Atrial septal defects are a common form of CHD and dependent on the size and nature of atrial septal defects, closure may be warranted. The paper aims to compare outcomes of transcatheter versus surgical repair of atrial septal defects.

A comprehensive electronic literature search was conducted. Primary studies were included if they compared both closure techniques. Primary outcomes included procedural success, mortality, and reintervention rate. Secondary outcomes included residual defect and mean hospital stay.

A total of 33 studies were included in meta-analysis. Mean total hospital stay was significantly shorter in the transcatheter cohort across both the adult (95% confidence interval, mean difference −4.05 (−4.78, −3.32) p < 0.00001) and paediatric populations (95% confidence interval, mean difference −4.78 (−5.97, −3.60) p < 0.00001). There were significantly fewer complications in the transcatheter group across both the adult (odds ratio 0.45, 95% confidence interval, [0.28, 0.72], p < 0.00001) and paediatric cohorts (odds ratio 0.26, 95% confidence interval, [0.14, 0.49], p < 0.00001). No significant difference in overall mortality was found between transcatheter versus surgical closure across the two groups, adult (odds ratio 0.76, 95% confidence interval, [0.40, 1.45], p = 0.41), paediatrics (odds ratio 0.62, 95% confidence interval, [0.21, 1.83], p = 0.39).

Both transcatheter and surgical approaches are safe and effective techniques for atrial septal defect closure. Our study has demonstrated the benefits of transcatheter closure in terms of lower complication rates and mean hospital stay. However, surgery still has a place for more complex closure and, as we have demonstrated, shows no difference in mortality.

To determine the optimal antithrombotic agent choice, timing of initiation, dosing and duration of therapy for paediatric patients undergoing cardiac surgery with cardiopulmonary bypass.

We used PubMed and EMBASE to systematically review the existing literature of clinical trials involving antithrombotics following cardiac surgery from 2000 to 2020 in children 0–18 years. Studies were assessed by two reviewers to ensure they met eligibility criteria.

We identified 10 studies in 1929 children across three medications classes: vitamin K antagonists, cyclooxygenase inhibitors and indirect thrombin inhibitors. Four studies were retrospective, five were prospective observational cohorts (one of which used historical controls) and one was a prospective, randomised, placebo-controlled, double-blind trial. All included were single-centre studies. Eight studies used surrogate biomarkers and two used clinical endpoints as the primary endpoint. There was substantive variability in response to antithrombotics in the immediate post-operative period. Studies of warfarin and aspirin showed that laboratory monitoring levels were frequently out of therapeutic range (variably defined), and findings were mixed on the association of these derangements with bleeding or thrombotic events. Heparin was found to be safe at low doses, but breakthrough thromboembolic events were common.

There are few paediatric prospective randomised clinical trials evaluating antithrombotic therapeutics post-cardiac surgery; most studies have been observational and seldom employed clinical endpoints. Standardised, validated endpoints and pragmatic trial designs may allow investigators to determine the optimal drug, timing of initiation, dosing and duration to improve outcomes by limiting post-operative morbidity and mortality related to bleeding or thrombotic events.

Prior to the recent release of appropriate use criteria for imaging valvulopathies in children, follow-up of valvular lesions, including isolated bicuspid aortic valve, was not standardised. We describe current follow up, treatment, and intervention strategies for isolated bicuspid aortic valve with varying degrees of stenosis, regurgitation, and dilation in children up to 18 years old and compare them with newly released appropriate use criteria.

Online survey was sent to members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and PediHeartNet.

Totally, 106 responses with interpretable data were received. For asymptomatic patients with isolated BAV without stenosis, regurgitation, or dilation follow-up-intervals increased from 7+/−4 months in the newborn period to 28 +/− 14 months at 18 years of age. Respondents recommended more frequent follow-up for younger patients and those with greater disease severity. More than 80% of respondents treat aortic regurgitation or aortic dilation in the setting of bicuspid aortic valve medically. In general, intervention was recommended once stenosis or regurgitation became severe (stenosis of >4 m/s; regurgitation with LV Z score 4) regardless of age, but was not routinely recommended for younger children (newborn – age 6 years) with severe dilation. Exercise was restricted at 38+/−11 mmHg echocardiographic mean gradient.

Current follow-up, treatment, and intervention strategies for isolated bicuspid aortic valve deviate from appropriate use criteria. Differences between the two highlight the need to better delineate the disease course, clarify recommendations for care, and encourage wider adoption of guidelines.

The Coronavirus Disease 2019 (COVID-19) pandemic negatively impacted global healthcare. Consequences in Pediatric and Congenital Heart Surgery programmes and mortality of congenital heart patients infected with severe acute respiratory syndrome coronavirus-2 (SARS-Cov-2) is still to be determined.

To study the COVID-19 pandemic implications in Brazilian Pediatric and Congenital Heart Surgery programmes.

We conducted a national online survey covering all states that perform Pediatric and Congenital Heart Surgery from 10 November to 22 November, 2020, using a Google forms questionnaire. We formulated questions related to impact on surgical volume, case-mix, and mortality. Then we asked about short-term post-operative COVID-19 infection and outcomes.

We received responses from 46 centres representing all states where there were a Pediatric and Congenital Heart Surgery programme and all high-volume centres across the country. All but one centre experienced a significant decrease in surgical volume, and 23.9% of the responders revealed less than one-quarter of volume decrement. On the other hand, in over 70% of the centres, there was a significant surgical volume reduction. In addition to this, there was a shift in case-mix in 41 centres (89.1%) towards more complex cases. More than one-third of the responders revealed increased mortality in 2020 compared to previous years, and 43.5% of the programmes (20 centres) had at least one patient contaminated by SARS-Cov-2, accounting for 48 patients. Mortality in post-operative infected patients was 45.8% (22 patients).

In general, Brazilian Pediatric and Congenital Heart Surgery programmes were severely affected by decreased surgical volume, unbalanced case-mix towards more complex cases, and increased mortality. Almost half of the programmes related post-operative COVID-19 contamination with high mortality.

Anomalous origin of the left coronary artery from the pulmonary artery is associated with high mortality if not timely surgery. We reviewed our experience with anomalous origin of the left coronary artery from the pulmonary artery to assess the preoperative variables predictive of outcome and post-operative recovery of left ventricular function.

A retrospective review was conducted and collected data from patients who underwent anomalous origin of the left coronary artery from the pulmonary artery repair at our institute from April 2005 to December 2019. Left ventricular function was assessed by ejection fraction and the left ventricular end-diastolic dimension index. The outcomes of reimplantation repair were analysed.

A total of 30 consecutive patients underwent anomalous origin of the left coronary artery from the pulmonary artery repair, with a median age of 14.7 months (range, 1.5–59.6 months), including 14 females (46.67%). Surgery was performed with direct coronary reimplantation in 12 patients (40%) and the coronary lengthening technique in 18 (60%). Twelve patients had concomitant mitral annuloplasty. There were two in-hospital deaths (6.67%), no patients required mechanical support, and no late deaths occurred. Follow-up echocardiograms demonstrated significant improvement between the post-operative time point and the last follow-up in ejection fraction (49.43%±19.92% vs 60.21%±8.27%, p < 0.01) and in moderate or more severe mitral regurgitation (19/30 vs 5/28, p < 0.01). The left ventricular end-diastolic dimension index decreased from 101.91 ± 23.07 to 65.06 ± 12.82 (p < 0.01).

Surgical repair of anomalous origin of the left coronary artery from the pulmonary artery has good mid-term results with low mortality and reintervention rates. The coronary lengthening technique has good operability and leads to excellent cardiac recovery. The decision to concomitantly correct mitral regurgitation should be flexible and be based on the pathological changes of the mitral valve and the degree of mitral regurgitation.

To assess the feasibility of percutaneous transcatheter device closure of ventricular septal defects in children weighing less than 10 kg.

Although percutaneous transcatheter device closure of ventricular septal defect is a well-established method of treatment in older children and adolescents, there is limited data on device closure in small children weighing less than 10 kg. We present our institutional experience of transcatheter VSD closure in children weighing less than 10 kg.

Medical records were reviewed for 16 children, who were selected for device closure of ventricular septal defects based on the inclusion criteria.

Out of 65 patients with a diagnosis of ventricular septal defect, 16 children less than 10 kg were attempted for percutaneous device closure. In 13 patients, the device was successfully released, and 3 patients needed surgical closure of the defect. Mean age and weight of the patients were 17.3 ± 12.7 months and 6.8 ± 3.2 kg, respectively. Mean defect size was 6 mm (range 3–10). There was no incidence of device embolisation or heart block or death. Five patients had residual left-to-right shunt immediately after the device release, which got closed by the first month’s follow-up. We had one accidental perforation of right ventricular free wall at the time of crossing of the defect, which was successfully repaired surgically.

Percutaneous device closure of ventricular septal defect in small children with weight below 10 kg is feasible with good short-term outcome. Careful patient selection is essential for procedural success and to avoid complications.

The standard transcatheter technique to profile the patent ductus arteriosus requires arterial access through the femoral artery and is associated with arterial complications, longer fluoroscopic time, contrast volume, and longer hospital stay.

To compare exclusive transvenous access with the standard procedures for patent ductus arteriosus closure and evaluate whether exclusive venous approach is a safe and effective alternative.

A total of 320 patients were included. A detailed echocardiographic evaluation of the duct morphology was performed. Patients were classified into group 1 included patients who underwent exclusive femoral venous access, without any injections of contrast media and group 2 included patients who underwent arterial and venous access.

Arterial access was achieved in 210 (65.6%). Successful closure of patent ductus arteriosus was achieved in 109 (99.1%) patients in group 1 and in 203 (96.7%) patients in group 2. The patent ductus arteriosus was large and was referred for surgical closure in one patient from group 1 and 7 patients from group 2. Residual patent ductus arteriosus was seen in 6 cases from group 1 (5.4%) and 12 patients from group 2 (5.7%). None of the cases in group 1 had vascular complications, while vascular complications were seen in 20 cases, all of them in group 2 (9.5%). Nonvascular complications were seen in one patient from group 1 (0.9%) and 15 patients in group 2 (7.1%). The procedure time and fluoroscopy times were less in patients with exclusive transvenous access.

Patent ductus arteriosus device closure without arterial access can be accomplished safely and effectively.

Cyanotic CHD is one of many disorders in paediatrics that influence the health of children in different clinical aspects. One of the fundamental aspects that may be affected is bone mineral density.

The aim of our study is to assess bone mineral density in children with congenital cyanotic heart disease of different anatomical diagnoses.

Cross-sectional, observational study included 39 patients (20 males) with congenital cyanotic heart disease of different anatomical diagnoses following with the cardiology clinic in Mansoura University children’s hospital. All patients were subjected to anthropometric measures, oxygen saturation assessment, and lumber bone mineral density using dual-energy X-ray absorptiometry.

Six patients (15.4%) out of the 39 included patients showed bone mineral density reduction, 13 patients (33.3%) showed bone mineral density with Z-score between −1 and −2, while 20 patients (51.3%) showed bone mineral density with Z-score more than −1.

Low bone mineral density can be found in children with cyanotic CHD, making it important to consider bone mineral density assessment and early treatment if needed to avoid further complications.

With increased survival, children with CHD are reaching adulthood, however, obesity amongst this cohort is an emerging problem. Making every contact count encourages clinicians to utilise contact to elicit behaviour change. The aim of this work was to identify whether the body habitus of children classified as obese was addressed during a clinical review.

A retrospective observational cohort study was completed using a cardiology outpatient dataset from 2010 to 2019. Inclusion criteria are all children with a body mass index z score classified as obese (≥ 2 z scores). Individual electronic patient records were reviewed to identify long-term anthropometric measures including (i) recognition of body habitus, (ii) prescription of physical activity or dietary intervention, and (iii) referral to a weight management programme or dietitian.

From the cohort of 95 patients, 285 “obese clinical encounters” were identified, at the time of a cardiology clinic attendance. Of those, obesity was acknowledged in 25 clinic letters (8.65%), but only 8 used the correct terms “obese” or “obesity” (2.77%). Action to tackle obesity was recorded in 9.3% of cases with a direct referral to a dietitian being made on 3 occasions (1.04%).

Body habitus is not being routinely addressed by cardiologists caring for paediatric and young adult cardiac patients. This study has recognised an alarmingly high incidence of missed opportunities to make every contact count, to manage those with obesity and associated risk factors.

Rheumatic heart disease is among the leading causes of acquired valvular heart disease in the developing world. However, there is no data available for rheumatic heart disease in the paediatric population of Sabah. This study collected data for acute rheumatic fever admissions among the paediatric population in Sabah over a period of 3 years.

This is a retrospective cohort study. All records for admissions to paediatric wards in Sabah for acute rheumatic fever from January 2016 to December 2018 were collected. The patient records were then traced and required information were collected.

A total of 52 cases of acute rheumatic fever were admitted. It was observed that the incidence of acute rheumatic fever was 74.4 per 100,000 paediatric admissions. Patients from the West Coast Division made up most of the admissions (n = 24, 46.2%). Male patients (n = 35, 67.3%) of the indigenous Kadazan-Dusun ethnicity (n = 21, 40.4%) were most commonly encountered. The mean age at time of presentation was 9.58 years. Most cases admitted (n = 38, 73.1%) were categorised as Priority 1 (severe rheumatic heart disease).

Most patients who were admitted had symptoms of heart failure and were diagnosed with severe rheumatic heart disease. Although this disease is preventable, the incidence in Sabah remains high. This study was limited as we only looked at patients who were admitted and we foresee the real incidence to be higher. Hence, there is an urgent need for a rheumatic heart disease registry in Malaysia to gather more data for prevention and early intervention.

There are a few number of case reports and small-scale case series reporting dilated cardiomyopathy due to vitamin D-deficient rickets. The present study evaluates the clinical, biochemical, and echocardiographic features of neonates with vitamin D deficiency.

In this prospective single-arm observational study, echocardiographic evaluation was performed on all patients before vitamin D3 and calcium replacement. Following remission of biochemical features of vitamin D deficiency, control echocardiography was performed. Biochemical and echocardiographic characteristics of the present cohort were compared with those of 27 previously published cases with dilated cardiomyopathy due to vitamin D deficiency.

The study included 148 cases (95 males). In the echocardiographic evaluation, none of the patients had dilated cardiomyopathy. All of the mothers were also vitamin D deficient and treated accordingly. Comparison of patients with normocalcaemia and hypocalcaemia at presentation revealed no statistically significant difference between the ejection fraction and shortening fraction, while left ventricle end-diastolic diameter and left ventricle end-systolic diameter were higher in patients with hypocalcaemia. Previously published historical cases were older and had more severe biochemical features of vitamin D deficiency.

To the best of our knowledge, in this first and largest cohort of neonates with vitamin D deficiency, we did not detect dilated cardiomyopathy. Early recognition and detection before developing actual rickets and preventing prolonged hypocalcaemia are critically important to alleviate cardiac complications.

The diagnosis of hypertension in adolescents aged ≥13 and <16 years is based on the percentile according to age, gender, and height in the European Society of Hypertension guidelines guideline; whereas, the American Academy of Pediatrics guideline uses blood pressure above 130/80 mmHg as a single criterion. Therefore, this study aimed to evaluate the compatibility of these two guidelines in adolescents aged ≥13 and <16 years.

This study was designed by retrospectively screening the records of 395 adolescents with both office and 24-hour ambulatory blood pressure measurements. Each blood pressure measurement was classified according to both the ESGH2016 and AAP2017 guidelines. Patients were divided into three subgroups according to body mass index. Cohen’s kappa analysis was used to evaluate the agreement between the two guidelines.

The majority of adolescents were normotensive according to both guidelines, 55.9% by ESHG2016 and 43.1% by AAP2017. For the whole group, the frequency of hypertension was 32.4% with ESHG2016 and 34.4% with AAP2017; while, in obese patients, hypertension frequencies were 38.8% and 43.3%, respectively. The diagnosis of hypertension was demonstrated with the two guidelines, and there was significant agreement at a substantial level, both for the obese subgroup and the whole study group (kappa value = 0.738 and 0.785, respectively). The frequency of white-coat hypertension was higher with the AAP2017 guideline (28.1% versus 16.2%, p < 0.001).

With our experience in this single-centre study, it seems that both the AAP2017 and the ESHG2016 guidelines can be used in the diagnosis of hypertension in adolescents.

Expert guidance from scientific societies and regulatory agencies recommend a framework of principles for frequency of in-person evaluations and remote monitoring for patients with cardiac implantable electronic devices. However, there are limited data regarding adherence to recommendations among paediatric electrophysiologists, and there are no data regarding cardiac implantable electronic device-related ancillary testing.

To assess current clinical practices for cardiac implantable electronic device in-person evaluation, remote monitoring, and cardiac implantable electronic device-related ancillary testing, the Paediatric and Congenital Electrophysiology Society members were surveyed. The main outcome measures were variations in frequency of in person evaluation, frequency of remote monitoring, and cardiac implantable electronic device-related ancillary testing.

All respondents performed in-person evaluation at least once a year, but <50% of respondents performed an in-person evaluation within 2 weeks of cardiac implantable electronic device implantation. Remote monitoring was performed every 3 months for pacemakers and implantable cardioverter defibrillators by 71 and 75% respondents, respectively. Follow-up echocardiography was performed every 2–3 years by 53% respondents for patients with >50% ventricular pacing. Majority of respondents (75%) did not perform either an exercise stress test or ambulatory Holter monitoring or chest X-ray (65%) after cardiac implantable electronic device implantation.

This survey identified significant practice variations in cardiac implantable electronic device in- person evaluation, remote monitoring, and ancillary testing practices among paediatric electrophysiologists. Cardiac implantable electronic device management may be optimised by development of a paediatric-specific guidelines for follow-up and ancillary testing.

Adult patients diagnosed with type 1 diabetes mellitus are at risk for ventricular arrhythmias and sudden cardiac death.

The objective of our study is to evaluate the electrocardiographic data of children diagnosed with type 1 diabetes mellitus and to determine the possibility of arrhythmia in order to prevent sudden death.

Electrocardiographic data of 60 patients diagnosed with type 1 diabetes mellitus and 86 controls, who were compatible with the patient group in terms of age and gender, were compared.

The duration of diabetes in our patients with type 1 diabetes mellitus was 5.23 ± 1.76 years, and the haemoglobin A1c levels were 9.63% ± 1.75%. The heart rate, QRS, QT maximum, QT dispersion, QTc minimum, QTc maximum, QTc dispersion, Tp-e maximum, Tp-e maximum/QTc maximum and the JTc were significantly higher compared to the control group. There was no significant correlation between the duration of type 1 diabetes mellitus and HbA1c levels and the electrocardiographic data.

We attributed the lack of a significant correlation between the duration of type 1 diabetes mellitus and the haemoglobin A1c levels and the electrocardiographic data to the fact that the duration of diabetes was short, since our patients were children. We believe that patients with type 1 diabetes mellitus should be followed up closely in terms of sudden death, as they have electrocardiographic changes that may cause arrhythmias compared to the control group. However, more studies with longer follow-up periods are necessary to support our data.