The citation history of a published article reflects its impact on the literature over time. We conducted a comprehensive bibliometric analysis to identify the most cited papers on CHD in children.

One-hundred and ninety journals listed in Journal Citation Reports were accessed via Web of Science. Publications with 250 or more citations were identified from Science Citation Index Expanded (1900–2020), and those relating to structural CHD in children were reviewed. Articles were ranked by citation count and the 100 most cited were analysed.

The number of citations ranged from 2522 to 309 (median 431, IQR 356–518), with 35 published since 2000. All were written in English, most originated from the United States (74%), and were published in cardiovascular journals, with Circulation (28%) the most frequent. There were 86 original research articles, including 50 case series, 14 cohort studies, and 10 clinical trials. The most cited paper was by Hoffman JI and Kaplan S on the incidence of CHD. Thirteen authors had 4 or more publications in the top 100, all of whom had worked in Boston, Philadelphia, San Francisco, or Dallas, and the most prolific author was Newburger JW (9 articles).

Citation analysis provides a historical perspective on scientific progress by assessing the impact of individual articles. Our study highlights the dominant position of US-based researchers and journals in this field. Most of the highly cited articles remain case series, with few randomised controlled trials in CHD appearing in recent years.

Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients.

From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]).

Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.

The aim of this study was to evaluate the safety and efficacy of the Cardi-O-Fix plug used for the treatment of muscular ventricular septal defects.

We retrospectively reviewed the medical records of five patients with muscular ventricular septal defects who underwent transcatheter closure using the Cardi-O-Fix Plug, from November 2017 to August 2019. The median age was 5.1 years (range: 3.2–6.5). Their median body weight was 18.1 kg (range: 13.4–21.8). All the patients underwent detailed two-dimensional Doppler and colour flow imaging by transthoracic echocardiography. The left ventricular median defect size of the muscular ventricular septal defects was 5.6 mm (range: 5.3–7.0). The right ventricular median defect size of the muscular ventricular septal defects was 3.9 mm (range: 3.3–4.7). All the procedures were performed on beating hearts.

All the patients underwent successful device implantation with no displacement or detachment, they have complete echocardiographic closure at the 1-year follow-up. There were no occluder-related arrhythmia, chordae tendineae rupture, tricuspid insufficiency, aortic regurgitation, haemolysis, or embolisation.

Application of the Cardi-O-Fix plug appears to be a feasible, safe, and effective treatment option for patients with muscular ventricular septal defects. Longer follow-up periods are warranted to prove the conclusion for long-term outcomes.

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.

To evaluate whether Kawasaki disease predisposes to premature atherosclerosis and to assess status of coronary artery abnormalities at least 10 years after diagnosis.

A prospective study was carried out on 21 patients who were diagnosed with Kawasaki disease at least 10 years back and are on regular follow-up. The study was conducted on 128 Slice Dual Source computed tomography scanner with electrocardiography-triggered radiation optimised protocols for assessment of coronary artery abnormalities and calcifications.

Study cohort had 21 subjects – 15 males and 6 females (age range: 11–23 years; mean: 15.76 + 3.72 years). Mean age at time of diagnosis was 3.21 + 2.48 years. Mean time interval from diagnosis of Kawasaki disease to computed tomography coronary angiography was 12.59 + 2.89 years. Four children had evidence of coronary artery abnormalities on transthoracic echocardiography at time of diagnosis. Of these, two had persistent abnormalities on computed tomography coronary angiography. One subject (4.76%) had coronary calcification that was localised to abnormal coronary artery segment. Four coronary artery abnormalities (one saccular; three fusiform aneurysms) were noted in two subjects.

Prevalence of coronary artery calcification is low and, if present, is localised to abnormal segments. This calcification is likely dystrophic rather than atherosclerotic. It appears that coronary artery abnormalities can persist for several years after acute episode of Kawasaki disease. Periodic follow-up by computed tomography coronary angiography is now a feasible non-invasive imaging modality for long term surveillance of patients with Kawasaki disease who had coronary artery abnormalities at time of diagnosis.

To assess the efficacy and safety of captopril, simvastatin, and L-carnitine as cardioprotective drugs in children with type 1 diabetes mellitus on different echocardiographic parameters, electrocardiographic parameter, lipid profile, and carotid intima–media thickness.

This randomised controlled trial was conducted on 100 children with type 1 diabetes mellitus for more than 3 years during the period from September 2018 to June 2020. Fifty healthy children of matched age and sex served as a control group. The patients were randomly assigned into four groups (25 children each): no-treatment group who received no cardioprotective drug, simvastatin group who received simvastatin (10–20 mg/day), captopril group who received captopril (0.2 mg/kg/day), and L-carnitine group who received L-carnitine (50 mg/kg/day) for 4 months. Lipid profile, serum troponin I, carotid intima–media thickness, and echocardiographic examinations were performed on all included children before and after the treatment.

Total cholesterol and low-density lipoprotein were significantly decreased in children who received simvastatin or L-carnitine. Triglycerides significantly decreased only in children who received simvastatin. High-density lipoprotein significantly increased in simvastatin and L-carnitine groups only. Serum troponin I decreased significantly in all the three treatment groups. Carotid intima–media thickness showed no significant change in all treatment groups. Echocardiographic parameters significantly improved in simvastatin, L-carnitine, and captopril groups.

Captopril, simvastatin, and L-carnitine have a significant beneficial effect on cardiac functions in children with type 1 diabetes mellitus. However, only simvastatin and L-carnitine have a beneficial effect on the lipid profile. The drugs were safe and well tolerated.

Clinical trial registration: The clinical trial was registered at www.clinicaltrial.gov (NCT03660293).

Total anomalous pulmonary venous connection is a rare cyanotic CHD that requires surgical repair. An unligated vertical vein after total anomalous pulmonary venous connection surgery may help to decrease the episodes of post-operative pulmonary hypertensive crises, low cardiac output syndrome, and mortality. The aim was to assess long-term outcome and our post-operative transcatheter vertical vein closure experiences in five patients with repaired total anomalous pulmonary venous connection patients.

A retrospective study was conducted in five cases with an unligated vertical vein following repair of supra-cardiac total anomalous pulmonary venous connection at our hospital from 2011 through 2018. Patients characteristics, cardiac catheterisation findings, surgical, and transcatheter procedural details were retrospectively analysed.

Transcatheter closure of the unligated vertical vein was technically successful in all the patients. Procedure-related complications were not observed in any of the patients. No long-term complication was found.

We suggest that transcatheter closure of the patent vertical vein is an effective and well-tolerated alternative to the surgical approach.

Balloon valvuloplasty and surgical aortic valvotomy have been the treatment mainstays for congenital aortic stenosis in children. Choice of intervention often differs depending upon centre bias with limited relevant, comparative literature.

This study aims to provide an unbiased, contemporary matched comparison of these balloon and surgical approaches.

Retrospective analysis of patients with congenital aortic valve stenosis who underwent balloon valvuloplasty (Queensland Children’s Hospital, Brisbane) or surgical valvotomy (Royal Children’s Hospital, Melbourne) between 2005 and 2016. Patients were excluded if pre-intervention assessment indicated ineligibility to either group. Propensity score matching was performed based on age, weight, and valve morphology.

Sixty-five balloon patients and seventy-seven surgical patients were included. Overall, the groups were well matched with 18 neonates/25 infants in the balloon group and 17 neonates/28 infants in the surgical group. Median age at balloon was 92 days (range 2 days – 18.8 years) compared to 167 days (range 0 days – 18.1 years) for surgery (rank-sum p = 0.08). Mean follow-up was 5.3 years. There was one late balloon death and two early surgical deaths due to left ventricular failure. There was no significant difference in freedom from reintervention at latest follow-up (69% in the balloon group and 70% in the surgical group, p = 1.0).

Contemporary analysis of balloon aortic valvuloplasty and surgical aortic valvotomy shows no difference in overall reintervention rates in the medium term. Balloon valvuloplasty performs well across all age groups, achieving delay or avoidance of surgical intervention.

Systemic ventricular end-diastolic pressure is important in patients with single ventricle heart disease. Predictors of an elevated systemic ventricular end-diastolic pressure prior to bidirectional Glenn operation have been incompletely identified.

All patients who underwent bidirectional Glenn operation operation at our centre between January 2007 and March 2017 were retrospectively identified and patient variables were extracted. For patients who had undergone Fontan operation at the time of this study, post-Fontan patient variables were also extracted.

One-hundred patients were included with a median age at pre-bidirectional Glenn operation catheterisation of 4.5 months. In total, 71 (71%) patients had a systemic right ventricle. At the pre-bidirectional Glenn operation catheterisation, the mean systemic ventricular end-diastolic pressure was higher amongst those with systemic right ventricle compared to left ventricle (9.1 mmHg ± 2.1 versus 7.7 ± 2.7 mmHg, p < 0.01). On univariate analysis, pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01), history of recoarctation (p = 0.03), history of Norwood operation (p = 0.04), and ventricular systolic pressure (p < 0.01). On multivariate analysis, systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01) and ventricular systolic pressure (p < 0.01). Amongst those who had undergone Fontan operation at the time of study (n = 49), those with a higher pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure were more likely to have experienced death, transplantation, or listed for transplantation (p = 0.02) and more likely to have had heart failure symptoms (p = 0.04) at a mean time from Fontan of 5.2 years ± 1.3.

In patients undergoing bidirectional Glenn operation operation, the volume-loaded, pre-bidirectional Glenn operation state may expose diastolic dysfunction that has prognostic value.

We aimed to investigate the effect of Celiac disease on myocardial functions and aortic elasticity parameters.

Thirty children with Celiac disease and 30 healthy children were enrolled in the study. Both the groups were similar in terms of age and gender. Cardiac functions of all children in the patients and control group were evaluated using conventional transthoracic echocardiography and tissue Doppler imaging. Aortic strain, distensibility, and stiffness index were calculated by M-mode echocardiography.

The demographic findings, height, weight, and body mass index of cases were similar among two groups. No statistical difference was found between E wave velocity for conventional transthoracic echocardiography and tissue Doppler imaging measurements of the mitral valve; early diastolic flow peak velocity, A wave velocity; late diastolic flow peak velocity; and E/A ratio. Isovolumetric relaxation time and isovolumetric contraction time ratios were statistically different between the groups (p = 0.000, p = 0.000, p = 0.000). The myocardial performance index calculated according to the pulse Doppler measurement results was found to be statistically different between the groups (p = 0.000). There was no statistical difference between the groups in terms of aortic strain, distensibility, and stiffness index.

In this study, both conventional transthoracic echocardiography and tissue Doppler imaging revealed the affection of the myocardial functions during systole and diastole in children with Celiac disease. Therefore, early follow-up and routine cardiac evaluation of celiac patients may be appropriate due to the increased risk of cardiac affection.

The objective of this study was to delineate the characteristics and incidence of congenital heart disease (CHD) in patients with isolated microtia and to determine whether the prevalence of CHD among patients with isolated microtia increases with the severity of microtia.

A total of 804 consecutive patients had a pre-operative colour Doppler echocardiographic examination. A retrospective study was performed with the clinical and imaging data from November, 2017 to January, 2019. The χ2 test was performed to analyse the interaction between isolated microtia and CHD.

With the colour Doppler echocardiographic examination’s data from 804 consecutive isolated microtia patients, we found CHD, including atrial septal defect, ventricular septal defect, tetralogy of Fallot, patent ductus arteriosus, and others, occurred in 52 of 804 patients (6.5%). Atrial septal defect prevalence in patients with isolated microtia was significantly higher than ventricular septal defect (24/804 versus 11/804, p < 0.05) and patent ductus arteriosus (24/804 versus 2/804, p < 0.001). Ventricular septal defect prevalence in patients with isolated microtia was significantly higher than patent ductus arteriosus (11/804 versus 2/804, p < 0.05). All four types of microtia (concha-type microtia, small concha-type microtia, lobule-type microtia, and anotia) had similar incidences of CHD with no difference in the incidences among these types (p > 0.05 respectively). Furthermore, there was no significant difference in the incidence of the atrial septal defect among the four subtypes (p > 0.05 respectively). Similarly, ventricular septal defect and patent ductus arteriosus also showed no differences (p > 0.05 respectively).

The overall incidences of CHD and three most common CHD subtypes (atrial septal defect, ventricular septal defect, and patent ductus arteriosus) in patients with isolated microtia are higher than general population. The prevalence of CHD among patients with isolated microtia does not increase with the severity of microtia. According to our experience in this study, we suggest colour Doppler echocardiographic imaging should be performed for isolated microtia patients soon after birth if possible. Furthermore, for the plastic surgeon and anaesthesiologist, it is important to take pre-operative colour Doppler echocardiographic images which can help evaluate heart function to ensure the safety of the peri-operative period. Future studies when investigating CHDs associated with isolated microtia could focus on genetic and molecular mechanisms.

Progress in the management of complex congenital heart disease (CHD) led to an improvement in survival rates of adults with a Fontan-like circulation. The objective of this study was to assess the subjective health status and quality of life of this population.

Patients aged more than 18 years at the time of the study, who underwent a Fontan-like procedure. Subjective health status was assessed by the SF-36 questionnaire and a linear analog scale was used to score patients’ self-perception of their quality of life; cardiac and demographic parameters were collected.

Among 65 eligible patients, 60 (23 females; mean ± SD age: 25.7 ± 7.2 years) answered the SF-36 questionnaire and 46 of these were interviewed to evaluate their perceived quality of life. Among them, 20 (33.3%) were working full-time and 21 (35%) experienced arrhythmias. The physical SF-36 scores were lower in patients than in the general population (p ≤ 0.05). The New York Hear Association (NYHA) class and occupation were correlated with SF-36 scores of physical activity (respectively, p = 0.0001 and p = 0.025). SF-36 scores of psychological status were associated with the number of drugs and occupation (respectively, p = 0.0001 and p = 0.02). The mean ± SD quality of life score measured using a linear analog scale was 7.02 ± 1.6 and was linked to education and occupation (p ≤ 0.05) but not with cardiac parameters.

Adult Fontan patients perceive an impaired physical health but report a good overall quality of life. Education and occupation impacts significantly on Fontan patients’ quality of life.

We aimed to assess the immediate haemodynamic response and the timing of cardiac remodelling in paediatric secundum atrial septal defect patients who underwent percutaneous transcatheter closure.

In this longitudinal cohort study with 41 paediatric secundum ASD patients who underwent PTC with Amplatzer Occluder device were assessed for immediate post-interventional haemodynamic response measured by catheterisation and was evaluated for right and left cardiac remodelling during a follow-up period of 12 months by transthoracic echocardiography. SPSS 20.0 was used for statistical analyses of pre- and post-interventional invasive haemodynamic parameters of the patients, and pre- and post-interventional TTE data compared with the values of the control group consisted of 39 healthy children.

The mean diameter of ASD was 13.9 ± 4.7 mm. PTC intervention in all patients completed with 100% success and 0% complication rates. All invasive haemodynamic data, except the ratio of pulmonary resistance to systemic resistance, significantly reduced after PTC (p < 0.05). TTE and PW Doppler revealed that right and left cardiac remodelling started as soon as the post-interventional 24th hour and completed in the 12th month.

This study with a very high interventional success rate can be counted as the first example of research on the haemodynamic response and timing of cardiac remodelling after PTC of secundum ASD in children. We suggest that future multicentric studies with larger cohorts and a comprehensive methodology like ours with longer follow-up periods would better serve to further assess the cardiac remodelling in children after PTC of secundum ASD.

Screening for congenital heart disease (CHD) in school students is well-established in high-income countries; however, data from low-to-middle-income countries including Indonesia are limited.

This study aimed to evaluate CHD screening methods by cardiac auscultation and 12-lead electrocardiogram to obtain the prevalence of CHD, confirmed by transthoracic echocardiography, among Indonesian school students.

We conducted a screening programme in elementary school students in the Province of Special Region of Yogyakarta, Indonesia. The CHD screening was integrated into the annual health screening. The trained general practitioners and nurses participated in the screening. The primary screening was by cardiac auscultation and 12-lead electrocardiogram. The secondary screening was by transthoracic echocardiography performed on school students with abnormal findings in the primary screening.

A total of 6116 school students were screened within a 2-year period. As many as 329 (5.38%) school students were detected with abnormalities. Of those, 278 students (84.49%) had an abnormal electrocardiogram, 45 students (13.68%) had heart murmurs, and 6 students (1.82%) had both abnormalities. The primary screening programme was successfully implemented. The secondary screening was accomplished for 260 school students, and 18 students (6.9%) had heart abnormalities with 7 (2.7%) who were confirmed with septal defects and 11 (4.2%) had valve abnormalities. The overall prevalence was 0.29% (18 out of 6116).

The primary screening by cardiac auscultation and 12-lead electrocardiogram was feasible and yielded 5.38% of elementary school students who were suspected with CHD. The secondary screening resulted in 6.9% confirmed cardiac abnormalities. The cardiac abnormality prevalence was 0.29%.

It is difficult to predict the complications and prognosis of ECMO, which is gaining widespread use in patients with pediatric surgery. Platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR) are emerging inflammatory markers that can be calculated from complete blood count, which is a cheap and easily accessible laboratory analysis. The ratios between cellular elements in peripheral blood have been demonstrated to provide information on inflammation, infection, and immune response.

Sixty-seven patients who needed ECMO application after undergoing pediatric cardiovascular surgery in our clinic, between May 2005 and April 2020, were included in this study. The age of patients varied between 4 days and 17 years with a mean of 30.59 ± 147.26 months.

The relationships between PLR or NLR values and various blood parameters and blood gas results were found to be statistically nonsignificant in our group of pediatric ECMO recipients. Even if the effect of PLR and NLR values on mortality and prognosis is statistically nonsignificant in patients who need ECMO after congenital heart surgery, PLR and NLR are typically elevated in the postoperative period. An increase in these values above a certain threshold may be a statistically significant indicator for the prediction of mortality.

There are few studies in the literature concerning PLR and NLR values in patients with pediatric heart surgery. We consider this study will make way for new studies in the future.

Subclinical atherosclerosis in childhood can be evaluated by carotid intima-media thickness, which is considered a surrogate marker for atherosclerotic disease in adulthood. The aims of this study were to evaluate carotid intima-media thickness and, to investigate associated factors.

Cross-sectional study with children and adolescents with congenital heart disease (CHD). Socio-demographic and clinical characteristics were assessed. Subclinical atherosclerosis was evaluated by carotid intima-media thickness. Cardiovascular risk factors, such as physical activity, screen time, passive smoke, systolic and diastolic blood pressure, waist circumference, dietary intake, lipid parameters, glycaemia, and C-reactive protein, were also assessed. Factors associated with carotid intima-media thickness were analysed using multiple logistic regression.

The mean carotid intima-media thickness was 0.518 mm and 46.7% had subclinical atherosclerosis (carotid intima-media thickness ≥ 97th percentile). After adjusting for confounding factors, cyanotic CHD (odds ratio: 0.40; 95% confidence interval: 0.20; 0.78), cardiac surgery (odds ratio: 3.17; 95% confidence interval: 1.35; 7.48), and be hospitalised to treat infections (odds ratio: 1.92; 95% confidence interval: 1.04; 3.54) were associated with subclinical atherosclerosis.

Clinical characteristics related to CHD were associated with subclinical atherosclerosis. This finding suggests that the presence of CHD itself is a risk factor for subclinical atherosclerosis. Therefore, the screen and control of modifiable cardiovascular risk factors should be made early and intensively to prevent atherosclerosis.

In resource limited settings, children with cardiac disease present late, have poor outcomes and access to paediatric cardiology programmes is limited. Cardiac point of care ultrasound was introduced at several Médecins Sans Frontières sites to facilitate cardiopulmonary assessment. We describe the spectrum of disease, case management and outcomes of cases reviewed over the Telemedicine platform.

Previously ultrasound naïve, remotely placed clinical teams received ultrasound training on focussed image acquisition. The Médecins Sans Frontières Telemedicine platform was utilised for remote case and imaging review to diagnose congenital and acquired heart disease and guide management supported by a remotely situated paediatric cardiologist.

Two-hundred thirty-three cases were reviewed between 2016 and 2018. Of 191 who underwent focussed cardiac ultrasound, diagnoses included atrial and ventricular septal defects 11%, atrioventricular septal defects 7%, Tetralogy of Fallot 9%, cardiomyopathy/myocarditis 8%, rheumatic heart disease 8%, isolated pericardiac effusion 6%, complex congenital heart disease 4% and multiple other diagnoses in 15%. In 17%, there was no identifiable abnormality while 15% had inadequate imaging to make a diagnosis. Cardiologist involvement led to management changes in 75% of cases with a diagnosis. Mortality in the entire group was disproportionately higher among neonates (38%, 11/29) and infants (20%, 16/81). There was good agreement on independent review of selected cases between two independent paediatric cardiologists.

Cardiac point of care ultrasound performed by remote clinical teams facilitated diagnosis and influenced management in cases reviewed over a Telemedicine platform. This is a feasible method to support clinical care in resource limited settings.