We aim to describe the early and upto 16 months follow-up of post-coronavirus disease (COVID), multi-system inflammatory syndrome in children (MIS-C), with special reference to cardiac involvement.

This cohort non-interventional descriptive study included patients <18 years admitted between May, 2020 and April, 2021. Based on underlying similarities, children were classified as post-COVID MIS-C with overlapping Kawasaki Disease, MIS-C with no overlapping Kawasaki Disease, and MIS-C with shock. Post-discharge, patients were followed at 1, 3, 6, 12, and 16 months.

Forty-one patients predominantly males (73%), at median age of 7 years (range 0.2–16 years) fulfilled the World Health Organisation criteria for MIS-C. Cardiac involvement was seen in 15 (36.5%); impaired left ventricle (LV) function in 5 (12.2%), coronary artery involvement in 10 (24.4%), pericardial effusion in 6 (14.6%) patients, and no arrhythmias. There were two hospital deaths (4.9%), both in MIS-C shock subgroup (2/10, 20%). At 1 month, there was persistent LV dysfunction in 2/5, coronary artery abnormalities in 7/10, and pericardial effusion resolved completely in all patients. By 6 months, LV function returned to normal in all but coronary abnormalities persisted in two patients. At last follow-up (median 9.8 months, interquartile range 2–16 months), in 36/38 (94.7%) patients, coronary artery dilatation was persistent in 2 (20%) patients.

Children with MIS-C have a good early outcome, though MIS-C with shock can be life-threatening subgroup in a resource-constrained country setting. On midterm follow-up, there is normalisation of LV function in all and recovery of coronary abnormalities in 80% of patients.

Parents of infants with complex CHDs often describe their infants as especially fussy, irritable, and difficult to sooth, which together with the illness caretaking demands add to their stress. Little is known about how the behavioural style or temperament in the early months after discharge relates to parental quality of life. This study aimed to explore the associations between early infant temperament characteristics and parental quality of life in parents of infants with complex CHD.

This descriptive, cross-sectional study, utilised data collected in a previously described multisite randomised clinical trial in the United States. Multivariable linear regression models were used to examine the associations of interest.

Results demonstrated negative significant associations between most infant temperament subscales and parental quality of life. Higher scores on the Activity (β = −3.03, p = 0.021), Approach (β = −1.05, p = 0.021), Adaptability (β = −3.47, p = 0.004), Intensity (β = −2.78, p = 0.008), Mood (β = −4.65, p < 0.001), and Distractibility (β = −3.36, p = 0.007 were all significantly associated with lower parental quality of life scores, adjusting for parental dyadic adjustment, insurance type, number of medications, and number of unscheduled cardiologist visits.

Parental perceptions of infant’s difficult behavioural style or temperament characteristics appear to be associated with poorer quality of life in parents of infants with complex CHD post-cardiac surgery. Findings can be used in the screening process of families at potential risk of increased stress and poor illness adaptation and in the design of interventions to target parental mental health in this vulnerable patient population.

Survival after paediatric in-hospital cardiac arrest is worse on nights and weekends without demonstration of disparity in cardiopulmonary resuscitation quality. It is unknown whether these findings differ in children with CHD. This study aimed to determine whether cardiopulmonary resuscitation quality might explain the hypothesised worse outcomes of children with CHD during nights and weekends.

In-hospital cardiac arrest data collected by the Pediatric Resuscitation Quality Collaborative for children with CHD. Chest compression quality metrics and survival outcomes were compared between events that occurred during day versus night, and during weekday versus weekend using multivariable logistic regression.

We evaluated 3614 sixty-second epochs of chest compression data from 132 subjects between 2015 and 2020. There was no difference in chest compression quality metrics during day versus night or weekday versus weekend. Weekday versus weekend was associated with improved survival to hospital discharge (adjusted odds ratio 4.56 [1.29,16.11]; p = 0.02] and survival to hospital discharge with favourable neurological outcomes (adjusted odds ratio 6.35 [1.36,29.6]; p = 0.02), but no difference with rate of return of spontaneous circulation or return of circulation. There was no difference in outcomes for day versus night.

For children with CHD and in-hospital cardiac arrest, there was no difference in chest compression quality metrics by time of day or day of week. Although there was no difference in outcomes for events during days versus nights, there was improved survival to hospital discharge and survival to hospital discharge with favourable neurological outcome for events occurring on weekdays compared to weekends.

Peripheral perfusion index has been proposed as a possible method for detecting circulatory impairment. We aimed to determine the normal range of peripheral perfusion index in healthy newborns and compare it with that of newborns with CHD.

Right-hand saturation and right-hand peripheral perfusion index levels were recorded, and physical examination and echocardiography were performed in newborns who were 0–28 days old and whom were evaluated in our paediatric cardiology outpatient clinic. The saturation and peripheral perfusion index levels of newborns with normal heart anatomy and function were compared with those of newborns with CHD.

Out of 358 newborns (238 mature and 75 premature) enrolled in the study, 39 had CHD (20 mild CHD, 13 moderate CHD, and 6 severe CHD), of which 29 had CHD with left-to-right shunting, 5 had obstructive CHD, and 5 had cyanotic CHD. No newborn had clinical signs of hypoperfusion or heart failure, such as prolonged capillary refill, weakened pulses, or coldness of extremities. Peripheral perfusion index level was median (interquartile range) 1.7 (0.6) in healthy newborns, 1.8 (0.7) in newborns with mild CHD, and 1.8 (0.4) in newborns with moderate and severe CHD, and there was no significant difference between the groups regarding peripheral perfusion index level.

Peripheral perfusion index remains unchanged in newborns with CHD without the clinical signs of hypoperfusion or heart failure. Larger studies with repeated peripheral perfusion index measurements can determine how valuable this method will be in the follow-up of newborns with CHD.

This study evaluated the effect of music intervention on the anxiety and stress responses of patients who underwent an interventional cardiac catheterisation.

The study design was a pre- and post-test randomised controlled trial that included 94 patients who underwent a transcatheter atrial septal defect closure. Patients were allocated to receive either music intervention (n = 47) or usual care (n = 47) during the interventional cardiac catheterisation. Music intervention effectiveness was examined in terms of anxiety, salivary cortisol level, and heart rate variability.

The average age of participants was 45.40 years (±16.04) in the experimental group and 47.26 years (±13.83) in the control group. Two-thirds (66.0%) of the participants in each group were women. State anxiety (F = 31.42, p < 0.001), anxiety-numerical rating scale (F = 20.08, p < 0.001), salivary cortisol levels (F = 4.98, p = 0.021), and low-frequency component/high-frequency component ratio (F = 17.31, p < 0.001) in the experimental group were significantly reduced compared with those in the control group at the end of the music intervention.

This study provides practical evidence of a reduction in anxiety and stress response from music intervention preceding an interventional cardiac catheterisation, indicating that this intervention should be considered in clinical management.

Compare rates, clinical characteristics, and outcomes of paediatric palliative care consultation in children supported on extracorporeal membrane oxygenation admitted to a single-centre 16-bed cardiac or a 28-bed paediatric ICU.

Retrospective review of clinical characteristics and outcomes of children (aged 0–21 years) supported on extracorporeal membrane oxygenation between January, 2017 and December, 2019 compared by palliative care consultation.

One hundred children (N = 100) were supported with extracorporeal membrane oxygenation; 19% received a palliative care consult. Compared to non-consulted children, consulted children had higher disease severity measured by higher complex chronic conditions at the end of extracorporeal membrane oxygenation hospitalisation (5 versus. 3; p < 0.001), longer hospital length of stay (92 days versus 19 days; p < 0.001), and higher use of life-sustaining therapies after decannulation (79% versus 23%; p < 0.001). Consultations occurred mainly for longitudinal psychosocial-spiritual support after patient survived device deployment with a median of 27 days after cannulation. Most children died in the ICU after withdrawal of life-sustaining therapies regardless of consultation status. Over two-thirds of the 44 deaths (84%; n = 37) occurred during extracorporeal membrane oxygenation hospitalisation.

Palliative care consultation was rare showing that palliative care consultation was not viewed as an acute need and only considered when the clinical course became protracted. As a result, there are missed opportunities to involve palliative care earlier and more frequently in the care of extracorporeal membrane survivors and non-survivors and their families.

Chest pain is a common complaint among paediatric patients and cardiac troponin (cTn) level is often part of the initial emergency department evaluation. It is well known that after intense endurance exercise cTn levels can be elevated in patients with otherwise healthy hearts, however the effect shorter duration exercise has on cTn levels in this population is not known.

Determine the behaviour of cTn levels in healthy children and adolescent patients after short burst, high-intensity aerobic exercise.

Patients without haemodynamically significant heart disease referred for a treadmill exercise stress test (EST) were recruited over a 6-month period. cTn levels were measured prior to exercise and 4 hours after exercise.

Thirteen patients enrolled. Indications for EST were exertional syncope (six), chest pain (four), and long QT syndrome (three). The median exercise time was 12.9 (9.9–13.7) minutes with an average endurance at the fiftieth percentile for age and maximum heart rate rose to an average of 92 (74–98)% of the predicted peak for age. cTn levels prior to exercise were undetectable in all patients. There was no cTn rise in any patient after exercise. There were no ischaemic changes or arrhythmias on exercise electrocardiograms.

Serum cTn levels do not rise significantly in healthy children after short duration, high-intensity aerobic exercise. Physicians evaluating paediatric patients with an elevated cTn level after less than prolonged strenuous activity likely cannot attribute this lab value solely to exercise and may need to undertake further cardiac investigation.

Health-related quality of life in children who have undergone the Ross procedure has not been well characterised. The aim of this study was to characterise health-related quality of life in this cohort and compare to children with other CHD.

In this cross sectional, single-centre study, health-related quality of life was assessed in patients who underwent a non-neonatal Ross procedure using the Pediatric Quality of Life Inventory. Ross cohort scores were compared with healthy norms, patients with CHD requiring no surgical intervention or had curative surgery (Severity 2, S2) and patients who were surgically repaired with ≥1 surgical procedure and with significant residual lesion or need for additional surgery (Severity 3, S3). Associations between Pediatric Quality of Life Inventory score and patient factors were also examined.

68 patients completed surveys. Nearly one-sixth of patients had overall scores below the cut-off for at-risk status for impaired health-related quality of life. There was no difference in overall health-related quality of life score between the Ross cohort and healthy children (p = 0.56) and S2 cohort (p = 0.97). Health-related quality of life was significantly higher in the Ross cohort compared to S3 cohort (p = 0.02). This difference was driven by a higher psychosocial health-related quality of life in the Ross cohort as compared to S3 cohort (p = 0.007). Anxiety scores were significantly worse in the Ross cohort compared to both S2 (p = 0.001) and S3 (p = 0.0017), respectively.

Children who have undergone a Ross procedure report health-related quality of life equivalent to CHD not requiring therapy and superior to CHD with residual lesions. Despite these reassuring results, providers should be aware of potential anxiety among Ross patients.

The optimal visceral preservation method during aortic arch reconstruction is still controversial. It has been thought that double aortic cannulation is effective. Herein, it was aimed to evaluate this technique in providing distal perfusion.

A total of 74 patients who underwent arch reconstruction between 2011 and 2019 were included. Patients were grouped according to ventricular physiology and cannulation strategies. Group 1 were univentricle patients, and all had double aortic cannulation. Group 2 were biventricular patients. Group 2A double aortic cannulation-done and Group 2B non-double aortic cannulation were included. Lactate, urea, creatinine values, renal functions, and need for peritoneal dialysis of patients were evaluated.

There were no complications observed due to descending aortic cannulation in any of the patients. A delayed sternal closure and the need for peritoneal dialysis were more common in the Group 1 (p < 0.01). The preoperative and postoperative 1st- and 2nd-day lactate, urea, and creatinine values in the Group 1 were higher (p < 0.05) when compared with the Group 2A and 2B. The same values were higher in Group 2A than the Group 2B (p < 0.05).

The positive effect of double aortic cannulation on renal dysfunction could not be demonstrated. This may be associated with a <1 month of age, low weight, complex surgical procedure, and high preoperative lactate, urea, and creatinine values in patients with double aortic cannulation.

Paediatric ICUs have shared the burden of the COVID-19 pandemic, including subspecialty cardiac ICUs. We sought to address knowledge gaps regarding patient characteristics, acuity, and sequelae of COVID-19 in the paediatric cardiac ICU setting.

Retrospective review of paediatric cardiac ICU admissions with COVID-19-related disease.

Single centre tertiary care paediatric cardiac ICU.

All patients with PCR/antibody evidence of primary COVID-19 infection, and/or Multisystem Inflammatory Syndrome in Children, were admitted between 26 March, 2020 and 31 March, 2021.

None.

Patient-level demographics, pre-existing conditions, clinical symptoms, and outcomes related to ICU admission were captured from medical records.

Among 1064 patients hospitalised with COVID-19/Multisystem Inflammatory Syndrome in Children, 102 patients (9.5%) were admitted to cardiac ICU, 76 of which were symptomatic (median age 12.5 years [IQR 7.5–16.0]). The primary system involved at presentation was cardiovascular in 48 (63%). Vasoactive infusions were required in 62% (n = 47), with eight patients (11%) requiring VA ECMO. Severity of disease was categorised as mild/moderate in 16 (21%) and severe/critical in 60 patients (79%). On univariate analysis, African-American race, presentation with gastrointestinal symptoms or elevated inflammatory markers were associated with risk for severe disease. All-cause death was observed in five patients (7%, n = 5/72) with four patients remaining hospitalised at the time of data query.

COVID-19 and its cardiovascular sequelae were associated with important morbidity and significant mortality in a notable minority of paediatric patients admitted to a paediatric cardiac ICU. Further study is required to quantify the risk of morbidity and mortality for COVID-19 and sequelae.

CHD was the most prevalent congenital anomaly (60.9 per 10,000, 95% CI 59.0–62.8) in England in 2018, with 1767 babies born with severe cardiac defects. The 30-day survival rates for complex procedures continue to improve; however despite care advances, the early post-operative period and first year of life remain a critical time for these infants. The Congenital Heart Assessment Tool was developed to support parental decision-making, standardise care provision, improve communication, and the safety and quality of care.

To further evaluate the Congenital Heart Assessment Tool.

A four centre collaborative mixed-methods quality improvement project funded by The Health Foundation, involving eight phases conducted during 2016–2018. Phases six to eight (clinical simulation exercise, parent workshop, and updated tool) are reported in this paper.

Four themes emerged from the clinical simulation exercise (phase six) including: improving documentation; preparation of parents; preparation of health care professionals; and communication. One main theme emerged from the parent workshop (phase seven): “what parents know versus what professionals know [about CHD]”.

These phases further validated the effectiveness of the CHATm in terms of triggering amber and red indicators and demonstrated parents’ ability to identify deterioration in their infant’s clinical condition. Recommendations arising from the quality improvement project enabled the project team to create an updated version of the Congenital Heart Assessment Tool, CHAT2.

The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature.

The hospital records, echocardiographic and other imaging modality data, outpatients’ records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed.

Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2–60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months – 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later.

Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.

This study aimed to examine the effects of theophylline use in preventing renal dysfunction in patients undergoing CHD surgery.

A total of 94 patients aged 1–60 months were included in the study. Patients in the theophylline group were enrolled according to a pre-defined protocol for treatment administration, while controls were selected retrospectively from patients without theophylline treatment during the same period – who were matched according to critical baseline characteristics.

The incidence of acute kidney injury was similar between the two groups (p = 1.000). Higher urinary output and lower fluid balance were found intraoperatively and also postoperatively in the theophylline group (all, p < 0.050). Postoperative decrease in urinary output and estimated glomerular filtration rate were higher in the theophylline group (p < 0.050). Lower postoperative urea and creatinine levels were shown in theophylline recipients (p < 0.050). Urea levels increased significantly in the non-theophylline group during surgery (p < 0.001), and no significant change was observed in theophylline group (p = 0.136). Postoperative increase in creatinine and lactate levels was demonstrated in theophylline group (p < 0.050), and lactate levels were higher in the non-theophylline group during and after cardiopulmonary bypass (p = 0.010). Multiple linear regression analysis revealed less reduction in estimated glomerular filtration rate with higher age and in the presence of theophylline use (p < 0.050).

Although we demonstrated a similar incidence of acute kidney injury in the both groups, we revealed an important decrease in serum creatinine, urea and lactate levels, accompanied by improved estimated glomerular filtration rate, increased urine output and decreased fluid overload, with theophylline treatment, suggesting that renal functions significantly improved with the use of theophylline.

Transposition of great arteries is one of newborns' most common cyanotic CHDs, and its treatment is arterial switch operation in the first days of life. Low cardiac output syndrome may develop in the early postoperative period. In this study, we evaluated perfusion index and left ventricular output blood flow changes in patients who underwent arterial switch operation and developed low cardiac output syndrome.

This study was conducted prospectively in newborns with transposition of great arteries who underwent arterial switch operation between 1st August 2020 and 1st August 2022. Low cardiac output syndrome score and left ventricular output were investigated. Initially, 6th, 12th, 18th, and 24th hour perfusion index and left ventricular output values of patients with and without low cardiac output syndrome were recorded. The results were evaluated statistically.

A total of 60 patients were included in the study. Sex distribution was equal. The median age at the time of surgery was 5 days (interquartile range 3–7 days), and the median weight was 3.1 kg (interquartile range 2.9–3. 4). Low cardiac output syndrome was detected in 30% (n = 18) of cases. The median perfusion index of patients who developed low cardiac output syndrome was significantly lower at the 12th, 18th, and 24th hours (p < 0.05) (0.99 versus 1.25, 0.86 versus 1.21, and 0.96 versus 1.33, respectively). Similarly, the median left ventricular output of patients who developed low cardiac output syndrome was significantly lower at 12th, 18th, and 24th hours (p < 0.05) (95 versus 110 ml/kg/min, 89 versus 109 ml/kg/min, and 92 versus 112 ml/kg/min, respectively). There was a significant correlation between perfusion index values and left ventricular output at all measurements (r > 0.500, p < 0.05).

Perfusion index and left ventricular output measurements decreased in newborns who developed low cardiac output syndrome after arterial switch operation, especially at 12th and 18th hours. Serial perfusion index and left ventricular output measurements can be instructive in predicting low cardiac output syndrome development.

Patients with single ventricle CHD have significant morbidity and healthcare utilisation throughout their lifetime, including non-cardiac hospital admissions. Respiratory viral infections are the main cause of hospitalisation in children, but few data exist for single ventricle patients. We sought to identify how respiratory viral infections impact patients with single ventricle CHD and potential differences between Glenn and Fontan circulation.

We conducted a retrospective study of patients seen from 01/01/2011–12/31/2020. We identified patients with a history of single ventricle CHD and Glenn palliation, and a normoxic control group with isolated atrial septal defect requiring surgical closure. We compared viral-related clinical presentations, admissions, and admission characteristics.

A total of 312 patients were included (182 single ventricle, 130 atrial septal defect). Single ventricle patients were more likely than children with isolated atrial septal defect to be admitted with a respiratory virus (odds ratio 4.15 [2.30–7.46]), but there was no difference in mechanical ventilation or hospital length of stay (p = 0.4709). Single ventricle patients with Glenn circulation were more likely than those with Fontan circulation to present and be admitted (odds ratio 3.25 [1.62–6.52]), but there was no difference in ICU admission, mechanical ventilation, or hospital length of stay (p = 0.1516).

Respiratory viral infections are prevalent but uncomplicated in patients with single ventricle CHD. Viral-related presentations and admissions are more prevalent during the period of Glenn circulation compared to Fontan circulation; however, rate of mechanical ventilation and hospital length of stay are similar.