Single ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care.

We reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician’s approach to single ventricle patients at different stages of surgical palliation.

Single ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern.

Management of “failing” and “failed” Fontan circulation, particularly the indications, timing, and type of re-intervention, currently remains nebulous. Factors contributing to pathogenesis and mortality following Fontan procedure differ between children and adults.

Since organ systems in individual patients are affected differently, we searched the extant literature for a “failing” and “failed” Fontan reviewing the clinical phenotypes, diagnostic modalities, pharmacological, non-pharmacological, and surgical techniques employed, and their outcomes.

A total of 410 investigations were synthesised. Although proper candidate selection, thoughtful technical modifications, timely deployment of mechanical support devices, tissue-engineered conduits, and Fontan takedown have decreased the peri-operative mortality from 9 to 15% and 1 to 3% per cent in recent series, pernicious changes in organ function are causing long-term patient attrition. In the setting of a failed Fontan circulation, literature documents three surgical options: Fontan revision, Fontan conversion, or cardiac transplantation. The reported morbidity of 25% and mortality of 8–10% among Fontan conversion continue to improve in select institutions. While operative mortality following cardiac transplantation for Fontan failure is 30% higher than for other CHDs, there is no difference in long-term survival with actuarial 10-year survival of around 54%. Mechanical circulatory assistance, stem cells, and tissue-engineered Fontan conduit for destination therapy or as a bridge to transplantation are in infancy for failing Fontan circulation.

An individualised management strategy according to clinical phenotypes may delay the organ damage in patients with a failing Fontan circulation. At present, cardiac transplantation remains the last stage of palliation with gradually improving outcomes.

Infective endocarditis is a major threat after prosthetic pulmonary valve replacement. Early diagnosis may improve outcomes.

A structured patient education programme for prevention and early diagnosis of infective endocarditis was developed at our institution since 2016. Time delay between onset of symptoms of prosthetic pulmonary valve endocarditis and its diagnosis (defined as initiation of appropriate high-dose intravenous antibiotic treatment) was compared for patients presenting before (cohort 1) and after (cohort 2) initiation of the patient education programme.

Between 2008–2019, 26 patients (median age 24.9, range: 16.8–62.0 years, 73% male) were diagnosed with prosthetic pulmonary valve endocarditis, 13 patients (cohort 1) before (1.7 cases/year) and 13 patients (cohort 2) after June 2016 (3.7 cases/year). There were no differences in baseline characteristics or clinical presentation between the study cohorts. Overall, the median delay between onset of symptoms and diagnosis of infective endocarditis was 6 days (range: 0–133 days) with a significantly longer delay among patients in cohort 1, compared to cohort 2 (25 days, range: 5–133 days versus 3 days, range: 0–13 days, p < 0.0001). A delay of >7 days was documented in 11/13 patients (85%) in cohort 1 as compared to 1/13 (8%) in cohort 2 (p < 0.001). Need for urgent valve replacement or permanent deterioration of prosthetic valve function was higher in cohort 1, compared to cohort 2 (11/13, 85% versus 5/13, 39%; p = 0.041).

Prosthetic pulmonary valve endocarditis is increasingly recognised. A structured patient education programme may improve early diagnosis and clinical outcomes.

Yasui procedure is surgical repair intended to preserve biventricular function for patients with left ventricle outflow tract obstruction associated with aortic arch lesions and ventricular septal defect.

Retrospective chart review analysis of all patients who had Yasui procedure (2008–2020) comparing midterm outcome of one versus two stage repair.

Twenty patients (70% female) underwent Yasui procedure in our center. Eight patients (40%) had left ventricle outflow tract obstruction /interrupted aortic arch, 7 patients (35%) had left ventricle outflow tract obstruction /coarctation of aorta, 3 patients (15%) had double outlet ventricle and ventricular septal defect that were unattainable for tunneling to one of the semilunar valves without creating obstruction, and 2 patients (10%) had aortic atresia with hypoplastic aortic arch. All patients had associated ventricular septal defect. Fifteen patients (75%) had one-stage repair and 5 patients (25%) had two-stage repair. Means age and weight for one and two-stage repair were 1.3 ± 2 months, 13.4 ± 11.5 months and 3.3 ± 0.6 kg, 7.8 ± 3.4 kg, respectively. During follow up, 8 patients (40%) required re- intervention, mainly for right ventricle–pulmonary artery conduit either dilation or replacement. The average duration of follow up was 5 years with nil mortality.

Yasui procedure is effective approach for children who have left ventricle outflow tract obstruction associated with aortic arch anomalies and ventricular septal defect. Survival rate with single or staged repair is comparably good. During the first 5 year of follow up, nearly 40% of operated patients required re-intervention.

This study aimed to explore the effects of eye masks on the sleep quality and pain of school-age children with CHD after surgery.

Forty school-age children with CHD who underwent open-heart surgery in the Cardiac Surgery Department of a provincial hospital in China from January 2020 to December 2020 were selected. The children were randomly divided into the experimental group (n = 20) and the control group (n = 20). Children in the control group were given routine sleep care, and the children in the experimental group were given a sleep intervention with eye masks for three nights following the removal of tracheal intubation. The Pittsburgh Sleep Quality Index was used to evaluate the sleep quality of the children. The Children’s Pain Behaviour Scale was used to evaluate the pain of the children.

After three nights of receiving the eye masks intervention, the sleep quality scores of the children in the experimental group were significantly lower than those of the control group, the difference was statistically significant (p < 0.05) and the sleep quality of the children in the experimental group was higher. The pain scores of the children in the experimental group were significantly lower than those of the children in the control group, the difference was statistically significant (p < 0.05), and the children in the experimental group suffered less post-operative pain.

Eye masks are a simple, safe and economical intervention, that is beneficial for improving the post-operative sleep quality and relieving post-operative pain in school-age children with congenital heart disease.

Patients with CHD can be exposed to high levels of cumulative ionising radiation. Utilisation of electroanatomic mapping during catheter ablation leads to reduced radiation exposure in the general population but has not been well studied in patients with CHD. This study evaluated the radiation sparing benefit of using three-dimensional mapping in patients with CHD.

Data were retrospectively collected from the Catheter Ablation with Reduction or Elimination of Fluoroscopy multi-institutional registry. Patients with CHD were selected. Those with previous ablations, concurrent diagnostic or interventional catheterisation and unknown arrhythmogenic foci were excluded. The control cohort was matched for operating physician, arrhythmia mechanism, arrhythmia location, weight and age. The procedure time, rate of fluoroscopy use, fluoroscopy time, procedural success, complications, and distribution of procedures per year were compared between the two groups.

Fifty-six patients with congenital heart disease and 56 matched patients without CHD were included. The mean total procedure time was significantly higher in patients with CHD (212.6 versus 169.5 minutes, p = 0.003). Their median total fluoroscopy time was 4.4 minutes (compared to 1.8 minutes), and their rate of fluoroscopy use was 23% (compared to 13%). The acute success and minor complication rates were similar and no major complications occurred.

With the use of electroanatomic mapping during catheter ablation, fluoroscopy use can be reduced in patients with CHD. The majority of patients with CHD received zero fluoroscopy.

We describe a cohort of children referred with multisystem inflammatory syndrome in children associated with severe acute respiratory syndrome coronavirus 2 and compare this cohort with a 2019 cohort of children with Kawasaki disease.

We conducted a retrospective cohort study of 2019 and 2020 referrals to the inflammatory cardiology service at Great Ormond Street Hospital for Children. We compared cardiac and inflammatory parameters of a sub-section of the 2020 cohort who presented with reduced left ventricular ejection fraction with the remainder of the cohort.

Referrals significantly increased between February and June 2020 compared to 2019 (19.8/30 days versus 3.9/30 days). Frequency of coronary artery aneurysms (11/79 (13.9%) versus 7/47 (14.9%)) or severe coronary artery aneurysms (6/79 (7.6%) versus 3/47 (6.4%)) was similar between 2020 and 2019, respectively. The 2020 cohort was older (median age 9.07 years versus 2.38 years), more likely to be of Black, Asian, or other minority ethnic group (60/76 (78.9%) versus 25/42 (59.5%)), and more likely to require inotropic support (22 (27.5%) versus 0 (0%)). Even children with significantly reduced left ventricular ejection fraction demonstrated complete recovery of cardiac function within 10 days (mean 5.25 days ± 2.7).

We observed complete recovery of myocardial dysfunction and an overall low rate of permanent coronary sequelae, indicating that the majority of children with multisystem inflammatory syndrome in children are unlikely to encounter long-term cardiac morbidity. Although the frequency of myocardial dysfunction and inotropic support requirement is not consistent with a diagnosis of Kawasaki disease, the frequency of coronary artery abnormalities and severe coronary artery abnormalities suggests a degree of phenotypic overlap.

Advances in surgical techniques and post-operative management of children with CHD have significantly lowered mortality rates. Unplanned cardiac interventions are a significant complication with implications on morbidity and mortality.

We conducted a single-centre retrospective case–control study for patients (<18 years) undergoing cardiac surgery for repair of Tetralogy of Fallot between January 2009 and December 2019. Data included patient characteristics, operative variables and outcomes. This study aimed to assess the incidence and risk factors for reintervention of Tetralogy of Fallot after cardiac surgery. The secondary outcome was to examine the incidence of long-term morbidity and mortality in those who underwent unplanned reinterventions.

During the study period 29 patients (6.8%) underwent unplanned reintervention, and were matched to 58 patients by age, weight and sex. Median age was 146 days, and median weight was 5.8 kg. Operative mortality was 7%, and 1-year survival was 86% for the entire cohort (cases and controls). Hispanic patients were more likely to have reinterventions (p = 0.04) in the unadjusted analysis, while Asian, Pacific Islander and Native American (p = 0.01) in the multi-variate analysis. Patients that underwent reintervention were more likely to have post-op arrhythmia, genetic syndromes and higher operative and 1-year mortality (p < 0.05).

Unplanned cardiac interventions following Tetralogy of Fallot repair are common, and associated with increased operative, and 1-year mortality. Race, genetic syndromes and post-operative arrhythmia are associated with increased odds of unplanned reinterventions. Future studies are needed to identify modifiable risk factors to minimise unplanned reinterventions.

Cardiopulmonary exercise testing represents the diagnostic tool for determining cardiopulmonary function. Especially in small children, exercise testing is extremely challenging. To address this problem, field testing has been implemented using small mobile devices. This study aims at using this protocol for developing normal values for cardiopulmonary exercise testing in very young children.

Healthy children aged 4–8 years were recruited. All children were tested according to an outdoor protocol, in which they were instructed to walk, then run slowly, then a little harder and at last run at full speed. Each step lasted for 2 minutes, except the last step, in which the children were instructed to maintain as long as possible.

A total of 104 children (64 female/35 male, mean age 6.6 years) performed outdoor cardiopulmonary exercise testing using a mobile device. Almost all tests were completed successfully (95%). Despite a predominance of female study subjects, anthropometric values did not differ between boys and girls. V̇O2peak/kg, respiratory exchange ratio, VT1, heart rate at VT1, and time of exercise were also comparable between sexes. Generally, a tendency of higher maximal oxygen uptake could be observed in older children.

Open field mobile cardiopulmonary exercise testing represents a novel approach in very young children. In this study, we were able to determine normal values of maximal oxygen uptake and OUES/kg for 4–8-year-old children. The method is easy to achieve and safe.

To describe the association between successful weaning of inhaled nitric oxide and trends in dead space ratio during such weans in patients empirically initiated on nitric oxide therapy out of concern of pulmonary hypertensive crisis.

Children in a cardiac intensive care unit initiated on inhaled nitric oxide out of clinical concern for pulmonary hypertensive crisis retrospectively over 2 years.

Twenty-seven patients were included, and nitric oxide was successfully discontinued in 23/27. These patients exhibited decreases in dead space ratio (0.18 versus 0.11, p = 0.047) during nitric oxide weaning, and with no changes in dead space ratio between pre- and post-nitric oxide initiation (p = 0.88) and discontinuation (p = 0.63) phases. These successful patients had a median age of 10 months [4.0, 57.0] and had a pre-existent diagnosis of CHD in 6/23 and pulmonary hypertension in 2/23. Those who failed nitric oxide discontinuation trended with a higher dead space ratio at presentation (0.24 versus 0.10), were more likely to carry a prior diagnosis of pulmonary hypertension (50% versus 8.7%), and had longer mechanical ventilation days (5 versus 12).

Patients empirically placed on nitric oxide out of concern of pulmonary hypertensive crisis and successfully weaned off showed unchanged or decreased dead space ratio throughout the initiation to discontinuation phases of nitric oxide therapy. Trends in dead space ratio may aid in determining true need for nitric oxide and facilitate effective weaning. Further studies are needed to directly compare trends between success and failure groups.

Mitral stenosis is the most common valvular heart disease during pregnancy. When severe, it leads to significant maternal and fetal morbidity and mortality. Percutaneous mitral valve balloon commissurotomy can be performed during pregnancy, and the present study aimed to describe the immediate maternal and fetal outcomes after percutaneous mitral valve balloon commissurotomy was done in a cohort of 23 pregnant patients with severe mitral stenosis in Addis Ababa, Ethiopia.

Included in the current study were all pregnant mothers who had severe rheumatic mitral valve stenosis and who underwent percutaneous mitral valve balloon commissurotomy at the Cardiac Center of Ethiopia over 6-year period. Data were collected through chart abstraction using a structured proforma and then analysed using STATA version 13.0.

Median gestational age was 22 weeks and percutaneous mitral valve balloon commissurotomy was successful resulting in a significant increase in the mean mitral valve area of the group from 0.78 ± 0.20 cm2 to 1.89 ± 0.31 cm2 (p < 0.001). The mean mitral valve inflow gradient of the group was 23.95 ± 6.27 mmHg and 6.80 ± 2.44 mmHg, respectively, before and after the percutaneous mitral valve balloon commissurotomy procedure (p < 0.001). Post-procedure, there was no significant increment in mitral valve incompetence. The mean pulmonary artery pressure of the group decreased from 77.68 ± 23.19 mmHg to 42.31 ± 9.95 mmHg (p < 0.001). There was no fetal or maternal death following the procedure. Pregnancy ended at term gestation for 19/23 (82.6%) of the mothers and the mean birth weight of the neonates was 2800 g.

Percutaneous mitral valve balloon commissurotomy procedure can safely be done for severe symptomatic rheumatic mitral stenosis in pregnancy in our setting.

Ceraflex septal occluder and the Figulla atrial septal defect occluder have the advantage of a pivoting mechanism and softer device architecture. This study sought to examine the safety and efficacy of these occluders compared to the Amplatzer septal occluder.

This was a retrospective study. Between January, 2013 and April, 2020, patients with at least 6 months of follow-up were included. Early and late-onset outcomes were examined.

Four hundred seven patients (range: 0.17–70.72 years; 53.1% >18 years; male: 29.2%) underwent atrial septal defect occlusion using Amplatzer septal occluder (n = 313), Ceraflex septal occluder (n = 36) and FSO (n = 58). A longer procedure time was observed in the Amplatzer septal occluder group. Early-onset complication rates in Amplatzer septal occluder, Ceraflex septal occluder and Figulla atrial septal defect occluder were 3.83%, 5.56% and 0%. Ten (2.46%) patients developed delayed complications (2.56%, 0% and 1.72% in the Amplatzer septal occluder, Ceraflex septal occluder and Figulla atrial septal defect occluder groups). Device erosion rate was not different between groups. The occlusion rates were comparable among all the devices.

There is no significant difference in safety and efficacies between the novel atrial septal defect occluding devices compared to Amplatzer septal occluder.

To assess current demographics and duties of physicians as well as the structure of paediatric cardiac critical care in the United States.

REDCap surveys were sent by email from May till August 2019 to medical directors (“directors”) of critical care units at the 120 United States centres submitting data to the Society of Thoracic Surgeons Congenital Heart Surgery Database and to associated faculty from centres that provided email lists. Faculty and directors were asked about personal attributes and clinical duties. Directors were additionally asked about unit structure.

Responses were received from 66% (79/120) of directors and 62% (294/477) of contacted faculty. Seventy-six percent of directors and 54% of faculty were male, however, faculty <40 years old were predominantly women. The majority of both groups were white. Median bed count (n = 20) was similar in ICUs and multi-disciplinary paediatric ICUs. The median service expectation for one clinical full-time equivalent was 14 weeks of clinical service (interquartile range 12, 16), with the majority of programmes (86%) providing in-house attending night coverage. Work hours were high during service and non-service weeks with both directors (37%) and faculty (45%).

Racial and ethnic diversity is markedly deficient in the paediatric cardiac critical care workforce. Although the majority of faculty are male, females make up the majority of the workforce younger than 40 years old. Work hours across all age groups and unit types are high both on- and off-service, with most units providing attending in-house night coverage.

Telemedicine gained an increasing use throughout the last years. Lifestyle tools like the Apple watch seem to have an increasing spread even in remote areas and underdeveloped regions. The increasing availability of these tools offers the chance to use the health care functions of these devices to improve provision of professional medical care. First data on the use of the Apple Watch as a remote monitoring device in children have been reported, showing good acceptability and usability of the Apple Watch for symptom monitoring in children. This study aimed to evaluate the accuracy of the Apple Watch iECG in comparison to a standard 12-lead ECG in pre-term babies.

In this prospective, single-arm study, consecutive preterm neonates hospitalised in Leipzig University Hospital neonatal ICU were eligible. A 12-lead ECG and an iECG using Apple Watch 4 were performed. iECG and 12-lead ECG measurements were performed by a paediatric cardiologist. Cardiac rhythm was classified and amplitudes and timing intervals were analysed for comparability.

Fifty preterm neonates, gestational week (23–36 weeks), and body weight (0.65–3.09 kg) were enrolled. Overall good quality and excellent correlation of the Apple Watch generated iECG in comparison to the standard 12-lead ECG could be demonstrated (p < 0.001). When interpreted by a paediatric cardiologist, a correct rhythm classification could be done in 100% of cases.

The Apple Watch iECG seems to be a valuable tool to record an ECG comparable to lead I of the standard 12-lead ECG even in pre-term neonates. With a widespread availability and excellent connectivity, the Apple Watch iECG function may provide practitioners with a tool to send an iECG for interpretation to a paediatric cardiac specialist.

The effect of prenatal diagnosis on prognosis in patients with transposition of the great arteries is not clear. In this study, we compared the outcomes after arterial switch operation.

Outcome of 112 patients who had arterial switch operation in the neonatal period were analysed. The patients were divided into two groups: those who had prenatal diagnosis (Group 1; n = 34) and those who did not (Group 2; n = 78). The patients were also classified based on their diagnosis: simple transposition, transposition with ventricular septal defect and/or aortic arch hypoplasia, and Taussig–Bing anomaly.

In Group 1, the C-section delivery rate was higher (82% vs. 44%; p = 0.004), and it was observed that patients in Group 1 were more often intubated upon admission to the neonatal ICU (38% vs. 9%; p = 0.005). No differences were found between the two groups in terms of operation time, cardiopulmonary bypass time, post-operative invasive respiratory support duration, or extracorporeal membrane oxygenation support. It was observed that those who had Taussig–Bing anomaly had a higher mortality.

Timely treatment have a positive effect on neonatal mortality and morbidity. That’s why all families with prenatal diagnosis of critical CHD should be recommended to have the delivery in a tertiary care hospital. Although it could not be demonstrated in this study, prenatal diagnosis has a potential to improve surgical results especially in countries or cities, which does not have enough resources for transfer and surgical units. Further efforts are needed to improve prenatal screening programmes.