As a result of medical advances, the adult congenital heart disease population is rapidly expanding. Nonetheless, most patients remain prone to increased morbidity and mortality. Therefore, long-term medical resource use is required. This systematic review aims to present the trends over the past decades of medical resource utilization in adult congenital heart disease as well as its current status, with a focus on hospitalizations, emergency department visits, outpatient cardiology visits, and visits to other healthcare professionals.

MEDLINE (Pubmed), Embase, and Web of Science were searched for retrospective database research publications. The ISPOR checklist for retrospective database research was used for quality appraisal. Trends over time are explored.

Twenty-one articles met the inclusion criteria. All but one of the studies was conducted in Western Europe and North America. The absolute number of hospitalizations has been increasing over the last several decades. This increase is highest in patients with mild lesions, although these numbers are largely driven by hospitalizations of patients with an atrial septal defect or a patent foramen ovale. Meanwhile, outpatient cardiology visits are increasing at an even higher pace, and occur most often in geriatric patients and patients with severe lesions. Conversely, the number of hospitalizations per 100 patients is decreasing over time. Literature is scarce on other types of healthcare use.

A strong rise in healthcare utilization is noticed, despite the mitigating effect of improved efficiency levels. As the population continues to grow, innovative medical management strategies will be required to accommodate its increasing healthcare utilization.

This research investigated patients who underwent surgery for a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents.

The medical records of 11 patients who underwent aortic surgery for dilatation resulting from a connective tissue disease or inflammatory vasculitis between 2000 and 2017 were retrospectively reviewed.

The median age and body weight of the patients were 9.6 years (range 5.4 months–15.5 years) and 25.8 kg (range 6.8–81.5), respectively. The associated diseases were Marfan syndrome (n = 3), Loeys-Dietz syndrome (n = 3), Kawasaki disease (n = 1), Takayasu arteritis (n = 1), PHACE syndrome (n = 1), tuberous sclerosis (n = 1), and unknown (n = 1). The most common initially affected area was the ascending aorta. During the 66.4 ± 35.9 months of follow-up, two Marfan syndrome patients died, and four patients (one Marfan syndrome and three Loeys-Dietz syndrome) had repeated aortic operation. Except for one patient, the functional class was well maintained in all patients who were followed up.

Cases of surgical treatment for a dilated aorta associated with a connective tissue disease and inflammatory vasculitis are rare in children and adolescents at our institution. Most of the patients in this study showed a tolerable postoperative course. However, the aorta showed progressive dilation over time even after surgical treatment, especially in patients with Loeys-Dietz syndrome. In these patients, close and more frequent regular follow-up is required.

There is limited experience about transcatheter closure of doubly committed subarterial ventricular septal defects with Amplatzer ductal occluder.

Between March, 2015 and July, 2017, a total of 22 patients with doubly committed subarterial ventricular septal defects received transcatheter closure using Amplatzer ductal occluder and underwent clinical follow-up for at least 6 months.

Device implantation was finally successful in 21 (95.4%) patients despite failed occlusion in one patient and intra-procedural replacement of unsuitable occluders in four (19.0%) patients. In mean 12.3 months of follow-up, there were no major complications (death, aortic valve or sinus rupture, device dislocation or embolisation, grade 2 new-onset aortic regurgitation, etc.), resulting in clinical occlusion success of 95.4%. Mechanical haemolysis occurred in one patient and resolved with medication. Residual shunt was observed in 11 (52.4%) patients (9 mild, 2 moderate-severe) post-procedurally, 14 (66.7%) patients (12 mild, 2 moderate-severe) in hospital stay, and 2 (9.5%) patients (2 mild, 0 moderate-severe) at the last follow-up. Device-induced new-onset aortic regurgitation was found in nine (42.8%) patients (9 mild, 0 moderate-severe) post-procedurally and in hospital stay, which was resolved in two (9.5%) patients and unchanged in seven (33.3%) patients at the last follow-up. Another four (19.0%) patients newly developed mild aortic regurgitation during follow-up.

Transcatheter closure of doubly committed subarterial ventricular septal defects with Amplatzer ductal occluder is technically feasible in the selected patients. However, further study is needed to confirm its long-term clinical outcomes.

Although sleep apnea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with congenital heart disease are limited.

In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia. The prevalence, characteristics, and risk factors of sleep apnea using a type-3 portable overnight polygraph in adult patients with congenital heart disease were evaluated.

There were 104 patients [median age: 36 (interquartile range: 28–48) years] who were admitted for heart failure 34% (n = 36), diagnostic catheterisation 26% (n = 27), interventional catheterisation 18% (n = 19), or arrhythmia 22% (n = 23). The prevalence of sleep apnea, defined as a respiratory disturbance index ≥5, was 63% (n = 63), with a distribution of 37%, 16%, and 10% for mild (5≤ respiratory disturbance index <15), moderate (15≤ respiratory disturbance index <30), and severe (respiratory disturbance index ≥30) sleep apnea, respectively. A large majority of the sleep apnea cases were categorised as obstructive sleep apnea (92%, n = 58). The respiratory disturbance index ≥15 group had a significantly higher proportion of male patients and higher body mass index, noradrenaline level, and aortic blood pressure than the group without sleep apnea (respiratory disturbance index <5). Multivariable analysis showed that New York Heart Association class ≥II (OR, 4.36; 95% CI, 1.09–20.87) and body mass index ≥25 (OR, 4.29; 95% CI, 1.32–15.23) were independent risk factors for a respiratory disturbance index ≥15.

Our results showed a high prevalence of sleep apnea in adult patients with congenital heart disease. Its unique haemodynamics may be associated with a high prevalence of sleep apnea. Congestive heart failure and being overweight are important risk factors for sleep apnea. Management of heart failure and general lifestyle improvements will be important for controlling sleep apnea symptoms in these patients.

To analyse the feasibility and effectiveness in humanitarian practice of surgical management of children with single-ventricle heart condition.

Retrospective study of children with a single ventricle, managed by the association Mécénat-Chirurgie Cardiaque since 1996, with long-term follow-up after their return home.

Of the 138 children in our cohort, 119 had one or more surgeries (180 procedures): palliative surgery alone (systemic-pulmonary anastomosis or banding), 41; partial cavo-pulmonary connection, 47; total cavo-pulmonary connection (mean age 8.5 years), 31. Operative mortality is 5.5%. After a mean follow-up of 5.6 years, 18 children (13%) were lost to follow-up. Survival at 10 years is 79% in children receiving surgery (palliative only, 72%; partial cavo-pulmonary connection, 77%; total cavo-pulmonary connection, 97%) versus 29% in children with no surgical intervention. The prognosis is better for tricuspid atresia and double-inlet left ventricle (86 and 83% survival at 10 years) than for double-outlet right ventricle or complete atrio-ventricular canal defect (64 and 68% at 5 years).

The surgery of the single ventricle in humanitarian medicine allows a very satisfactory survival after one or more surgeries tending towards a total cavo-pulmonary connection as soon as possible.

Enteral sildenafil may be used in the intensive care unit for treatment of pulmonary arterial hypertension. We aimed to determine if initial enteral sildenafil dosing is safe in children receiving concurrent vasoactive infusions.

We performed a single-centre retrospective chart review that included patients less than 2 years of age in paediatric and cardiovascular intensive care units at an academic medical centre from 1 January, 2010 to 30 November, 2016. Included patients received concomitant enteral sildenafil and a continuously infused vasoactive agent. Exclusion criteria consisted of mechanical circulatory support, any form of dialysis, or a suspicion of septic shock at the time of sildenafil initiation. We sought to identify patients who developed worsening hemodynamic instability after initiation of enteral sildenafil defined as one or more of the following observations within 24 hours of sildenafil initiation: sildenafil discontinuation, total fluid bolus receipt >10 ml/kg, increased vasoactive support, epinephrine intravenous push administration, and/or the initiation of mechanical circulatory support.

Worsening hemodynamic instability was identified in 35% of the 130-patient cohort. Patients younger than 4 months were at increased risk of further hemodynamic instability compared with older patients (56% versus 44%, p = 0.0003) despite receiving lower median doses (1.28 mg/kg/day versus 1.78 mg/kg/day, p = 0.01).

Critically ill children receiving vasoactive infusions may be at increased risk for further hemodynamic instability after initiation of enteral sildenafil, particularly in younger patients. This population may benefit from lower starting enteral sildenafil doses of 0.25 mg/kg/dose or less every 8 hours to avoid further hemodynamic compromise.

Children with congenital heart disease are at high risk for malnutrition. Standardisation of feeding protocols has shown promise in decreasing some of this risk. With little standardisation between institutions’ feeding protocols and no understanding of protocol adherence, it is important to analyse the efficacy of individual aspects of the protocols.

Adherence to and deviation from a feeding protocol in high-risk congenital heart disease patients between December 2015 and March 2017 were analysed. Associations between adherence to and deviation from the protocol and clinical outcomes were also assessed. The primary outcome was change in weight-for-age z score between time intervals.

Increased adherence to and decreased deviation from individual instructions of a feeding protocol improves patients change in weight-for-age z score between birth and hospital discharge (p = 0.031). Secondary outcomes such as markers of clinical severity and nutritional delivery were not statistically different between groups with high or low adherence or deviation rates.

High-risk feeding protocol adherence and fewer deviations are associated with weight gain independent of their influence on nutritional delivery and caloric intake. Future studies assessing the efficacy of feeding protocols should include the measures of adherence and deviations that are not merely limited to caloric delivery and illness severity.

Late Fontan survivors are at high risk to experience heart failure and death. Therefore, the current study sought to investigate the role of non-invasive diagnostics as prognostic markers for failure of the systemic ventricle following Fontan procedure.

This monocentric, longitudinal observational study included 60 patients with a median age of 24.5 (19–29) years, who were subjected to cardiac magnetic resonance imaging, echocardiography, cardiopulmonary exercise testing, and blood analysis. The primary endpoint of this study was decompensated heart failure with symptoms at rest, peripheral and/or pulmonary edema, and/or death.

During a follow-up of 24 months, 5 patients died and 5 patients suffered from decompensated heart failure. Clinical (NYHA class, initial surgery), functional (VO2 peak, ejection fraction, cardiac index), circulating biomarkers (N-terminal pro brain natriuretic peptide), and imaging parameters (end diastolic volume index, end systolic volume index, mass-index, contractility, afterload) were significantly related to the primary endpoint. Multi-variate regression analysis identified afterload as assessed by cardiac magnetic resonance imaging as an independent predictor of the primary endpoint (hazard ratio 1.98, 95% confidence interval 1.19–3.29, p = 0.009).

We identified distinct parameters of cardiopulmonary exercise testing, cardiac magnetic resonance imaging, and blood testing as markers for future decompensated heart failure and death in patients with Fontan circulation. Importantly, our data also identify increased afterload as an independent predictor for increased morbidity and mortality. This parameter is easy to assess by non-invasive cardiac magnetic resonance imaging. Its modulation may represent a potential therapeutic approach target in these high-risk patients.

Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/MAPCAs) are at risk for post-operative respiratory complications after undergoing unifocalisation surgery. Thus, we assessed and further defined the incidence of airway abnormalities in our series of over 500 children with TOF/MAPCAs as determined by direct laryngoscopy, chest computed tomography (CT), and/or bronchoscopy.

The medical records of all patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery from March, 2002 to June, 2018 were reviewed. Anaesthesia records, peri-operative bronchoscopy, and/or chest CT reports were reviewed to assess for diagnoses of abnormal or difficult airway. Associations between chromosomal anomalies and airway abnormalities – difficult anaesthetic airway, bronchoscopy, and/or CT findings – were defined.

Of the 564 patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery at our institution, 211 (37%) had a documented chromosome 22q11 microdeletion and 28 (5%) had a difficult airway/intubation reported at the time of surgery. Chest CT and/or peri-operative bronchoscopy were performed in 234 (41%) of these patients. Abnormalities related to malacia or compression were common. In total 35 patients had both CT and bronchoscopy within 3 months of each other, with concordant findings in 32 (91%) and partially concordant findings in the other 3.

This is the largest series of detailed airway findings (direct laryngoscopy, CT, and bronchoscopy) in TOF/MAPCAS patients. Although these findings are specific to an at-risk population for airway abnormalities, they support the utility of CT and /or bronchoscopy in detecting airway abnormalities in patients with TOF/MAPCAs.

Respiratory syncytial virus infection is the most frequent cause of acute lower respiratory tract disease in infants. A few reports have suggested that pulmonary hypertension is associated with increased severity of respiratory syncytial virus infection. We sought to determine the association between the pulmonary hypertension detected by echocardiography during respiratory syncytial virus bronchiolitis and clinical outcomes.

We retrospectively reviewed 154 children admitted with respiratory syncytial virus bronchiolitis who had an echocardiography performed during the admission. The association between pulmonary hypertension and clinical outcomes including mortality, intensive care unit (ICU) admission, prolonged ICU stay (>10 days), tracheal intubation, and need of high frequency oscillator ventilation was evaluated.

Echocardiography detected pulmonary hypertension in 29 patients (18.7%). Pulmonary hypertension was observed more frequently in patients with congenital heart disease (CHD) (n = 11/33, 33%), chronic lung disease of infancy (n = 12/25, 48%), prematurity (<37 weeks gestational age, n = 17/59, 29%), and Down syndrome (n = 4/10, 40%). The presence of pulmonary hypertension was associated with morbidity (p < 0.001) and mortality (p = 0.02). However, in patients without these risk factors (n = 68), pulmonary hypertension was detected in five patients who presented with shock or poor perfusion. Chronic lung disease was associated with pulmonary hypertension (OR = 5.9, 95% CI 2.2–16.3, p = 0.0005). Multivariate logistic analysis demonstrated that pulmonary hypertension is associated with ICU admission (OR = 6.4, 95% CI 2.2–18.8, p = 0.0007), intubation (OR = 4.7, 95% CI 1.8–12.3, p = 0.002), high frequency oscillator ventilation (OR = 8.4, 95% CI 2.95–23.98, p < 0.0001), and prolonged ICU stay (OR = 4.9, 95% CI 2.0–11.7, p = 0.0004).

Pulmonary hypertension detected by echocardiography during respiratory syncytial virus infection was associated with increased morbidity and mortality. Chronic lung disease was associated with pulmonary hypertension detected during respiratory syncytial virus bronchiolitis. Routine echocardiography is not warranted for previously healthy, haemodynamically stable patients with respiratory syncytial virus bronchiolitis.

To analyse the relationship of altered birth weight with metabolic and cardiovascular outcomes among adolescents, as well as to identify if sports participation is able to attenuate or even eliminate the impact of birth weight on health outcomes.

Cross-sectional study (Analysis of Behaviours of Children During Growth [ABCD Growth Study]). Adolescents with age ranging from 11 to 18 years old (14.7±2.1) stratified according to normal (n = 230) and altered (n = 35) birth weight composed the sample. Birth weight was self-reported by adolescent’s parents. Sports participation was assessed by face-to-face interview. Carotid intima–media thickness (CIMT) and femoral intima–media thickness (FIMT) were measured using an ultrasound device. C-reactive protein levels were used to assess the inflammatory status. Blood pressure, Z score of metabolic risk (dyslipidemia and glucose), adiposity, and insulin resistance were covariates.

In the crude model, FIMT (p value = 0.037) and C-reactive protein (p value = 0.029) were affected by altered birth weight. In the adjusted models, altered birth weight affected FIMT (p value = 0.048; small effect size of 1.7%), independently of sports participation. For C-reactive protein, previous time of engagement in sports (p value = 0.001; small effect size of 4.8%) affected C-reactive protein, independently of birth weight.

Vascular structure seems to be affected by birth weight in adolescents, while its impact on inflammation seems to be attenuated by the regular engagement in sports.

Atrial septal defect is the third most common CHD. A hemodynamically significant atrial septal defect causes volume overload of the right side of the heart. Preterm children may suffer from both pulmonary and cardiac comorbidities, including altered myocardial function. The aim of this study was to compare the rate of adverse events following atrial septal defect closure in preterm- and term-born children.

We performed a retrospective cohort study including children born in Sweden, who had a surgical or percutaneous atrial septal defect closure at the children’s hospitals in Lund and Stockholm, between 2000 and 2014, assessing time to the first event within 1 month or 1 year. We analysed differences in the number of and the time to events between the preterm and term cohort using the Kaplan–Meier survival curve, a generalised model applying zero-inflated Poisson distribution and Gary-Anderson’s method.

Overall, 413 children were included in the study. Of these, 93 (22.5%) were born prematurely. The total number of adverse events was 178 (110 minor and 68 major). There was no difference between the cohorts in the number of events, whether within 1 month or within a year, between major (p = 0.69) and minor (p = 0.84) events or frequencies of multiple events (p = 0.92).

Despite earlier procedural age, larger atrial septal defects, and higher comorbidity than term children, preterm children appear to have comparable risk for complications during the first year after surgical or percutaneous closure.

In patients after atrioventricular septal defect correction, altered geometry leads to a changed position and subsequent flow over the left ventricular outflow tract. We hypothesised that this altered flow may influence haemodynamics in the ascending aorta.

In total, 30 patients after atrioventricular septal defect correction (age 27.6 ± 12.8 years) and 28 healthy volunteers (age 24.8 ± 13.7 years) underwent 4D flow cardiovascular magnetic resonance. Left ventricular ejection fraction and mean and peak wall shear stress calculated at ascending aortic peak systole were obtained from cardiovascular magnetic resonance. Left ventricular outflow tract data including velocity and diameter were obtained from echocardiography.

Patients showed a higher mean (911 ± 173 versus 703 ± 154 mPa, p = 0.001) and peak ascending aortic wall shear stress (1264 ± 302 versus 1009 ± 240 mPa, p = 0.001) compared to healthy volunteers. Increased blood flow velocities over the left ventricular outflow tract (1.49 ± 0.30 m/s versus 1.22 ± 0.20 m/s, p < 0.001) correlated well with mean and peak ascending aortic wall shear stress (r = 0.67, p < 0.001 and r = 0.77, p < 0.001).

After atrioventricular septal defect correction, increased wall shear stress was observed, which correlated to velocities over the left ventricular outflow tract. These findings imply that altered outflow tract geometry contributes to changed aortic haemodynamics.

To assess the effectiveness of radiofrequency catheter ablation for lone atrial fibrillation in young adults.

This single-centre, retrospective, observational study enrolled 75 consecutive patients (86.7% men) under 35 (median, 30) years old with lone atrial fibrillation (68% paroxysmal, 26.7% persistent, and 5.3% long-standing persistent) without other cardiopulmonary diseases who underwent catheter ablation between April 2009 and May 2017. Procedural endpoints were circumferential pulmonary vein ablation for atrial fibrillation with pulmonary vein trigger, and target ablation or bidirectional block of lines and disappearance of complex fractionated atrial electrograms for atrial fibrillation with clear and unclear non-pulmonary vein triggers, respectively.

Main study outcome was rate of survival free from atrial tachyarrhythmia recurrence, which at median 61 (range, 5–102) months follow-up was 62.7% (64.7 and 58.3% for paroxysmal and non-paroxysmal atrial fibrillation, respectively) after single ablation, and 69.3% (68.6 and 70.8% for paroxysmal and non-paroxysmal atrial fibrillation, respectively) after mean 1.2 ablations (two and three ablations in 11 and 2 patients, respectively). In multivariate analysis, non-pulmonary vein trigger was a significant independent predictor of recurrent atrial tachyarrhythmia (OR, 10.60 [95%CI, 2.25–49.96]; p = 0.003). There were no major periprocedural adverse events.

In patients under 35 years old with lone atrial fibrillation, radiofrequency catheter ablation appeared effective particularly for atrial fibrillation with pulmonary vein trigger and regardless of left atrial size or atrial fibrillation duration or type. Atrial tachyarrhythmia recurrence after multiple ablations warrants further study.

Two-stage arterial switch operation and left ventricle retraining are necessary for the patients with left ventricle dysfunction and transposition of great vessels with intact ventricular septum (TGA-IVS) who are referred late.

Forty-seven patients with the diagnosis of TGA-IVS and left ventricle dysfunction who underwent arterial switch operation in our centre between July 2013 and August 2017 were analysed retrospectively. The inclusion criteria for left ventricle retraining were patients older than 2 months of age at presentation, having an echocardiographic left ventricle mass index of less than 35 g/m², and having an echocardiographic “banana-shaped” left ventricle geometric appearance. The patients were divided into two groups: pulmonary artery banding and Blalock Taussig shunt were performed as the initial surgical procedure for later arterial switch operation in Group I (n = 19) and pulmonary artery banding and bidirectional cava-pulmonary shunt in Group 2 (n = 28).

The average age was found to be 122.3 ± 45.6 days in Group I and 145.9 ± 37.2 days in Group II. There was no statistically significant difference (p = 0.232 versus p = 0.373) between the average left ventricle mass index of the two groups neither before the first stage nor the second stage (26.6 ± 4.8 g/m² versus 25.0 ± 4.9 g/m² and 70.5 ± 12 g/m² versus 673.8 ± 12.0 g/m², respectively). The average time interval for the left ventricle to retrain was 97.7 ± 42.9 days for Group I and 117.3 ± 40.3 days for Group II, significantly lower in Group I (p = 0.027). The time spent in ICU, length of the period during which inotropic support was required, and the duration of hospital stay were significantly higher in Group I (p<0.001, p < 0.001, and p < 0.00, respectively).

Pulmonary artery banding and bidirectional cava-pulmonary shunt can be performed as a safe and effective alternative to pulmonary artery banding and arterial Blalock Taussig shunt for patients with TGA-IVS in whom arterial switch operation is needed beyond the neonatal period. This approach involves a shorter hospital stay and fewer post-operative complications.

Guaiac stool testing has been routinely used as a method to detect gastrointestinal complications in infants with critical congenital heart disease (CHD); however, the sensitivity and specificity have not been established.

A retrospective chart review was performed investigating the presence of heme-positive stools and subsequent gastrointestinal complications as well as time to goal caloric intake and radiograph exposure.

The presence of heme-positive stools was not a statistically significant factor in patients with critical CHD that experienced gastrointestinal complications. Additionally, patients with heme-positive stools did undergo more abdominal X-rays than those with heme-negative stools.

The routine use of guaiac stool testing in infants with critical CHD is not a predictor of possible gastrointestinal complications and leads to more radiograph exposure for the patient. Close clinical monitoring can be used to evaluate feeding tolerance in infants with critical CHD.

Interstage mortality causes are often unknown in infants with shunt-dependent univentricular defects. For 2 years, screening catheterisation was encouraged before neonatal discharge to determine if routine evaluation improved interstage outcomes.

Retrospective single-centre review of home monitoring programme from December, 2010 to June, 2012. Composite scores were created for physical examination/echocardiography risk factors; catheterisation risk factors; and interstage adverse events. Composite scores were compared between usual care and screening catheterisation groups. The ability of each risk factor composite to predict interstage adverse events, individually and in combination, was assessed with sensitivity, specificity, and receiver operating characteristic curves.

There were 27 usual care and 32 screening catheterisation patients. There were no significant differences between groups except rates of catheterisation before discharge (29.6 versus 100%, p < 0.001). Usual care patients who underwent catheterisation for clinical indications had higher intervention rates (37.5 versus 3.1%, p = 0.004). Physical examination/echocardiography risk factor frequency was similar, but usual care patients with catheterisation had a higher catheterisation risk factor frequency. Interstage adverse event frequency was similar (48.2 versus 53.1%, p = 0.7). For interstage adverse event prediction, sensitivity for the physical examination/echocardiography, catheterisation, and either risk factor composites was 53.3, 72, and 80%, respectively; specificity was 59, 60, and 48%. The area under the receiver operating characteristic curve was 0.56, 0.66, and 0.64.

Screening catheterisation evaluation offered slightly increased sensitivity and specificity, but no difference in interstage adverse event frequency. Given this small advantage versus known risks, screening catheterisations are no longer encouraged.

Cardiopulmonary exercise testing has been used to measure functional capacity in children who have undergone a heart transplant. Cardiopulmonary exercise testing results have not been compared between children transplanted for a primary diagnosis of CHD and those with a primary diagnosis of cardiomyopathy despite differences in outcomes. This study is aimed to compare cardiopulmonary exercise testing performance between these two groups.

Patients who underwent heart transplant with subsequent cardiopulmonary exercise testing at least 6 months after transplant at our institution were identified. They were then divided into two groups based on primary cardiac diagnosis: CHD or cardiomyopathy. Patient characteristics, echocardiograms, cardiac catheterisations, outcomes, and cardiopulmonary exercise test results were compared between the two groups.

From the total of 35 patients, 15 (43%) had CHD and 20 (57%) had cardiomyopathy. Age at transplant, kidney disease, lung disease, previous rejection, coronary vasculopathy, catheterisation, and echocardiographic data were similar between the groups. Mean time from transplant to cardiopulmonary exercise testing, exercise duration, and maximum oxygen consumption were similar in both groups. There was a difference in heart rate response with CHD heart rate response of 63 beats per minute compared to cardiomyopathy group of 78 (p = 0.028). Patients with CHD had more chronotropic incompetence than those with cardiomyopathy (p = 0.036).

Primary diagnosis of CHD is associated with abnormal heart rate response and more chronotropic incompetence compared to those transplanted for cardiomyopathy.

We developed the Long-term Early Development Research (LEADER) project to investigate the development of children with CHD and/or after cardiopulmonary resuscitation. Both populations are at risk for delays in motor, cognitive, and language development. However, few studies to date have investigated the longitudinal development in these children.

To establish a clinical research unit, we planned three studies: a cross-sectional study in children after cardiopulmonary resuscitation (LEADER-REA Pilot Study), a longitudinal study in children after cardiopulmonary resuscitation, with a focus on evaluating various biomarkers as predictors for developmental outcome (LEADER-CPR study), and a longitudinal study in children with ventricular septal defect, tetralogy of Fallot, or transposition of the great arteries after cardiac surgery (LEADER-CHD study).

Implementation of all three LEADER studies was successful and study protocols were conducted as planned. Findings from the LEADER-REA Pilot study have been recently published and data collection for both prospective trials is ongoing. Descriptive analysis of the first 20 assessments of the LEADER-CHD study showed no severe deficits in overall cognitive, motor, and language developments in the children.

Children with CHD and/or after cardiopulmonary resuscitation are at risk for developmental delay. Therefore, a detailed developmental assessment is necessary as a pre-requisite for individual developmental support. Our LEADER project has been shown to be feasible in a clinical setting and is the first step towards the establishment of a clinical research unit in our clinic with a focus on longitudinal research.

Challenges remain in the judgement of pathological murmurs in newborns at maternity hospitals, and there are still many simple major CHD patients in developing countries who are not diagnosed in a timely fashion. This study aimed to evaluate the accuracy of cardiac auscultation on neonatal CHD by general paediatricians.

We conducted a prospective study at three hospitals. All asymptomatic newborns underwent auscultation, pulse oximetry monitoring, and echocardiography. Major CHD was classified and confirmed through follow-up. We evaluated the accuracy of various degrees of murmurs for detecting major CHD to determine the most appropriate standards and time of auscultation.

A total of 6750 newborns were included. The median age of auscultation was 43 hours. Cardiac murmurs were identified in 6.6% of newborns. For all CHD, 44.4% had varying degrees of murmurs. A murmur of grade ≥2 used as a reference standard for major CHD had a sensitivity of 89.58%. The false positive rate of murmurs of grade ≥2 for detecting major CHD was significantly negatively related to auscultation time, with 84.4% of false positives requiring follow-up for non-major CHD cardiac issues. Auscultation after 27 hours of life could reduce the false positive rate of major CHD from 2.7 to 0.9%.

With appropriate training, maternity hospital’s paediatricians can detect major CHD with high detection rates with an acceptable false positive rate.