sadikin General Hospital, Bandung, from May 2023 to June 2023. Disease activity was assessed by Mexican Version of the Systemic Lupus Erythematosus Disease Activity Index (MEX-SLEDAI) with a score of 2–5 classified as mild activity, 6–9 as moderate, and ≥10 as severe. Each subject underwent conventional echocardiography and speckle-tracking echocardiography with a Philips EPIQ machine performed by a Pediatric Cardiologist Consultant 10 days after inclusion.

Fifteen (30.6%) subjects had mild disease activity, and 34 (69.4%) subjects had moderate disease activity. Most subjects (81.96%) were female with an average age of 15 years. The mean ejection fraction and fractional shortening as well as the median E/A ratio in the mild and moderate disease activity groups were not significantly different (65.76 versus 67.38%, 35.73 versus 37.11%, 1.6 versus 1.5%, respectively, p > 0.005). The global longitudinal strain in the moderate activity group was reduced more significantly than in the mild activity group (−16.58 versus −19.65, p = 0.008).

Left ventricular function as measured by speckle-tracking echocardiography was lower in children with moderate systemic lupus erythematosus activity than those with mild disease activity.

Paediatric cardiac electrophysiologists are essential in CHD inpatient care, but their involvement is typically limited to consultation with individual patients. In our integrated heart centre, an electrophysiologist reviews all cardiac inpatient telemetry over the preceding 24 hours and participates in daily multidisciplinary morning report. This study investigates the impact of the strategy of consistent, formalised electrophysiologist presence at multidisciplinary morning report.

This is a single-centre, prospective, observational study of electrophysiologist participation in patient encounters during heart centre multidisciplinary morning report from 10/20/2021 to 10/31/2022. Multidisciplinary morning report includes discussion of all intensive care and non-intensive care cardiac patients. An encounter was defined as reporting on one patient for one day. Electrophysiologists were initially blinded to observations.

Two electrophysiologists were observed over 215 days encompassing 6413 patient encounters. Electrophysiologists made comments on 581(9.1%) encounters in 234 unique patients with diverse diagnoses, equating to a median of 3[interquartile range:1–4] encounters per day. These included identifications of arrhythmias and describing electrocardiographic findings. Recommendation to change management occurred in 282(48.5%) encounters, most commonly regarding medications (n = 142, 24.4%) or pacemaker management (n = 48, 8.3%). Of the 581 encounters, there were 61(10.5%) in which they corrected another physician’s interpretation of rhythm or electrocardiogram.

Routine electrophysiologist involvement in multidisciplinary morning report provides significant, frequent, and timely input in patient management by identifying precise rhythm-related diagnoses and allowing nuanced, patient-specific medication and pacemaker management of all cardiac patients, not just those consulted. Electrophysiologist presence at multidisciplinary morning report is a vital resource and this practice should be considered at integrated paediatric cardiac centres.

Cardiac catheterisation is crucial for diagnosing and treating paediatric heart diseases, but it is poorly tolerated by small children, infants, and newborns without sedation. This study investigated whether maternal voice during sedation could lower stress and pain in children undergoing cardiac catheterisation and also assessed mothers’ stress levels before and after the procedure.

This was a prospective, monocentric, randomised, controlled interventional study at the University Hospital Bonn. Children aged 4 years or younger scheduled for elective cardiac catheterisation under procedural sedation and American Society of Anaesthesiologists class between 1 and 3 were eligible.

At the end of cardiac catheterisation, the intervention group showed a higher Newborn Infant Parasympathetic Evaluation index with an adjusted mean difference of 9.5 (± 4.2) (p = 0.026) and a lower median Children’s and Infants Postoperative Pain Scale score of 2.0 (IQR: 0.0–5.0) versus 4.5 (IQR: 3.0–6.0) than the control group (p = 0.027). No difference in the children’s cortisol level was found (p = 0.424). The mothers in the intervention group had a lower cortisol level than those in the control group before cardiac catheterisation (adjusted mean difference: −4.5 nmol/l (± 1.8 nmol/l), p = 0.011).

Listening to the maternal voice during cardiac catheterisation could lead to less postoperative pain and significantly lower stress and discomfort level in children. Less pain could reduce the incidence of postoperative delirium.

Additionally, mothers perceived involvement as positive. A reduced stress level of mothers can positively influence children and possibly reduce pain and anxiety.

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.

Malignant vasovagal syncope in children seriously affects their physical and mental health. Our study aimed to explore the efficacy of catheter ablation in ganglionated plexus with malignant vasovagal syncope children.

Catheter ablation of ganglionated plexus was safe and effective in children with malignant vasovagal syncope and can be used as a treatment option for these children.

A total of 20 children diagnosed with malignant vasovagal syncope were enrolled in Beijing Children’s Hospital, affiliated with Capital Medical University. All underwent catheter ablation treatment of ganglionated plexus. Ganglionated plexuses of the left atrium were identified by high-frequency stimulation and/or anatomic landmarks being targeted by radiofrequency catheter ablation. The efficacy of the treatment was evaluated by comparing the remission rate of post-operative syncopal symptoms and the rate of negative head-up tilt results. Safety and adverse events were evaluated.

After follow-up for 2.5 (0.6–5) years, the syncope symptom scores were decreased significantly compared with before treatment [3 (2–4) versus 5 (3–8) scores, P < 0.01]. Eighty-five per cent (17/20) children no longer experienced syncope, whilst 80% (16/20) children showed negative head-up tilt test after treatment. No adverse effects such as cardiac arrhythmia occurred in the children.

Transcatheter patent ductus arteriosus closure in premature infants is gaining attention with rapidly growing experience. The KA micro plug device poses many favourable features for ductus arteriosus closure in premature infants. The study aimed to describe the technique and outcomes of a KA micro plug in closing the ductus arteriosus in premature infants weighing less than 1500 g.

This is a single-centre retrospective study in premature infants with patent ductus arteriosus weighing less than 1500 g who underwent off-label use of a KA micro plug at the Stead Family Children’s Hospital from February 2022 to December 2023.

Eighteen premature infants weighing less than 1500 g underwent attempted transcatheter ductus arteriosus closure using a KA micro plug device. Median weight, age, and corrected gestational age at the procedure were 943 g (682–1225), 26 days (9–79), and 28.5 weeks (25.6–32), respectively. The procedure was successful with complete closure achieved in all patients. No major haemodynamic instabilities were encountered. No catheterization-related complications were encountered. On a median follow-up of 10.9 months (0.1–19), all patients are alive with the device in a good position without residual shunt or abnormal findings.

KA micro plug device for patent ductus arteriosus in infants weighing less than 1500 g is feasible, safe, and effective. We recommend that manufacturers of devices used to close the ductus arteriosus in small infants enhance both the devices and their delivery systems to simplify the procedure and mitigate the risk of haemodynamic instability.

Modified Blalock-Taussig shunt (BT shunt) is a palliative operation used for cyanotic heart diseases with decreased pulmonary blood supply. The definitive management of tetralogy of Fallot (TOF) is total corrective surgery, but these patients can be palliated with BT shunt. In the modern world, the BT shunt is getting out of favour in patients with TOF. In this article, we will share our 5-year experience at our institute, which also shows a decreasing trend.

It is a retrospective study. Files of all the patients admitted in our department from January 2019 to December 2023 were reviewed. Age, weight, hospital stay, inotropic support duration, mechanical ventilation duration, and outcomes were studied.

From January 2019 to December 2023, 173 patients underwent BT shunt for TOF. The mean age was 31 months, and the mean weight was 9.3 kg. The overall mortality for BT shunt was 15% after BT shunt. Hypercyanotic spell not controlled by medical management was the most common indication for BT shunt in our setup. Most of the patients with hypercyanotic spells were also candidates for total correction but due to the emergency, BT shunt was performed.

The role of BT shunt in patients with TOF is decreasing due to PDA/RVOT stenting, it is likely that the BT shunt in TOF will become a thing of the past in the future even in developing countries like ours.

Pulmonary regurgitation is the most common complication in repaired tetralogy of Fallot patients. Severe chronic pulmonary regurgitation can be tolerated for decades, but if not treated, it can progress to symptomatic, irreversible right ventricular dilatation and dysfunction. We investigated clinical associations with pulmonary valve replacement among patients with significative pulmonary regurgitation and how interventional developments can change their management.

All adult patients with repaired tetralogy of Fallot who were followed at an adult CHD Clinic at a single centre from 1980 to 2022 were included on their first outpatient visit. Follow-up was estimated from the time of correction surgery until one of the following events occurred first: pulmonary valve replacement, death, loss to follow-up or conclusion of the study.

We included 221 patients (116 males) with a median age of 19 (18–25). At a median age of 33 (10) years old, 114 (51%) patients presented significant pulmonary regurgitation. Among patients with significant pulmonary regurgitation, pulmonary valve replacement was associated with male gender, older age at surgical repair, and longer QRS duration in adulthood. Pulmonary valve replacement was performed in 50 patients, including four transcatheter pulmonary valve implantations, at a median age of 34 (14) years.

Pulmonary regurgitation affects a large percentage of tetralogy of Fallot adult patients, requiring a long-term clinical and imaging follow-up. Sex, age at surgical repair and longer QRS are associated with the need of PVR among patients with significative pulmonary regurgitation. Clinical practice and current literature support TPVI as the future gold standard intervention.

Children with left aortic arch and aberrant right subclavian artery may present with either respiratory or swallowing symptoms beyond the classically described solid-food dysphagia. We describe the clinical features and outcomes of children undergoing surgical repair of an aberrant right subclavian artery.

This was a retrospective review of children undergoing repair of an aberrant right subclavian artery between 2017 and 2022. Primary outcome was symptom improvement. Pre- and post-operative questionnaires were used to assess dysphagia (PEDI-EAT-10) and respiratory symptoms (PEDI-TBM-7). Paired t-test and Fisher’s exact test were used to analyse symptom resolution. Secondary outcomes included perioperative outcomes, complications, and length of stay.

Twenty children, median age 2 years (IQR 1–11), were included. All presented with swallowing symptoms, and 14 (70%) also experienced respiratory symptoms. Statistically significant improvements in symptoms were reported for both respiratory and swallowing symptoms. Paired (pre- and post-op) PEDI-EAT-10 and PEDI-TBM-7 scores were obtained for nine patients, resulting in mean (± SD) scores decreasing (improvement in symptoms) from 19.9 (± 9.3) to 2.4 (± 2.5) p = 0.001, and 8.7 (± 4.7) to 2.8 (± 4.0) p = 0.006, respectively. Reoperation was required in one patient due to persistent dysphagia from an oesophageal stricture. Other complications included lymphatic drainage (n = 4) and transient left vocal cord hypomobility (n = 1).

Children with a left aortic arch with aberrant right subclavian artery can present with oesophageal and respiratory symptoms beyond solid food dysphagia. A thorough multidisciplinary evaluation is imperative to identify patients who can benefit from surgical repair, which appears to be safe and effective.