The aim of this study is to evaluate clinical and surgical outcomes of children with subaortic stenosis, to determine the risk factors for surgery and reoperation and to compare isolated subaortic stenosis and those concomitant with CHDs.

The study involved 80 children with subaortic stenosis. The patients were first classified as isolated and CHD group, and the isolated group was further classified as membranous/fibromuscular group. The initial, pre-operative, post-operative and the most recent echocardiographic data, demographic properties and follow-up results of the groups were analysed and compared. The correlation of echocardiographic parameters with surgery and reoperation was evaluated.

There was a significant male predominance in all groups. The frequency of the membranous type was higher than the fibromuscular type in the whole and the CHD group. The median time to the first operation was 4.6 years. Thirty-five (43.7%) patients underwent surgery, 5 of 35 (14%) patients required reoperation. The rate of surgery was similar between groups, but reoperation was significantly higher in the isolated group. The gradient was the most important factor for surgery and reoperation in both groups. In the isolated group besides gradient, mitral-aortic separation was the only echocardiographic parameter correlated with surgery and reoperation.

Reoperation is higher in isolated subaortic stenosis but similar in membranous and fibromuscular types. Early surgery may be beneficial in preventing aortic insufficiency but does not affect the rate of reoperation. Higher initial gradients are associated with adverse outcomes, recurrence and reoperation.

Caring for infants after the first-stage palliative surgery for single-ventricle heart disease bring challenges beyond the usual parenting responsibilities. Current studies fail to capture the nuances of caregivers’ experiences during the most critical “interstage” period between the first and second surgery.

To explore the perceptions of caregivers about their experiences while transitioning to caregiver roles, including the successes and challenges associated with caregiving during the interstage period.

Constructivist Grounded Theory methodology guided the collection and analysis of data from in person or telephonic interviews with caregivers after their infants underwent the first-stage palliative surgery for single-ventricle heart disease, and were sent to home for 2–4 months before returning for their second surgery. Symbolic interactionism informed data analyses and interpretation.

Our sample included 14 parents, who were interviewed 1–2 times between November, 2019 and July, 2020. Most patients were mothers (71%), Latinx (64%), with household incomes <$30K (42%). Data analysis led to the development of a Grounded Theory called Developing a Sense of Self-Reliance with three categories: (1) Owning caregiving responsibilities despite grave fears, (2) Figuring out how “to make it work” in the interstage period, and (3) Gaining a sense of self-reliance.

Parents transitioned to caregiver roles by developing a sense of self-reliance and, in the process, gained self-confidence and decision-making skills. Our study responded to the key research priority from the AHA Scientific Statement to address the knowledge gap in home monitoring for interstage infants through qualitative research design.

Coronavirus disease 2019 (COVID-19) is responsible for significant lung disease in adults. Despite mild manifestations in most children, multisystem inflammatory syndrome (MIS-C) associated with COVID-19 is well described in older children with cardiac manifestations. However, MIS-C-related cardiac manifestations are not as well described in younger children.

The study is a retrospective analysis of MIS-C patients under the age of 5 years admitted between May and November 2020 to a single centre. Included cases fulfilled the case definition of MIS-C according to Royal College of Pediatrics and Child Health criteria with laboratory, electrocardiogram, or echocardiographic evidence of cardiac disease. Collected data included patients’ demographics, laboratory results, echocardiographic findings, management, and outcomes.

Out of 16 MIS-C cases under 5 years of age, 10 (62.5%) had cardiac manifestations with a median age of 12 months, 9 (90%) were previously healthy. Cardiac manifestations included coronary arterial aneurysms or ectasia in five (50%) cases, two (20%) with isolated myopericarditis, coronary ectasia with myocarditis in two (20%), and supraventricular tachycardia in one (10%). Intravenous immunoglobulins were given in all cases with coronary involvement or myocarditis. The median duration of hospitalisation was 7 (6–14) days; two (20%) cases with cardiac disease were mechanically ventilated and mortality in MIS-C cases below 5 years was 12.5%. Normalisation of systolic function occurred in half of the affected cases within 1 week and reached 100% by 30 days of follow-up.

MIS-C associated with SARS-CoV-2 has a high possibility of serious associated cardiac manifestations in children under the age of 5 years with mortality and/or long-term morbidities such as coronary aneurysms even in previously healthy children.

We carried out a prospective pilot observational experimental study in peri-operative cardiac infants with feeding intolerance between 2019 and 2021. Feeding tube insertion depends on a combination of ultrasound and gastric insufflation with air-saline mixture. Insertion was confirmed by bedside abdominal X-ray.

Out of 500 peri-operative cardiac infants, 15 needed post-pyloric feeding tube insertion in median 15 postoperative day. All were under 6 months of age with average weight of 3 ± 0.2 kg. Median Risk Adjustment for Congenital Heart Surgery Categories was 4. Median insertion time was 15 minutes. No complications have been reported. First pass success rate was 87%, while a second successful insertion attempt was needed in 2 cases (13%). Target daily calorie intake was achieved within average of 3.5 ± 0.4 days. Mean post-pyloric feeding tube stay was 20 ± 3 days. Out of 15 infants, 3 patients died, 1 patient needed gastrostomy tube, and 11 patients were discharged home on oral feeds.

Ultrasound-guided post-pyloric feeding tube insertion using gastric insufflation with air-saline mixture in peri-operative cardiac infants with feeding intolerance is a useful and practical bedside tool, and it can be performed by a trained paediatric cardiac ICU intensivist. It may have potential positive effects on morbidity and outcome.

Laterality anomalies are almost always associated with severe cardiac anomalies. Demographic properties, type of the procedures, associated anomalies, and early and mid-term prognosis of four types of laterality anomalies were analysed.

A total of 64 consecutive patients with laterality anomalies were enrolled between July 2014 and July 2020. We grouped the patients as situs solitus dextrocardia (SSD) (n = 12; 18.7%); situs inversus (SI) (n = 16; 25%); right atrial isomerism (RAI) (n = 29; 45.3%); and left atrial isomerism (LAI) (n = 7; 10.9%). TAPVC was only present in the RAI group (31%). Incidence of mitral or tricuspid atresia was higher in the SSD group (25%). All the patients were followed up with a mean of 19.06 ± 17.6 (0.1–72) months.

Early postoperative mortality was 17 patients, among 107 procedures (15.8%). Twelve patients were in the neonatal period. All ten patients survived after isolated ductal stenting. Fourteen of the deaths were in the RAI group (48.3%). The 3-year survival rates were 85% in LAI, 78.7% in SI, 55.8% in SSD, and 38% in RAI groups. According to the multivariable Cox regression model, mechanical ventilation, kidney injury, RAI, and complex surgery in the neonatal period were independent risk factors for early mortality.

Laterality anomalies are one of the most challenging patients who commonly had univentricular physiology. The most prevalent anomaly was RAI, and RAI had the worst outcome and survival. Ductal stent is an acceptable first intervention during the neonatal period in suitable patients. Complex procedures may carry a high risk of death in the neonatal period.

This retrospective case series study sought to describe the safety and clinical effectiveness of propafenone for the control of arrhythmias in children with and without CHD or cardiomyopathy.

We reviewed baseline characteristics and subsequent outcomes in a group of 63 children treated with propafenone at 2 sites over a 15-year period Therapy was considered effective if no clinically apparent breakthrough episodes of arrhythmias were noted on the medication.

Sixty-three patients (29 males) were initiated on propafenone at a median age of 2.3 years. CHD or cardiomyopathy was noted in 21/63 (33%). There were no significant differences between demographics, clinical backgrounds, antiarrhythmic details, side effect profiles, and outcomes between children with normal hearts and children with CHD or cardiomyopathy. Cardiac depression at the initiation of propafenone was more common amongst children with CHD or cardiomyopathy compared to children with normal hearts. Systemic ventricular function was diminished in 15/63 patients (24%) prior to starting propafenone and improved in 8/15 (53%) of patients once better rhythm control was achieved. Other than one child in whom medication was stopped due to gastroesophageal reflux, no other child experienced significant systemic or cardiac side effects during treatment with propafenone. Propafenone achieved nearly equal success in controlling arrhythmias in both children with normal hearts and children with congenital heart disease or cardiomyopathy (90% versus 86%, p = 0.88).

Propafenone is a safe and effective antiarrhythmic medication in children.

This study aimed to investigate whether the third trimester fetal cardiac diastolic function measured by selected conventional Doppler indices is affected in appropriate-for-gestational-age or macrosomic fetuses of gestational diabetic mothers with poor glycaemic control.

This cross-sectional study included 93 pregnant women divided into two groups. Group 1 included 45 appropriate-for-gestational-age or macrosomic fetuses from gestational diabetic mothers with poor glycaemic control (study group). Group 2 included 48 appropriate-for-gestational-age fetuses from gestational age-matched healthy mothers (control group). Functional fetal cardiac parameters and fetoplacental Doppler parameters were measured. Data were compared between the two groups.

Maternal characteristics did not differ significantly between the study and the control group. There were no significant differences in the early and late velocity, early/late velocity ratio of both mitral and tricuspid valves, the fetal pulmonary vein pulsatility index, and the ductus venosus pulsatility index between the study and the control group. Moreover, the rate of abnormal Doppler findings in pulmonary vein (pulmonary vein pulsatility index >95th centile), ductus venosus (ductus venosus pulsatility index >95th centile), and peripheral vessels (umbilical artery pulsatility index >95th centile, middle cerebral artery pulsatility index <5th centile, cerebra-placental index >95th centile) were comparable in both groups.

The third trimester fetal diastolic functions measured by selected conventional Doppler techniques do not seem to be altered in appropriate-for-gestational-age or macrosomic fetuses of gestational diabetic mothers who have poor glycaemic control.

We aimed to evaluate the efficacy and role of high-sensitivity troponin T in children with a confirmed SARS-CoV-2 infection and also the correlation of troponin T levels with symptoms, and echocardiographic findings were analysed.

Two hundred and fourteen patients with a confirmed SARS-CoV-2 infection between the dates of 28 March and 15 August 2020 were enrolled in this retrospective single-centre study. Patients with comorbidities and diagnosed as multisystem inflammatory syndrome in children were excluded. Demographic data, clinical and laboratory parameters were evaluated. The patients were classified and compared according to the troponin positivity. The correlation of troponin T with symptoms and echocardiographic findings was analysed.

The most common symptoms in the whole study group were fever (53.3%) and cough (24.8%). Troponin T levels were elevated in 15 (7%) patients. The most common symptom in patients with troponin positivity was also fever (73.3%). Troponin T positivity was significantly higher in patients under the age of 12 months and troponin T levels were negatively correlated with age. C-reactive protein levels were elevated in 77 (36%) of the patients in the whole group and 7 (46.7%) of 15 patients with troponin positivity. C-reactive protein levels were similar between groups.

Routine troponin screening does not yield much information in previously healthy paediatric COVID-19 patients without any sign of myocardial dysfunction. Elevated troponin levels may be observed but it is mostly a sign of myocardial injury without detectable myocardial dysfunction in this group of patients.

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.

The aim of this study was to describe the development and assess the usefulness of a feeding clinic to help infants with CHD tolerate the highest level of oral feeding while achieving growth velocity and supporting neurodevelopment.

This retrospective, cohort study assessed feeding outcomes for infants who underwent cardiac surgery at <30 days of age with cardiopulmonary bypass between February 2016 and April 2020. Diagnoses, age at surgery, hospitalisation variables, and feeding outcomes were compared between two cohorts, pre- and post-implementation of a specialised feeding clinic using Exact Wilcoxon signed-rank test, chi-squared, or Fisher’s exact test. The association between time to full oral feed and risk factors was assessed using univariable and multivariable Cox regression model.

Post-clinic infants (n = 116) surgery was performed at a median of 6 days of life (interquartile range: 4, 8) with median hospital length of stay of 19 days (interquartile range: 16, 26). Infants’ median age at first clinic visit was at 30 days old (interquartile range: 24, 40) and took median 10 days (interquartile range: 7, 12) after hospital discharge to first clinic visit. In the post-clinic cohort, the median time to 100% oral feeding was 47 days (interquartile range: 27, 96) compared to the 60 days (interquartile range: 20, 84) in the pre-clinic cohort (n = 22), but the difference was not statistically significant.

The cardiac feeding clinic was utilised by our neonatal surgery population and feasible in coordination with cardiology follow-up visits. Future assessment of cardiac feeding clinic impact should include additional measures of feeding and neurodevelopmental success.