To evaluate the feasibility, efficacy, and safety of Amplatzer vascular plug II in large and elongated ducts in infants.

Patent arterial duct device closure is technically challenging in infants with large and elongated ducts because Amplatzer duct occluder and Amplatzer duct occluder II have high chances of causing aortic coarctation and left pulmonary artery stenosis, respectively. The Amplatzer vascular plug II being soft with no retention discs on either sides helps in mitigating these problems.

This is a prospective, observational study involving infants with clinical, echocardiographic and angiographic evidence of large left to right shunt. All the children underwent duct closure using Amplatzer vascular plug II.

Eighteen infants qualified for the study. Mean age and weight were 8.63 ± 3.84 months and 6.3 ± 1.7 kg, respectively. The angiographic mean duct diameter at the pulmonary artery end was 4.66 ± 0.92 mm, and the mean duct length was 9.4 ± 2.48 mm. The size of Amplatzer vascular plug II used varied from 6 mm to 10 mm. Technical success was achieved in 16/18 cases. One patient had device embolisation, and in the other, the device was found to be unstable. The ratio of Amplatzer vascular plug II size to the duct diameter was 1.65 ± 0.27, while the ratio of ductal length to device length was 1.48 ± 0.46 in those with successful outcome.

Amplatzer vascular plug II is a safe and effective option in appropriately selected infants with elongated ducts. Diameter and length of Amplatzer vascular plug II vis-a-vis those of the ductus are important determinants of the successful outcome.

There is variation in care of secundum atrial septal defects. Defects <3 mm and patent foramen ovale are not clinically significant. Defects >3 mm are often followed clinically and may require closure. Variation in how these lesions are monitored may result in over-utilisation of routine studies and higher than necessary patient charges.

To determine utilisation patterns for patients with secundum atrial septal defects diagnosed within the first year of life and compare to locally developed optimal utilisation standard to assess charge savings.

This was a retrospective chart review of patients with secundum atrial septal defects diagnosed within the first year of life. Patients with co-existing cardiac lesions were excluded. Total number of clinic visits, electrocardiograms, and echocardiograms were recorded. Total charge was calculated based on our standard institutional charges. Patients were stratified based on lesion and provider type and then compared to “optimal utilisation” using analysis of variance statistical analysis.

Ninety-seven patients were included, 40 had patent foramen ovale (or atrial septal defect <3 mm), 43 had atrial septal defects not requiring intervention and 14 had atrial septal defects requiring intervention. There was a statistically significant difference in mean charge above optimal for these lesions of $1033, $2885, and $5722 (p < 0.02), respectively. There was statistically significant variation of charge among types of provider as well. Average charge savings per patient would be $2530 with total charge savings of $242,472 if the optimal utilisation pathway was followed.

Using optimal utilisation and decreasing variation could save the patient significant unnecessary charges.

CHDs can be complicated by renal injury which worsens morbidity and mortality. Urinary neutrophil gelatinase-associated lipocalin, a sensitive and specific biomarker of renal tubular injury, has not been studied in children with uncorrected CHDs. This study evaluated renal injury in children with uncorrected CHDs using this biomarker.

The patients were children with uncorrected CHDs with significant shunt confirmed on echocardiogram with normal renal ultrasound scan, in the paediatric cardiology clinic of a tertiary hospital. The controls were age-matched healthy children recruited from general practice clinics. Information on bio-data and socio-demographics were collected and urine was obtained for measurement of urinary neutrophil gelatinase-associated lipocalin levels.

A total of 65 children with uncorrected CHDs aged 2 to 204 months were recruited. Thirty-one (47.7%) were males while 36 (55.4%) had acyanotic CHDs. The median urinary neutrophil gelatinase-associated lipocalin level of patients of 26.10 ng/ml was significantly higher than controls of 16.90 ng/ml (U = 1624.50, p = 0.023). The median urinary neutrophil gelatinase-associated lipocalin level of patients with cyanotic and acyanotic CHDs were 30.2 ng/ml and 22.60 ng/ml respectively; (Mann–Whitney U = 368.50, p = 0.116). The prevalence of renal injury using 95th percentile cut-off value of urinary neutrophil gelatinase-associated lipocalin was 16.9%. Median age of patients with renalinjury was 16 (4–44) months.

Children with uncorrected CHDs have renal injury detected as early as infancy. The use of urinary neutrophil gelatinase-associated lipocalin in early detection of renal injury in these children may enhance early intervention and resultant prevention of morbidity and reduction in mortality.

The use of statins in children, although not frequent, is recommended in specific clinical contexts, namely, familial hypercholesterolaemia, conditions carrying a moderate-high cardiovascular risk and sub-optimal cholesterol levels after implementation of lifestyle modifications. The aim of this study is to characterise children with dyslipidaemia managed with statins, followed at a tertiary referral centre in central Portugal.

The authors carried out a retrospective and descriptive study made up of 66 patients (50% males, mean age of therapy onset 11.9 years) followed up at the Cardiovascular Clinic of a tertiary referral centre between January, 2012, and May, 2018. Clinical, analytical, and echocardiographic parameters were analysed. About 60.6% had clinical and/or molecular diagnosis of familial hypercholesterolaemia. On average, each patient had three cardiovascular risk factors, obesity (31%) being most prevalent, followed by arterial hypertension (14%). Statin therapy showed a statistically significant reduction in the lipid profile, particularly in the total cholesterol (23%) and low-density lipoprotein cholesterol (30%) levels, as well as in the carotid intima-media thickness (p = 0.015). Hepatic and muscle integrity markers were within normal range.

Statins are safe and efficient in the management of children with hypercholesterolaemia. Our study showed that apart from its lipid-lowering properties, it also reduced significantly the carotid intima-media thickness and, implicitly, the cardiovascular risk of these patients.

Predictors of compliance with aspirin in children following cardiac catheterisation have not been identified. The aim of this study is to identify the caregivers’ knowledge, compliance with aspirin medication, and predictors of compliance with aspirin in children with Congenital Heart Disease (CHD) post-percutaneous transcatheter occlusion.

A cross-sectional explorative design was adopted using a self-administered questionnaire and conducted between May 2017 and May 2018. Recruited were 220 caregivers of children with CHD post-percutaneous transcatheter occlusion. Questionnaires included child and caregivers’ characteristics, a self-designed and tested knowledge about aspirin scale (scoring scale 0–2), and the 8-item Morisky Medication Adherence Scale (scoring scale 0–8). Data were analysed using multivariate binary logistic regression analysis to identify predictors of compliance with aspirin.

Of the 220 eligible children and caregivers, 210 (95.5%) responded and 209 surveys were included in the analysis. The mean score of knowledge was 7.25 (standard deviation 2.27). The mean score of compliance was 5.65 (standard deviation 1.36). Child’s age, length of aspirin use, health insurance policies, relationship to child, monthly income, and knowledge about aspirin of caregivers were independent predictors of compliance with aspirin (p < 0.05).

Caregivers of children with CHD had an adequate level of knowledge about aspirin. Compliance to aspirin medication reported by caregivers was low. Predictors of medium to high compliance with aspirin were related to the child’s age and socio-economic reasons. Further studies are needed to identify effective strategies to improve knowledge, compliance with medication, and long-term outcomes of children with CHD.

To evaluate the impact of state-mandated policies for pulse oximetry screening on healthcare utilisation, with a focus on use of echocardiograms.

Healthcare Cost and Utilisation Project, Statewide Inpatient Databases from 2008 to 2014 from six states.

We defined pre- and post-mandate cohorts based on dates when pulse oximetry became mandated in each state. Linear segmented regression models for interrupted time series assessed associations between implementation of the screening and changes in rate of newborns with Critical CHD-negative echocardiogram results. We also evaluated the changes in rate of newborns who underwent echocardiogram but were not diagnosed with any health issues that could cause hypoxemia.

We identified 5967 critical CHD-negative echocardiograms (2847 and 3120 in the pre- and post-mandate periods, respectively). Our models detected a statistically significant increasing trend in rate of critical CHD-negative echocardiograms in the pre-mandate period (Incidence Rate Ratio: 1.08, p = 0.02), but did not detect any statistical differences in changes between pre- and post-mandate periods (Incidence Rate Ratio: 0.93, p = 0.14). Among non-Whites, an increasing trend of Critical CHD-negative echocardiogram during the pre-mandate period was detected (Incidence Rate Ratio 1.12, p < 0.01) and was attenuated during the post-mandate period (Incidence Rate Ratio 0.89, p = 0.02). Similar results were observed in the sensitivity analyses among both Whites and non-Whites.

Results suggest that mandatory state screening policies are associated with reductions in false-positive screening rates for hypoxemic conditions, with reductions primarily attributed to trends among non-Whites.

To explore the mediating effects of adiposity indices in the association between physical activity level and blood pressure in a Nigerian schoolchildren population.

One thousand five hundred and seventeen schoolchildren (714 males and 803 females) from randomly selected primary schools participated. Physical activity level, sum of skinfold thickness at three sites, waist circumference, body mass index, and blood pressure were measured using standardised procedures. The statistical significance of the mediating effects of adiposity indices was determined using Sobel Test.

Some obesity indices mediated the association between physical activity level and systolic blood pressure [males: waist circumference (t = 5.31; p < 0.001), skinfold thickness (t = 3.80; p < 0.001), and waist circumference/height (t = 2.21; p < 0.001); females: body mass index (t = 8.03; p < 0.001), waist circumference (t = 7.80; p < 0.001), and skinfold thickness (t = 5.94; p < 0.001)]. Similarly, some obesity indices mediated the prediction of diastolic blood pressure in females [males: body mass index (t = 1.95; p = 0.05), waist circumference (t = 2.65; p = 0.01), and skinfold thickness (t = 1.97; p = 0.05); females blood pressure: body mass index (t = 6.49; p < 0.001), waist circumference (t = 6.29; p < 0.001), skinfold thickness (t = 2.31; p = 0.02), and waist circumference/height (t = 2.59; p = 0.01)].

The obesity indices that mediate the association between physical activity level and blood pressure vary, and their mediating effects are graded. While waist circumference and skinfold thickness exert the greatest mediating effects on the association in males, body mass index and waist circumference do in females.

The association of atrioventricular septal defect and transposition of the great arteries is very rare. As a rule, these patients have unbalanced ventricles. However, there have been no studies describing the results of single-ventricle palliation in these children.

All children who underwent surgery with a diagnosis of atrioventricular septal defect and transposition of the great arteries were included in the study. Data were obtained from medical records.

A total of 38 patients with atrioventricular septal defect and transposition of the great arteries underwent single-ventricle palliation at the study institution between 1971 and 2016. The mean follow-up was 12.4 years (median: 14.6 years, range 2–43.3 years). Most children had unbalanced atrioventricular septal defect (94.7%, 36/38). Survival was 67.6% (95% confidence interval [CI]: 50.0–80.2%) at 10 years and 57.8% (95% CI: 38.0–73.4%) at 20 years. By 10 years, 58.6% (95% CI: 40.8–72.7%) had progressed to Fontan completion, while 32.5% (95% CI: 18.2–47.6%) had died. In patients achieving Fontan completion, 20-year event-free survival was 73.3% (95% CI: 34.8–91.3%), while 5.0% (95% CI: 0.4–20.5%) had undergone cardiac transplantation and 21.7% (95% CI: 3.2–50.8%) had undergone takedown of the Fontan circulation. Freedom from atrioventricular valve surgery was 57.0% (95% CI: 37.2–72.7%) at 10 and 20 years.

The association of atrioventricular septal defect and transposition of the great arteries is very rare, and most of these children have unbalanced ventricles. Single-ventricle palliation results in 25-year overall survival of 50%. However, in patients, who had Fontan completion, survival was 75% at 25 years after Fontan operation.

Postural tachycardia syndrome is more frequently being recognised in adolescents and adults. However, its pathophysiology remains undefined. We evaluated our database for patterns in family history of clinical symptoms and associated disorders in these patients.

Patients with postural tachycardia syndrome diagnosed in our clinic between 2014 and 2018 and who were less than 19 years at diagnosis were included. The history was reviewed for family members with postural tachycardia syndrome, dizziness and/or syncope, joint hypermobility with or without hypermobile Ehlers–Danlos syndrome, and autoimmune disorders. Statistical analysis assessed the entire cohort plus differences in gender, presence or absence of joint hypermobility, and presence or absence of familial autoimmune disease.

A total of 579 patients met inclusion criteria. We found that 14.2% of patients had a family member with postural tachycardia syndrome, with male patients more likely to have an affected family member (20% versus 12.7%, p = 0.04). If the patient also had joint hypermobility, male patients were more likely to have a family member with postural tachycardia syndrome (25% versus 12.6%, p = 0.017), more than one affected family member (7.1% versus 0.74%, p = 0.001), and a family member with joint hypermobility (37.5% versus 23.7%, p = 0.032). Autoimmune disease was seen in 45.1% of family members, but more likely in female patients with concurrent hypermobility (21.1% versus 8.9%, p = 0.035).

This in-depth analysis of associated familial disorders in patients with postural tachycardia syndrome offers further insight into the pathophysiology of the disorder, and informs further screening of family members in these patients.

Early weight trends after cardiac surgery in infants from low- and middle-income countries where the majority are undernourished have not been defined. We studied the early post-operative weight trends to identify specific factors associated with early weight loss and poor weight gain after discharge following congenital heart surgery in consecutive infants undergoing cardiac surgery at a referral hospital in Southern India.

This was a prospective observational study. Weights of the babies were recorded at different time points during the hospital stay and at 1-month post-discharge. A comprehensive database of pre-operative, operative, and post-operative variables was created and entered into a multivariate logistic regression analysis model to identify factors associated with excessive early weight loss after cardiac surgery, and poor weight gain following hospital discharge.

The study enrolled 192 infants (mean age 110.7 ± 99.9 days; weight z scores − 2.5 ± 1.5). There was a small but significant (p < 0.001) decline in weight in the hospital following surgery (1.6% decline (interquartile range −5.3 to +1.7)); however, there was substantial growth following discharge (26.7% increase (interquartile range 15.3–41.8)). The variables associated with post-operative weight loss were cumulative nil-per-oral duration and cardiopulmonary bypass time, while weight gain following discharge was only associated with age.

Weight loss is almost universal early after congenital heart surgery and is associated with complex surgery and cumulative nil-per-oral duration. After discharge, weight gain is almost universal and not associated with any of the perioperative variables.

We sought to systematically study determinants of “clinical status at arrival after transport” of neonates with CHD and its impact on clinical outcomes in a low- and middle-income country environment.

Consecutive neonates with CHD (n = 138) transported (median distance 138 km; 5–425 km) to a paediatric cardiac programme in Southern India were studied prospectively. Among 138 neonatal transports, 134 were in ambulances. Four neonates were transported by family in private vehicles; 60% with duct-dependent circulation (n = 57) were transported without prostaglandin E1. Clinical status at arrival after transport was assessed using California modification of TRIPS Score (Ca-TRIPS), evidence of end-organ injury and metabolic insult.

Upon arrival, 42% had end-organ injury, 24% had metabolic insult and 36% had Ca-TRIPS Score >25. Prior to surgery or catheter intervention, prolonged ICU stay (>48 hours), prolonged ventilation (>48 hours), blood stream sepsis, and death occurred in 48, 15, 19, and 3.6%, respectively. Ca-TRIPS Score >25 was significantly associated with mortality (p = 0.005), sepsis (p = 0.035), and prolonged ventilation (p < 0.001); end-organ injury with prolonged ICU stay (p = 0.031) and ventilation (p = 0.045); metabolic insult with mortality (p = 0.012) and sepsis (p = 0.015).

Fifteen babies needed only medical management, 10 received comfort care (due to severe end-organ injury in 3), 107 underwent cardiac surgery (n = 83) or catheter intervention (n = 24), with a mortality of 6.5%. Clinical status at arrival after transport did not impact post-procedure outcomes.

Neonates with CHD often arrive in suboptimal status after transport in low- and middle-income countries resulting in adverse clinical outcomes. Robust transport systems need to be integrated in plans to develop newborn heart surgery in low- and middle-income countries.

Monitoring for acute allograft rejection improves outcomes after cardiac transplantation. Endomyocardial biopsy is the gold standard test defining rejection, but carries risk and has limitations. Cardiac magnetic resonance T2 mapping may be able to predict rejection in adults, but has not been studied in children. Our aim was to evaluate T2 mapping in identifying paediatric cardiac transplant patients with acute rejection.

Eleven paediatric transplant patients presenting 18 times were prospectively enrolled for non-contrast cardiac magnetic resonance at 1.5 T followed by endomyocardial biopsy. Imaging included volumetry, flow, and T2 mapping. Regions of interest were manually selected on the T2 maps using the middle-third technique in the left ventricular septal and lateral wall in a short-axis and four-chamber slice. Mean and maximum T2 values were compared with Student’s t-tests analysis.

Five cases of acute rejection were identified in three patients, including two cases of grade 2R on biopsy and three cases of negative biopsy treated for clinical symptoms attributed to rejection (new arrhythmia, decreased exercise capacity). A monotonic trend between increasing T2 values and higher biopsy grades was observed: grade 0R T2 53.4 ± 3 ms, grade 1R T2 54.5 ms ± 3 ms, grade 2R T2 61.3 ± 1 ms. The five rejection cases had significantly higher mean T2 values compared to cases without rejection (58.3 ± 4 ms versus 53 ± 2 ms, p = 0.001).

Cardiac magnetic resonance with quantitative T2 mapping may offer a non-invasive method for screening paediatric cardiac transplant patients for acute allograft rejection. More data are needed to understand the relationship between T2 and rejection in children.

Acute respiratory infection is one of the main causes of morbidity in children. Some studies have suggested that pulmonary hypertension and congenital heart disease with haemodynamic repercussion increase the severity of respiratory infections, but there are few publications in developing countries.

This was a prospective cohort study evaluating the impact of pulmonary hypertension and congenital heart disease (CHD) with haemodynamic repercussion as predictors of severity in children under 5 years of age hospitalised for acute respiratory infection.

Altogether, 217 children hospitalised for a respiratory infection who underwent an echocardiogram were evaluated; 62 children were diagnosed with CHD with haemodynamic repercussion or pulmonary hypertension. Independent predictors of admission to intensive care included: pulmonary hypertension (RR 2.14; 95% CI 1.06–4.35, p = 0.034), respiratory syncytial virus (RR 2.52; 95% CI 1.29–4.92, p = 0.006), and bacterial pneumonia (RR 3.09; 95% CI 1.65–5.81, p = 0.000). A significant difference was found in average length of hospital stay in children with the cardiovascular conditions studied (p = 0.000).

Pulmonary hypertension and CHD with haemodynamic repercussion as well as respiratory syncytial virus and bacterial pneumonia were predictors of severity in children with respiratory infections in this study. Early recognition of cardiovascular risks in paediatric populations is necessary to lessen the impact on respiratory infections.

There is a paucity of functional data on mid-to-late preterm infants between 30+0 and 34+6 weeks gestation. We aimed to characterise transitional cardiopulmonary and haemodynamic changes during the first 48 hours in asymptomatic mid-to-late preterm infants.

Forty-five healthy preterm newborns (mean ± standard deviation) gestation of 32.7 ± 1.2 weeks) underwent echocardiography on Days 1 and 2. Ventricular mechanics were assessed by speckle tracking-derived deformation, rotational mechanics, tissue Doppler imaging, and right ventricle-focused measures (tricuspid annular plane systolic excursion, fractional area change). Continuous haemodynamics were assessed using the NICOM™ system to obtain left ventricular output, stroke volume, heart rate, and total peripheral resistance by non-invasive cardiac output monitoring.

Right ventricular function increased (all measures p < 0.005) with mostly stable left ventricular performance between Day 1 and Day 2. NICOM-derived left ventricular output [mean 34%, 95% confidence interval 21–47%] and stroke volume [29%, 16–42%] increased with no change in heart rate [5%, −2 to 12%]. There was a rise in mean blood pressure [11%, 1–21%], but a decline in total peripheral resistance [−14%, −25 to −3%].

Left ventricular mechanics remained persevered in mid-to-late premature infants, but right ventricular function increased. Non-invasive cardiac output monitoring is feasible in preterm infants with an increase in left ventricular output driven by an improvement in stroke volume during the transitional period.

This study describes short-term and long-term outcome after treatment of critical valvular aortic stenosis in neonates in a national cohort, with surgical valvotomy as first choice intervention.

All neonates in Sweden treated for critical aortic stenosis between 1994 and 2016 were included. Patient files were analysed and cross-checked against the Swedish National Population Registry as of December 2017, giving complete survival data. Diagnosis was confirmed by reviewing echo studies. Critical aortic stenosis was defined as valvular stenosis with duct-dependent systemic circulation or depressed left ventricular function. Primary outcome was all-cause mortality and secondary outcomes were reintervention and aortic valve replacement.

Sixty-one patients were identified (50 boys, 11 girls). Primary treatment was surgical valvotomy in 52 neonates and balloon valvotomy in 6. Median age at initial treatment was 5 days (0–26), and median follow-up time was 10.8 years (0.14–22.6). There was no 30-day mortality but four late deaths. Freedom from reintervention was 66%, 61%, 54%, 49%, and 46% at 1, 5, 10, 15, and 20 years, respectively. Median time to reintervention was 3.4 months (4 days to 17.3 years). Valve replacement was performed in 23 patients (38%).

Surgical valvotomy is a safe and reliable treatment in these critically ill neonates, with no 30-day mortality and long-term survival of 93% in this national study. At 10 years of age, reintervention was performed in 54% and at end of follow-up 38% had had an aortic valve replacement.

The optimal management of symptomatic tetralogy of Fallot in neonates and younger infants with unfavourable anatomy is unclear and is further constrained by resource limitations in low and middle income countries.

Retrospective medical record review of infants with tetralogy of Fallot undergoing corrective or palliative procedures between January 2016 and June 2019.

The study included 120 infants; of whom 83 underwent primary complete repair, four underwent surgical palliation, and 33 underwent catheter-based palliation, including balloon pulmonary valvuloplasty (n = 18), right ventricular outflow tract stenting (n = 14), and stenting of the patent arterial duct (n = 1). Infants undergoing catheter-based procedures were younger in age (median 32 days; inter-quartile range (IQR) 7–144 versus 210 days; IQR 158–250), with lower baseline saturation (65 ± 12% versus 87 ± 7%) and had smaller pulmonary artery z-scores compared to the complete repair cohort. Follow-up was available for 31/33 (94%) infants (median 7 months [IQR 4–11]) who underwent trans-catheter palliation; 12 underwent complete repair, 10 are well, awaiting repair, eight required further palliation (catheter: 6; surgical: 2), and one died post-discharge from non-cardiac causes.

Catheter-based palliation is a safe and effective alternative in infants with tetralogy of Fallot who are at high risk for primary surgical repair.

We evaluated a cohort of 35 children diagnosed with long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, hypertrophic cardiomyopathy, or arrhythmogenic right ventricular cardiomyopathy with regard to physical and psychosocial well-being.

Patients wore an accelerometer to record their time involved in moderate- to vigorous-intensity physical activity and completed the Pediatric Quality of Life Inventory and the Pediatric Cardiac Quality of Life Inventory. Parents were also asked to describe if their child had changed their physical activity because of their diagnosis and how difficult and upsetting it was for the child to adapt to the physical activity recommendations.

Patients were involved in less moderate- to vigorous-intensity physical activity per day (35 min/day versus 55 min/day) and had lower Pediatric Quality of Life Inventory total health scores (79 versus 84) compared to normative data. Overall, 51% of the cohort modified their physical activity in some way because of their diagnosis and changing physical activity was associated with lower Pediatric Quality of Life Inventory and Pediatric Cardiac Quality of Life Inventory scores.

Our cohort was involved in less moderate- to vigorous-intensity physical activity and had lower Pediatric Quality of Life Inventory total health scores compared to normative paediatric data. Modifying one’s physical activity was associated with worse health-related quality of life scores, highlighting a vulnerable sub-group of children. These findings are useful for families and healthcare professionals caring for children who are adjusting to a new cardiac diagnosis of an inherited arrhythmia or cardiomyopathy.