Ventricular septal defect is the most common CHD, leading to pulmonary hypertension. Significantly lower 25-hydroxyvitamin D level was reported in children with CHD compared with healthy controls. The current study aimed to investigate the correlation between 25-hydroxyvitamin D level and pulmonary hypertension in children with ventricular septal defect.

A cross-sectional study was conducted on ventricular septal defect paediatric patients from January to June, 2019. Serum 25-hydroxyvitamin D was measured using electrochemiluminescence. Pulmonary hypertension was defined as mean pulmonary artery systolic pressure > 20 mmHg for children >3 months of age at sea level, measured by Doppler echocardiography.

From forty-four subjects, the majority of the subjects were female (56.8%) with normal nutritional status and perimembranous ventricular septal defect. Bivariate analysis showed that 25-hydroxyvitamin D level was associated with pulmonary hypertension (p < 0.01), type and size of ventricular septal defect (p = 0.02), and heart failure (p < 0.01). Higher 25-hydroxyvitamin D level was correlated with better nutritional status (p = 0.04, r = 0.26), and lower 25-hydroxyvitamin D level was correlated with the occurence of perimembranous ventricular septal defect (p = 0.01, r = −0.39), larger defect size (p < 0.01, r = −0.70), history of pneumonia (p = 0.02, r = −0.31), and heart failure (p < 0.01, r = −0.64). Subjects with 25-hydroxyvitamin D deficiency had prevalence ratio of 24.0 times for pulmonary hypertension. Higher pulmonary artery pressure was correlated to the occurence perimembranous ventricular septal defect (p = 0.01, r = 0.47), larger defect size (p < 0.01, r = 0.78), history of pneumonia (p = 0.01, r = 0.38), and heart failure (p < 0.01, r = 0.75).

Children with ventricular septal defect who had low 25-hydroxyvitamin D level posed a higher risk of having pulmonary hypertension.

The safe closure of atrial septal defect with deficient posterior-inferior or inferior vena cava rim is a controversial issue. Few studies have been conducted on the closure of atrial septal defect with deficient posterior-inferior or inferior vena cava rim without fluoroscopy. This study evaluated the feasibility and safety of echocardiography-guided transcatheter closure of atrial septal defect with deficient posterior-inferior or inferior vena cava rim.

The data of 136 patients who underwent transcatheter atrial septal defect closure without fluoroscopy from March 2017 to March 2020 were retrospectively analysed. The patients were classified into the deficient (n = 45) and sufficient (n = 91) posterior-inferior or inferior vena cava rim groups. Procedure and the follow-up results were compared between the two groups.

Atrial septal defect indexed diameter and the device indexed diameter in the deficient rim group were both larger than that in the sufficient rim group (22.12 versus 17.38 mm/m2, p < 0.001; 24.77 versus 21.21 mm/m2, p = 0.003, respectively). There was no significant difference in the success rate of occlusion between two groups (97.78% in the deficient rim group versus 98.90% in the sufficient rim group, p = 1.000). During follow-up, the incidence of severe adverse cardiac events was not statistically significant (p = 0.551).

Atrial septal defect with deficient posterior-inferior or inferior vena cava rim can safely undergo transcatheter closure under echocardiography alone if precisely evaluated with transesophageal or transthoracic echocardiography and the size of the occluder is appropriate. The mid-term results after closure are similar to that for an atrial septal defect with sufficient rim.

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.

While the efficacy and guidelines for implementation of rapid response systems are well established, limited information exists about rapid response paradigms for paediatric cardiac patients despite their unique pathophysiology.

With endorsement from the Paediatric Cardiac Intensive Care Society, we designed and implemented a web-based survey of paediatric cardiac and multidisciplinary ICU medical directors in the United States of America and Canada to better understand paediatric cardiac rapid response practices.

Sixty-five (52%) of 125 centres responded. Seventy-one per cent of centres had ∼300 non-ICU beds and 71% had dedicated cardiac ICUs. To respond to cardiac patients, dedicated cardiac rapid response teams were utilised in 29% of all centres (39% and 5% in centres with and without dedicated cardiac ICUs, respectively) [p = 0.006]. Early warning scores were utilised in 62% of centres. Only 31% reported that rapid response teams received specialised training. Transfers to ICU were higher for cardiac (73%) compared to generalised rapid response events (54%). The monitoring and reassessment of patients not transferred to ICU after the rapid response was variable. Cardiac and respiratory arrests outside the ICU were infrequent. Only 29% of centres formally appraise critical deterioration events (need for ventilation and/or inotropes post-rapid response) and 34% perform post-event debriefs.

Paediatric cardiac rapid response practices are variable and dedicated paediatric cardiac rapid response systems are infrequent in the United States of America and Canada. Opportunity exists to delineate best practices for paediatric cardiac rapid response and standardise practices for activation, training, patient monitoring post-rapid response events, and outcomes evaluation.

There are several studies reporting the outcomes of hypoplastic aortic arch and aortic coarctation repair with combination of techniques. However, only few studies reported of aortic arch and coarctation repair using a homograft patch through sternotomy and circulatory arrest with antegrade cerebral perfusion. We report our experience and outcomes of this cohort of neonates and infants.

We performed retrospective data collection for all neonates and infants who underwent aortic arch reconstruction between 2015 and 2020 at our institute. Data are presented as median and inter-quartile range (IQR).

The cohort included 76 patients: 49 were males (64.5%). Median age at operation was 16 days (IQR 9–43.25 days). Median weight was 3.5 kg (IQR 3.10–4 kg). There was no 30 days mortality. Three patients died in hospital after 30 days (3.95%), neurological adverse events occurred in only one patient (1.32%) and recurrent laryngeal nerve injury was noted in four patients (5.26%). Only three patients required the support of extracorporeal membrane oxygenation (ECMO) with a median ECMO run of 4 days. Median follow-up was 35 months (IQR 18.9–46.4 months); 5 years survival was 93.42% (n = 71). The rate of re-intervention on the aortic arch was 9.21% (n = 7).

Our experience shows excellent outcomes in repairing aortic arch hypoplasia with homograft patch under moderate to deep hypothermia with low in-hospital and 5 years mortality rates.

Paediatric cardiology fellows, tasked with studying a large and dynamic field, may benefit from a quick-access digital resource that reflects contemporary practice. The objective of this study was to evaluate the effectiveness of a paediatric cardiology handbook smartphone app in enhancing the accessibility of information such as guidelines and recommendations for paediatric cardiology fellows.

The Peds Cardiology Handbook app (iOS) was designed using XCode and Swift programming. A pre-app survey and download instructions were sent to the US paediatric cardiology fellows, followed by a post-app survey. Fellows were asked to rate the ease of rapid access to various types of information. Responses were compared pre- and post-app using McNemar’s test and Wilcoxon signed-rank test.

Two-hundred and thirty paediatric cardiology fellows were contacted; 23% (n = 53) completed both the pre- and post-app surveys and were included for analysis. After using the app, fellows found it easier to quickly access information in eight out of nine domains evaluated (p < 0.05). All fellows found the app easy to use (100%), most felt that the app was well-organised (98%), contained reliable information (92%), and was useful for finding quick answers to clinical questions (87%).

The Peds Cardiology Handbook iPhone app is a useful, reliable tool that provides quick access to high-yield information, including guidelines and references. Overall, paediatric cardiology fellows found it easier to rapidly access clinically relevant information after using the app. Future studies may be necessary to evaluate long-term use and impact on evidence-based practice.

Cardiopulmonary exercise test (CPET) allows quantification of functional capacity of patients with Fontan. The objective of this study was to determine the role of CPET parameters in predicting a higher maximum oxygen consumption (VO2 max) and to analyse the role of CPET parameters in predicting an unfavourable outcome.

A retrospective, cross-sectional, descriptive study was carried out on 57 patients with Fontan, who had undergone incremental CPET with cycloergometer between 2010 and 2020. Determinants of VO2 max and determinants of clinical deterioration were analysed.

In the univariate analysis, the variables significantly related to VO2 max were: age, sex, body mass index (BMI), years of Fontan evolution, intracardiac Fontan, oxygen consumption at anaerobic threshold (VO2AT), CO2 equivalents at anaerobic threshold (VE/VCO2) and chronotropic insufficiency. The multiple linear regression model that best fitted the relationship between VO2 max and independent variables (correlation coefficient 0.73) included sex (correlation index 3.35; p = 0.02), BMI (−0.27; p = 0.02), chronotropic failure (−2.79; p = 0.01) and VO2AT (0.92; p < 0.0001). In the univariate analysis of the prognostic CPET variables related to an unfavourable clinical situation, significance was only obtained with chronotropic insufficiency (p = 0.003). In multivariate analysis, chronotropic insufficiency maintains its association [p= 0.017, OR = 4.65 (1.3–16.5)].

In conclusion, together with the anthropometric parameters universally related to VO2 max, chronotropic insufficiency and VO2AT are the main determinants of functional capacity in patients with Fontan. Moreover, chronotropic insufficiency is closely related to unfavourable clinical evolution. Our data would support the intensive treatment of chronotropic insufficiency in order to improve the quality of life and the clinical situation of patients with Fontan.

Regular physical activity is safe and effective therapy for adults with CHD and is recommended by European Society of Cardiology guidelines. The COVID-19 pandemic poses enormous challenges to healthcare teams and patients when ensuring guideline compliance. We explored the implications of COVID-19 on physical activity levels in adult CHD patients.

A data-based questionnaire was distributed to adult CHD patients at a regional tertiary centre from October to November 2020.

Prior to the COVID-19 pandemic, 96 (79.3%) of 125 respondents reported participating in regular physical activity, with 66 (52.8%) meeting target levels (moderate physical activity for at least 150 minutes per week). Commonest motivations for physical activity were general fitness (53.6%), weight loss (36.0%), and mental health benefits (30.4%). During the pandemic, the proportion that met target levels significantly decreased from 52.8% to 40.8% (p = 0.03). The commonest reason was fear of COVID-19 (28.0%), followed by loss of motivation (23.2%) and gym/fitness centre closure (15.2%).

The COVID-19 pandemic has negatively impacted exercise levels of adult CHD patients. Most do not meet recommended physical activity levels, mainly attributable to fear of COVID-19. Even before the pandemic, only half of respondents met physical activity guidelines. Availability of online classes can positively impact exercise levels so could enhance guideline compliance. This insight into health perceptions and behaviours of adult CHD patients may help develop quality improvement initiatives to improve physical activity levels in this population.

The mini right axillary thoracotomy is an alternative surgical approach to repair certain congenital heart defects. Quality-of-life metrics and clinical outcomes in children undergoing either the right axillary approach or median sternotomy were compared.

Patients undergoing either approach for the same defects between 2018 and 2020 were included. Demographic details, operative data, and outcomes were compared between both groups. An abbreviated quality of life questionnaire based on the Infant/Toddler/Child Health Questionnaires focused on the patient’s global health, physical activity, and pain/discomfort was administered to all parents/guardians within two post-operative years.

Eighty-seven infants and children underwent surgical repair (right axillary thoracotomy, n = 54; sternotomy, n = 33) during the study period. There were no mortalities in either group. The right axillary thoracotomy group experienced significantly decreased red blood cell transfusion, intubation, intensive care, and hospital durations, and earlier chest tube removal. Up to 1 month, parents’ perception of their child’s degree and frequency of post-operative pain was significantly less after the right axillary thoracotomy approach. No difference was found in the patient’s global health or physical activity limitations beyond a month between the two groups.

With the mini right axillary approach, surrogates of faster clinical recovery and hospital discharge were noted, with a significantly less perceived degree and frequency of post-operative pain initially, but without the quality of life differences at last follow-up. While providing obvious cosmetic advantages, the minimally invasive right axillary thoracotomy approach for the surgical repair of certain congenital heart lesions is a safe alternative to median sternotomy.

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.

The Pediatric Acute Care Cardiology Collaborative (PAC3) was established to improve acute care cardiology outcomes through the development of an accurate and well-validated clinical registry. We report the validation results of the initial PAC3 registry audits and describe a novel regional audit format developed to accommodate a rapidly expanding membership facilitate collaborative learning and allow for necessary modification due to the COVID-19 pandemic.

Six hospitals were audited using a regional audit format and three hospitals were subsequently audited virtually. Critical and challenging-to-collect data elements were audited among at least 40 randomly selected cases. Discrepancies were categorised as either major or minor depending on their relative importance to patient outcomes and clinical care. Results were tabulated and reported.

We audited 386 encounters and 27,086 individual data fields across 9 hospitals. The aggregate overall accuracy rate was 99.27% and the aggregate major discrepancy rate was 0.51%. The overall accuracy rate ranged from 98.77% to 99.59%, and the major discrepancy rate ranged from 0.26% to 0.88% across the cohort. No appreciable difference was seen between audit formats. Both the regional and virtual audit methods were viewed favourably by participants.

A low data discrepancy rate was found demonstrating that the PAC3 registry is a highly accurate data source for use in quality improvement, benchmarking, and research. Regional audits and virtual audits were both successfully implemented.

Truncus arteriosus is a rare CHD. Neonatal and early infancy repair is recommended though some cases may present late. The aim of our study is to investigate the current results of truncus arteriosus repair and to analyse the differences in outcome and reintervention need between early versus late truncus arteriosus surgical repair.

In this cohort study, we reviewed all children who underwent truncus arteriosus repair from 2001 till 2021. We divided patients into two groups; early repair group including patients repaired at age less than 3 months and late repair group including patients who had repair at 3 months of age and later. We compared both groups for outcome variables.

Sixty-four children had truncus arteriosus repair including 48(75%) patients in early repair and 16(25%) patients in late repair groups. Peri-operative course was comparable between both groups. Post-surgery, we observed pulmonary hypertension in 6(12%) patients in early repair group comparing with 11(69%) patients in late repair group (p = 0.0001). In the last follow-up visit, pulmonary hypertension resolved in all early repair group patients while 6(37.5%) patients in late repair group continued to have pulmonary hypertension (p = 0.0001). Twenty-three(36%) patients required reintervention including 22(48%) in early repair group versus 1(6%) in late repair group (p = 0.007).

In general, the outcome of early truncus arteriosus repair is excellent with resolution of pulmonary hypertension following early repair. Late repair caries higher risk of persistent pulmonary hypertension (37.5%). About one-third of the patients who had truncus arteriosus repair will require re-intervention within 38±38.4 months after initial surgery.

The Pediatric and Congenital Electrophysiology Society (PACES) is a global organisation committed to the care of children and adults with CHD and arrhythmias.

To evaluate the global needs and potential inequities as it relates to cardiac implantable electronic devices.

ARROW (Assessment of Rhythm Resources arOund the World) is an online survey about cardiac implantable electronic devices, sent electronically to physicians within the field of Cardiology, Pediatric Cardiology, Electrophysiology and Pediatric Electrophysiology.

ARROW received 42 responders from 28 countries, 50% from low-/middle-income regions. The main differences between low-/middle- and high-income regions include availability of expertise on paediatric electrophysiology (50% versus 93%, p < 00.5) and possibility to perform invasive procedures (35% versus 93%, p < 0.005). Implant of devices in low-income areas relies significantly on patient’s resources (71%). The follow-up of the devices is on the hands of paediatric cardiologist/electrophysiologist in higher resources centres (93% versus 50%, p < 0.05).

The ARROW survey represents an initial assessment of the geographical characteristics in the field of Pediatric Electrophysiology. The next step is to make this “state of the art” more extensive to other aspects of the expertise. The relevance of collecting this data before the World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) in 2023 in Washington DC was emphasised in order to share the resulting information with the international community and set a plan of action to assist the development of arrhythmia services for children within developing regions of the world.

Coronary artery aneurysms are well-described in Kawasaki disease and the Multisystem Inflammatory Syndrome in Children and are graded using Z scores. Three Z score systems (Boston, Montreal, and DC) are widely used in North America. The recent Pediatric Heart Network Z score system is derived from the largest diverse sample to-date. The impact of Z score system on the rate of coronary dilation and management was assessed in a large real-world dataset.

Using a combined dataset of patients with acute Kawasaki disease from the Children’s Hospital at Montefiore and the National Heart, Lung, and Blood Institute Kawasaki Disease Study, coronary Z scores and the rate of coronary lesions (Z ≥ 2.0) and aneurysms (Z ≥ 2.5) were determined using four Z score systems. Agreement among Z scores and the effect on Kawasaki management were assessed.

Of 333 patients analysed, 136 were from Montefiore and 197 from the Kawasaki Disease Study. Age, sex, body surface area, and rate of coronary lesions did not differ between the samples. Among the four Z score systems, the rate of acute coronary lesions varied from 24 to 55%. The mean left anterior descending Z scores from Pediatric Heart Network and Boston had a large uniform discrepancy of 1.3. Differences in Z scores among the four systems may change anticoagulation management in up to 22% of a Kawasaki population.

Choice of Z score system alone may impact Kawasaki disease diagnosis and management. Further research is necessary to determine the ideal coronary Z score system.

This study aimed to determine the effect of donor-transmitted atherosclerosis on the late aggravation of cardiac allograft vasculopathy in paediatric heart recipients aged ≥7 years.

In total, 48 patients were included and 23 had donor-transmitted atherosclerosis (baseline maximal intimal thickness of >0.5 mm on intravascular ultrasonography). Logistic regression analyses were performed to identify risk factors for donor-transmitted atherosclerosis. Rates of survival free from the late aggravation of cardiac allograft vasculopathy (new or worsening cardiac allograft vasculopathy on following angiograms, starting 1 year after transplantation) in each patient group were estimated using the Kaplan–Meier method and compared using the log-rank test. The effect of the results of intravascular ultrasonography at 1 year after transplantation on the late aggravation of cardiac allograft vasculopathy, correcting for possible covariates including donor-transmitted atherosclerosis, was examined using the Cox proportional hazards model.

The mean follow-up duration after transplantation was 5.97 ± 3.58 years. The log-rank test showed that patients with donor-transmitted atherosclerosis had worse survival outcomes than those without (p = 0.008). Per the multivariate model considering the difference of maximal intimal thickness between baseline and 1 year following transplantation (hazard ratio, 22.985; 95% confidence interval, 1.948–271.250; p = 0.013), donor-transmitted atherosclerosis was a significant covariate (hazard ratio, 4.013; 95% confidence interval, 1.047–15.376; p = 0.043).

Paediatric heart transplantation recipients with donor-transmitted atherosclerosis aged ≥7 years had worse late cardiac allograft vasculopathy aggravation-free survival outcomes.