Paediatric cardiac surgery on cardiopulmonary bypass induces substantial physiologic changes that contribute to post-operative morbidity and mortality. Fluid overload and oedema are prevalent complications, routinely treated with diuretics. The optimal diuretic choice, timing of initiation, dose, and interval remain largely unknown.

To guide clinical practice and future studies, we used PubMed and EMBASE to systematically review the existing literature of clinical trials involving diuretics following cardiac surgery from 2000 to 2020 in children aged 0–18 years. Studies were assessed by two reviewers to ensure that they met eligibility criteria.

We identified nine studies of 430 children across four medication classes. Five studies were retrospective, and four were prospective, two of which included randomisation. All were single centre. There were five primary endpoints – urine output, acute kidney injury, fluid balance, change in serum bicarbonate level, and required dose of diuretic. Included studies showed early post-operative diuretic resistance, suggesting higher initial doses. Two studies of ethacrynic acid showed increased urine output and lower diuretic requirement compared to furosemide. Children receiving peritoneal dialysis were less likely to develop fluid overload than those receiving furosemide. Chlorothiazide, acetazolamide, and tolvaptan demonstrated potential benefit as adjuncts to traditional diuretic regimens.

Early diuretic resistance is seen in children following cardiopulmonary bypass. Ethacrynic acid appears superior to furosemide. Adjunct diuretic therapies may provide additional benefit. Study populations were heterogeneous and endpoints varied. Standardised, validated endpoints and pragmatic trial designs may allow investigators to determine the optimal diuretic, timing of initiation, dose, and interval to improve post-operative outcomes.

Targeted drug development efforts in patients with CHD are needed to standardise care, improve outcomes, and limit adverse events in the post-operative period. To identify major gaps in knowledge that can be addressed by drug development efforts and provide a rationale for current clinical practice, this review evaluates the evidence behind the most common medication classes used in the post-operative care of children with CHD undergoing cardiac surgery with cardiopulmonary bypass.

We systematically searched PubMed and EMBASE from 2000 to 2019 using a controlled vocabulary and keywords related to diuretics, vasoactives, sedatives, analgesics, pulmonary vasodilators, coagulation system medications, antiarrhythmics, steroids, and other endocrine drugs. We included studies of drugs given post-operatively to children with CHD undergoing repair or palliation with cardiopulmonary bypass.

We identified a total of 127 studies with 51,573 total children across medication classes. Most studies were retrospective cohorts at single centres. There is significant age- and disease-related variability in drug disposition, efficacy, and safety.

In this study, we discovered major gaps in knowledge for each medication class and identified areas for future research. Advances in data collection through electronic health records, novel trial methods, and collaboration can aid drug development efforts in standardising care, improving outcomes, and limiting adverse events in the post-operative period.

This study investigated the impact of the Webinar on deep human learning of CHD.

This cross-sectional survey design study used an open and closed-ended questionnaire to assess the impact of the Webinar on deep learning of topical areas within the management of the post-operative tetralogy of Fallot patients. This was a quantitative research methodology using descriptive statistical analyses with a sequential explanatory design.

One thousand-three-hundred and seventy-four participants from 100 countries on 6 continents joined the Webinar, 557 (40%) of whom completed the questionnaire. Over 70% of participants reported that they “agreed” or “strongly agreed” that the Webinar format promoted deep learning for each of the topics compared to other standard learning methods (textbook and journal learning). Two-thirds expressed a preference for attending a Webinar rather than an international conference. Over 80% of participants highlighted significant barriers to attending conferences including cost (79%), distance to travel (49%), time commitment (51%), and family commitments (35%). Strengths of the Webinar included expertise, concise high-quality presentations often discussing contentious issues, and the platform quality. The main weakness was a limited time for questions. Just over 53% expressed a concern for the carbon footprint involved in attending conferences and preferred to attend a Webinar.

E-learning Webinars represent a disruptive innovation, which promotes deep learning, greater multidisciplinary participation, and greater attendee satisfaction with fewer barriers to participation. Although Webinars will never fully replace conferences, a hybrid approach may reduce the need for conferencing, reduce carbon footprint. and promote a “sustainable academia”.

This study demonstrates the clinical and electrophysiological details of catheter ablation conducted in children with focal atrial tachycardia using three-dimensional electroanatomic mapping systems.

Electrophysiological procedures were performed using the EnSite™ system.

Between 2014 and 2020, 60 children (median age 12.01 years [16 days–18 years]; median weight 41.5 kg [3–98 kg]) with focal atrial tachycardia and treated with catheter ablation were evaluated retrospectively. Tachycardia-induced cardiomyopathy was developed in 15 patients (25%). Most of the focal atrial tachycardia foci were right-sided (75%), and more than one focus was found in four patients. Radiofrequency ablation was performed in 47 patients (irrigated radiofrequency ablation in seven cases), cryoablation in 9, and radiofrequency ablation and cryoablation in the same session in 4 patients. The median procedural time was 163.5 minutes (82–473 minutes). Fluoroscopy was used in 29 of (48.3%) patients (especially for left-side substrate) with a mean time of 8.6 ± 6.2 minutes. The acute success rate was 95%. The procedure failed in three patients, and recurrence was observed in 3.5% of patients (2/57) during a median follow-up of 17 months (2–69 months). The second ablation was performed in four cases, of which three were successful. Overall success rate was 96.6% with no major complications observed, except in one patient with minimal pericardial effusion.

Catheter ablation seems to be an effective and safe treatment in focal atrial tachycardia. Electroanatomic mapping system can facilitate the ablation procedure and minimise radiation exposure.

The single- and double-patch repairs are undoubtedly the most commonly used techniques for the surgical management of partial anomalous pulmonary venous connection associated with sinus venosus atrial septal defect. The aim of this study was to retrospectively compare early and long-term surgical outcomes in paediatric and adult patients, focusing in particular on the occurrence of ectopic atrial rhythm.

Seventy patients (male: 38, 54.2%) underwent surgical repair for partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. Forty-nine patients (70%) underwent surgical repair in paediatric age (<16 years old), while 21 of (30%) patients were operated in adulthood. Thirty patients (42.8%) underwent single-patch repair and 39 patients (55.7%) underwent double-patch repair. In only one patient, the Warden procedure was performed (1.4%). Median follow-up time was 52 months (IQ 15.1–113).

The type of surgical technique didn’t affect the incidence of ectopic atrial rhythm (26.6% in single-patch group and 25.6% in double-patch groups, p = 0.9). At long-term follow-up, ectopic atrial rhythm, as an expression of sinoatrial node disturbance, was however significantly more frequent in the paediatric population (28.8% paediatric group and 4.7% adult group, p = 0.02).

The higher incidence of ectopic atrial rhythm in children is probably related to the closer position of the sinus node to the superior cavoatrial incision, which makes irreversible iatrogenic traumatism more likely to occur. Surgical techniques that avoid any manipulation on the superior cavoatrial junction should, therefore, be preferred for children undergoing partial anomalous pulmonary venous connection repair.

Medical advancements have encouraged minimally invasive surgical repair of congenital heart defects such as ventricular septal defects (VSDs), and the diagnostic process can now be carried out using non-traditional techniques such as pulse oximetry. This, in turn, has improved clinical outcomes with reduced complication rates post-surgery. However, the variations in type of VSDs, age of patient, comorbidities, and access to closure devices may limit the efficacy of surgical advancements.

Articles were identified amongst Scopus, MEDLINE, and PubMed using various relevant search strings using PRISMA guidelines. Of the 115 articles initially extracted, 10 were eventually reviewed after duplicates and irrelevant studies were removed.

Of the 24 eligible articles, 10 papers were selected for analysis. Minimally invasive approaches to VSD repair was associated with satisfactory short-term outcomes when compared to open repair. For diagnosis of congenital VSD, whilst recent advances such as pulse oximetry method and genome analysis are more sensitive, the limited availability and access to such investigatory methods must be recognised.

Pulse oximetry and fetal echocardiography are established non-invasive diagnostic tools for VSD. The recent advances in minimally invasive treatment options including periventricular approach and transcatheter techniques have improved patient outcomes, yet at the expense of higher residual rates. Careful patient selection for each technique and follow-up should be planned through multidisciplinary team meetings.

The number of adults requiring surgeries for CHD is increasing. We sought to evaluate the utility of the vasoactive-ventilation-renal (VVR) score as a predictor of prolonged length of stay in adults following CHD surgery.

This is a retrospective review of 158 adult patients who underwent CHD surgery involving cardiopulmonary bypass. VVR score was calculated upon arrival to ICU and every 6 hours for the first 48 hours post-operatively. Our primary outcome was prolonged length of stay defined as hospital length of stay greater than 75th percentile for the cohort (≥8 days).

The study cohort had a median age of 25.6 years (18–60 years), and 83 (52.5%) were male. The groups with and without prolonged length of stay were comparable in age, gender, race, and surgical severity score. VVR score was significantly higher at all time points in the group with prolonged length of stay. The first post-operative day peak VVR score ≥13 had a sensitivity of 81% and specificity of 75% for predicting prolonged length of stay (p = 0.0001). On regression analysis, peak VVR score during the first day was independently associated with prolonged length of stay.

Peak VVR score during the first post-operative day was a strong predictor of prolonged length of stay in adults following CHD surgery.

The contribution of neonatal cyanosis, inherent to cyanotic congenital heart disease, to the magnitude of neurologic injury during deep hypothermic circulatory arrest has not been fully delineated. This study investigates the impact of cyanosis and deep hypothermic circulatory arrest on brain injury.

Neonatal piglets were randomised to placement of a pulmonary artery to left atrium shunt to create cyanosis or sham thoracotomy. At day 7, animals were randomised to undergo deep hypothermic circulatory arrest or sham. Arterial oxygen tension and haematocrit were obtained. Neurobehavioural performance was serially assessed. The animals were sacrificed on day 14. Brain tissue was assessed for neuronal necrosis using a 5-point histopathologic score.

Four experimental groups were analysed (sham, n = 10; sham + deep hypothermic circulatory arrest, n = 8; shunt, n = 9; shunt + deep hypothermic circulatory arrest, n = 7). Cyanotic piglets had significantly higher haematocrit and lower partial pressure of oxygen at day 14 than non-cyanotic piglets. There were no statistically significant differences in neurobehavioural scores at day 1. However, shunt + deep hypothermic circulatory arrest piglets had evidence of greater neuronal injury than sham animals (median (range): 2 (0–4) versus 0 (0–0), p = 0.02).

Cyanotic piglets undergoing deep hypothermic circulatory arrest had increased neuronal injury compared to sham animals. Significant injury was not seen for either cyanosis or deep hypothermic circulatory arrest alone relative to shams. These findings suggest an interaction between cyanosis and deep hypothermic circulatory arrest and may partially explain the suboptimal neurologic outcomes seen in children with cyanotic heart disease who undergo deep hypothermic circulatory arrest.

To examine the use of early intervention services in infants with CHD after open-heart surgery and identify factors associated with receipt of services.

Surveys were administered to caregivers of infants who underwent open-heart surgery before 1 year of age at a single institution between July, 2017 and July, 2018. Information regarding the infant’s use of early intervention services and the caregiver’s experience with the programme was obtained. Clinical data were retrieved from the medical record review. Logistic regression identified factors associated with receipt of services.

The study included 158 eligible infants. Ninety-eight caregivers (62%) completed the surveys. Of those surveyed, 53.1% of infants were currently or previously enrolled in early intervention services. Infants most frequently received physical therapy (76.9%). The majority of caregivers found services to be moderately/very helpful (92.3%) and sufficient for their child (76.9%). In the univariate analysis, single-ventricle disease, known syndrome/genetic abnormality, extracardiac anomaly, and longer intensive care and hospital length of stay were associated with receipt of services. Single-ventricle disease (p = 0.004) and known syndrome/genetic abnormality (p < 0.0001) remained independently associated with receipt of services in the multivariable analysis.

Amongst infants at risk for neurodevelopmental deficits, approximately half received services after open-heart surgery. Caregivers expressed satisfaction with the programme. While infants with single-ventricle disease and a known syndrome/genetic abnormality were more likely to receive early intervention services, many at-risk infants with CHD failed to receive services. Further research is needed to identify barriers to early intervention services and promote developmental outcomes.

Patients with CHD exhibit reduced isometric muscle strength and muscle mass; however, little is known how these parameters relate. Therefore, the aim was to investigate the relation between isometric limb muscle strength and muscle mass for patients in comparison to age- and sex-matched control subjects.

Seventy-four patients (35.6 ± 14.3 years, women n = 22) and 74 matched controls were included. Isometric muscle strength in elbow flexion, knee extension, and hand grip was assessed using dynamometers. Lean mass, reflecting skeletal muscle mass, in the arms and legs was assessed with dual-energy x-ray absorptiometry.

Compared to controls, patients had lower muscle strength in elbow flexion, knee extension, and hand grip, and lower muscle mass in the arms (6.6 ± 1.8 kg versus 5.8 ± 1.7 kg, p < 0.001) and legs (18.4 ± 3.5 kg versus 15.9 ± 3.2 kg, p < 0.001). There was no difference in achieved muscle force per unit muscle mass in patients compared to controls (elbow flexion 0.03 ± 0.004 versus 0.03 ± 0.005 N/g, p = 0.5; grip strength 0.008 ± 0.001 versus 0.008 ± 0.001 N/g, p = 0.7; knee extension 0.027 ± 0.06 versus 0.028 ± 0.06 N/g, p = 0.5). For both groups, muscle mass in the arms correlated strongly with muscle strength in elbow flexion (patients r = 0.86, controls, r = 0.89), hand grip (patients, r = 0.84, controls, r = 0.81), and muscle mass in the leg to knee extension (patients r = 0.64, controls r = 0.68).

The relationship between isometric muscle strength and limb muscle mass in adults with CHD indicates that the skeletal muscles have the same efficiency as in healthy controls.

The research was to introduce the experience of doubly committed subarterial ventricular septal defect (DCVSD) repaired through tricuspid approach.

From January, 2015 to September, 2019, 86 consecutive DCVSD paediatrics underwent repair via right subaxillary vertical incision (RAVI) through tricuspid approach. Perioperative and follow-up data were collected.

The age and weight at operation were 28.1 ± 18.5 (range: 7–101) months and 12.2 ± 4.2 (6–26.5) kg. There were two patients combined with discrete subaortic membrane, two patients with patent ductus arteriosus, one patient with atrial septal defect, and two patients with abnormal muscle bundle in right ventricular outflow tract. The mean size of ventricular septal defect was 7.0 ± 2.4 (3–13) mm. The defect was repaired with a piece of Dacron patch in 68 patients or directly with 1–2 pledgetted polypropylene sutures in 18 patients. The cardiopulmonary bypass time and aortic cross-clamp time were 46.2 ± 13.3 (23–101) minutes and 29.2 ± 11.5 (12–84) minutes. After 3.1 ± 2.4 (0–14) hours’ ventilator assist and 23.2 ± 32.1 (0–264) hours’ ICU stay, all patients were discharged safely. At the latest follow-up (27.9 ± 14.6 months), echocardiography showed trivial residual shunt in two patients. There was no malignant arrhythmia occurred and there was no chest deformity or asymmetrical development of the breast was found.

DCVSD repaired via right subaxillary vertical incision through tricuspid approach was safe and feasible, providing a feasible alternative to median sternotomy, and it can be performed with favourable cosmetic results.

Cardiac rehabilitation programmes for paediatric patients with congenital heart disease (CHD) have been shown to promote emotional and physical health without any associated adverse events. While prior studies have demonstrated the effectiveness of these types of interventions, there has been limited research into how the inclusion of psychological interventions as part of the programme impacts parent-reported and patient-reported quality of life.

Patients between the ages of 7 and 24 years with CHD completed a cardiac rehabilitation programme that followed a flexible structure of four in person-visits with various multidisciplinary team members, including paediatric psychologists. Changes in scores from the earliest to the latest session were assessed regarding exercise capacity, patient functioning (social, emotional, school, psychosocial), patient general and cardiac-related quality of life, patient self-concept, and patient behavioural/emotional problems.

From their baseline to final session, patients exhibited significant improvement in exercise capacity (p = 0.00009). Parents reported improvement in the patient’s emotional functioning, social functioning, school functioning, psychosocial functioning, cognitive functioning, communication, and overall quality of life. While patients did not report improvement in these above areas, they did report perceived improvement in certain aspects of cardiac-related quality of life and self-concept.

This paediatric cardiac rehabilitation programme, which included regular consultations with paediatric psychologists, was associated with divergent perceptions by parents and patients on improvement related to quality of life and other aspects of functioning despite improvement in exercise capacity. Further investigation is recommended to identify underlying factors associated with the differing perceptions of parents and patients.

Vasovagal syncope is the most common cause of syncope in childhood and its treatment is not at a satisfactory level yet. We aimed to investigate patients who were diagnosed with vasovagal syncope, did not benefit from conventional treatment, received midodrine treatment, and to evaluate their response to midodrine treatment.

Files of 24 patients who were diagnosed with recurrent vasovagal syncope, did not benefit from non-pharmacological treatments, and received midodrine treatment during June 2017–October 2019 were retrospectively analysed.

In total, 24 patients received a treatment dose of midodrine at 5 mg/day (2.5 mg BID) included in the study. The mean number of syncope was 5.75 ± 2.67 prior to treatment. Following treatment, the mean number of syncope was 0.42 ± 0.89. It was observed that syncope episodes did not recur in 17 patients, but it recurred in 4 out of 7 patients in the first 3 months of the treatment and did not recur in the following months. The episodes improved in two patients with an increase in the treatment dose, but the syncope episodes continued in only one patient.

It was concluded that midodrine treatment was effective and safe in adolescents with recurrent vasovagal syncope.

Understanding the impact of the COVID-19 pandemic on paediatric non-COVID-19-related care, as well as patient and caregiver concerns and stressors, is critical for informing healthcare delivery. It was hypothesised that high care disruptions and psychological stress would be observed among paediatric and adult CHD patients in the early phase of the pandemic.

A cross-sectional, international, electronic survey study was completed. Eligible participants included parents of children with acquired or CHD, adults with CHD, or caregivers of adults with CHD.

A total of 1220 participants from 25 countries completed the survey from 16 April to 4 May, 2020. Cardiac care disruption was significant with 38% reporting delays in pre-pandemic scheduled cardiac surgeries and 46% experiencing postponed cardiac clinic visits. The majority of respondents (75%) endorsed moderate to high concern about the patient with heart disease becoming ill from COVID-19. Worry about returning for in-person care was significantly greater than worry of harm to patient due to postponed care. Clinically significant psychological stress was high across the sample including children (50%), adults with CHD (42%), and caregivers (42%).

The early phase of the COVID-19 pandemic contributed to considerable disruptions in cardiac care for patients with paediatric and adult CHD. COVID-19-related fears are notable with potential to impact willingness to return to in-person care. Psychological stress is also very high necessitating intervention. Further study of the impact of delays in care on clinical outcomes is warranted.