Infection with Sars-CoV-2 is known to cause cardiac injury and coronary artery changes in moderate to severe acute COVID-19 and post-acute multisystem inflammatory syndrome in children (MIS-C). However, little is known about the potential for cardiac involvement, in particular coronary artery dilation, in asymptomatic or mild cases of COVID-19.

A retrospective review of children ≤ 18 years of age with a history of asymptomatic or mild COVID-19 disease who underwent echocardiography after Sars-CoV-2 infection is conducted. Patients were excluded if they had been hospitalised for COVID-19/MIS-C or had a history of cardiac disease that could affect coronary artery dimension. Coronary artery dilation was defined as the Boston Z-score greater than 2.0.

One hundred and fifty-seven patients met inclusion criteria with a mean age of 9.4 years (+/– 5.4 years). Eighty-four (54%) patients were identified as having COVID-19 through positive antibody testing. All patients underwent electrocardiogram and echocardiogram as part of their cardiology evaluation. One hundred and thirty-five (86%) patients had a normal evaluation or only a minor variant on electrocardiogram, while 22 patients had abnormalities on echocardiogram, 4 of which demonstrated coronary artery dilation based on the Boston Z-score.

Much of the literature for post-infectious screening and follow-up focuses on patients with a history of moderate to severe COVID-19 disease, emphasising the need for surveillance for the potential development of myocarditis. In this study, 4 out of 157 (2.5%) children with a history of asymptomatic or mild COVID-19 disease without MIS-C were found to have some degree of coronary artery dilation. The significance of this finding currently remains unknown.

Left ventricular diastolic dysfunction is a recognised sequela following transplantation in paediatric heart transplant patients. Traditional echocardiographic indices do not correlate well with left ventricular filling pressure immediately after transplantation. This study aimed to assess whether these indices have any long-term correlation after transplantation in paediatric patients.

A retrospective chart review of 41 patients who had a heart transplant before the age of 24 years was performed. The median time since the transplantation was 11 years. Data obtained from surveillance cardiac catheterisation and echocardiographic examination were reviewed. Traditional echocardiographic indices of diastolic function were compared with the pulmonary capillary wedge pressure and left ventricular end-diastolic pressure obtained from cardiac catheterisation.

The median age at transplant was 12.1 years, and the median time since transplant was 11 years. Eighteen patients (43%) had a history of at least one rejection episode and 12 patients (29%) had a history of cardiac allograft vasculopathy. There was no correlation between mitral inflow E velocity, mitral E/A ratio, tissue Doppler velocities, mitral E/e’ (mitral inflow E velocity to mitral annular velocity), and elevated pulmonary capillary wedge pressure or elevated left ventricular end-diastolic pressure. There was no correlation between mitral valve deceleration time or isovolumetric relaxation time with elevated pulmonary capillary wedge pressure or elevated left ventricular end-diastolic pressure.

Our findings suggest that traditional echocardiographic indices of diastolic function do not correlate well with elevated invasive pulmonary capillary wedge pressure or elevated left ventricular end-diastolic pressure in paediatric heart transplant patients’ long-term post-transplantation.

Persistent pulmonary hypertension of the newborn is a life-threatening condition that affects about 1–2 per 1,000 live births worldwide. Bosentan is an oral dual endothelin receptor antagonist that may have a beneficial effect on persistent pulmonary hypertension of the newborn by reducing pulmonary vascular resistance and improving oxygenation. However, its role in persistent pulmonary hypertension of the newborn remains unclear.

To systematically evaluate the efficacy and safety of bosentan as an adjuvant therapy for persistent pulmonary hypertension of the newborn in newborns.

We searched six English and two Chinese databases from their inception to 1 January 2023 following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We included randomised controlled trials and retrospective studies that compared bosentan with placebo or other drugs for persistent pulmonary hypertension of the newborn in newborns. We performed a meta-analysis using random-effects models and assessed the risk of bias and heterogeneity in the included studies.

We included 10 studies with a total of 550 participants. Bosentan significantly reduced the treatment failure rate (relative risk = 0.25, P < 0.001), pulmonary artery pressure (mean difference = −11.79, P < 0.001), and length of hospital stay (mean difference = −1.04, P = 0.003), and increased the partial pressure of oxygen (mean difference = 10.02, P < 0.001) and blood oxygen saturation (SpO2) (mean difference = 8.24, P < 0.001) compared with a placebo or other drugs. The occurrence of adverse reactions was not significantly different between bosentan and a placebo or other drugs.

Bosentan is effective in the treatment of persistent pulmonary hypertension of the newborn but adverse reactions such as abnormal liver function should be observed when using it.

Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge.

We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation.

Between 1994 and 2021, 202 patients with univentricular heart and two separate atrioventricular valve morphology underwent surgical palliation, with 15.8% (32/202) requiring atrioventricular valve surgery. Primary diagnoses were double inlet left ventricle (n = 14, 43.8%), double outlet right ventricle (n = 7, 21.9%), and congenitally corrected transposition of the great arteries (n = 7, 21.9%). Median weight at valve surgery was 10.6 kg (interquartile range, 7.9–18.9). Isolated left or right atrioventricular valve surgery was required in nine (28.1%) and 22 patients (68.8%), respectively. Concomitant left and right atrioventricular valve surgery was performed in one patient (3.1%). Closure of the left valve was conducted in four patients (12.5%) and closure of the right valve in three (9.4%). Operative and late mortality were 3.1% and 9.7%, respectively. Reoperation-free survival and cumulative incidence of reoperation at 10 years after surgery were 62.3% (standard error of the mean: 6.9) and 30.9% (standard error of the mean: 9.6), respectively.

In patients with univentricular heart and two separate atrioventricular valves, surgical intervention on these valves is required in a minority of patients and is associated with low mortality but high incidence of reoperation.

Packed red blood cell transfusions occur frequently after congenital heart surgery to augment haemodynamics, with limited understanding of efficacy. The goal of this study was to analyse the hemodynamic response to packed red blood cell transfusions in a single cohort, as “proof-of-concept” utilising high-frequency data capture of real-time telemetry monitoring.

Retrospective review of patients after the arterial switch operation receiving packed red blood cell transfusions from 15 July 2020 to 15 July 2021. Hemodynamic parameters were collected from a high-frequency data capture system (SickbayTM) continuously recording vital signs from bedside monitors and analysed in 5-minute intervals up to 6 hours before, 4 hours during, and 6 hours after packed red blood cell transfusions—up to 57,600 vital signs per packed red blood cell transfusions. Variables related to oxygen balance included blood gas co-oximetry, lactate levels, near-infrared spectroscopy, and ventilator settings. Analgesic, sedative, and vasoactive infusions were also collected.

Six patients, at 8.5[IQR:5-22] days old and weighing 3.1[IQR:2.8-3.2]kg, received transfusions following the arterial switch operation. There were 10 packed red blood cell transfusions administered with a median dose of 10[IQR:10-15]mL/kg over 169[IQR:110-190]min; at median post-operative hour 36[IQR:10-40]. Significant increases in systolic and mean arterial blood pressures by 5-12.5% at 3 hours after packed red blood cell transfusions were observed, while renal near-infrared spectroscopy increased by 6.2% post-transfusion. No significant changes in ventilation, vasoactive support, or laboratory values related to oxygen balance were observed.

Packed red blood cell transfusions given after the arterial switch operation increased arterial blood pressure by 5-12.5% for 3 hours and renal near-infrared spectroscopy by 6.2%. High-frequency data capture systems can be leveraged to provide novel insights into the hemodynamic response to commonly used therapies such as packed red blood cell transfusions after paediatric cardiac surgery.

Pain management for infants undergoing cardiothoracic surgery primarily utilises opioid analgesics. There is a paucity of data available for the use of non-steroidal anti-inflammatory medications such as ketorolac in this patient population.

This retrospective study evaluated patients between 30 days and 6 months undergoing cardiothoracic surgery. The primary endpoint evaluates ketorolac on reducing post-operative opioid use.

Of 243 evaluated patient, 145 met inclusion. Baseline demographics were similar amongst the cohorts. Patients administered ketorolac used less cumulative opiates, in morphine milligram equivalents, for post-op days (POD) 1–3 after surgery compared to patients not receiving ketorolac (9.47 versus 12.68; p = 0.002). The no-ketorolac group required more opiates on POD 1 (10.9 versus 5; p < 0.001) and POD 2 (4.2 versus 2.5; p = 0.006) with no difference found on POD 3 (2 versus 1.6; p = 0.2). There was a mean increase from baseline to highest serum creatinine level on POD 1–3 in the no-ketorolac group compared to the ketorolac group (0.15 versus 0.09 mg/dL; p < 0.014), with no difference in stage 1 or stage 2 acute kidney injury. There were no differences in average chest tube output in mL/kg/day (0.24 versus 0.32; p = 0.569) or need for transfusion (36% versus 24%; p = 0.125), respectively.

Scheduled administration of ketorolac after cardiothoracic surgery resulted in a significant reduction in opioid exposure, with no difference in rates of acute kidney injury or bleeding.

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.

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.

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.

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.

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.

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.

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.