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In patients with right ventricular diastolic dysfunction after complete repair of tetralogy of Fallot, some employ the use of beta-blockade. The theoretical benefit of this therapy is felt to be one of the two: 1) reduction in heart rate with subsequent increase in diastolic filling time and stroke volume; 2) halting or reversal of right ventricular remodelling. This study aimed to characterise the use of beta-blockade in paediatric admissions with complete repair of tetralogy of Fallot and characterise the effects of beta-blockade on admission characteristics.
Admissions from 2004 to 2015 in the Pediatric Health Information System database with complete repair of tetralogy of Fallot were identified. Characteristics between admissions with and without beta-blockade were compared by univariate analysis. Next, regression analyses were conducted to determine the independent association of beta-blockade on length of admission, billed charges, cardiac arrest, and inpatient mortality while controlling for demographic variables and comorbidities.
A total of 3594 admissions were included in the final analyses. Of these, 371 employed beta-blockade. Admissions with beta-blockade were more likely to have heart failure and tachyarrhythmias. These admissions also tended to be longer by univariate analysis. Regression analyses demonstrated that beta-blockade was independently associated with a 2.8-day increase in length of stay and no statistically significant change in billed charges, cardiac arrest, or inpatient mortality.
Beta-blockade after complete repair of tetralogy of Fallot is associated with a longer length of stay but did not statistically significantly impact billed charges, cardiac arrest, or inpatient mortality.
This manuscript investigates clinical decisions and the management of ‘intersex’ children at the University Children’s Hospital Zurich between 1945 and 1970. This was an era of rapid change in paediatric medicine, something that was mirrored in Zurich. Andrea Prader, the principal figure in this paper, started his career during the late 1940s and was instrumental in moving the hospital towards focusing more on expertise in chronic diseases. Starting in 1950, he helped the Zurich hospital to become the premier centre for the treatment of so-called ‘intersex’ children. It is this treatment, and, in particular, the clinical decision-making that is the centre of our article. This field of medicine was itself not stable. Rapid development of diagnostic tools led to the emergence of new diagnostic categories, the availability of new drugs changed the management of the children’s bodies and an increased number of medical experts became involved in decision-making, a particular focus lay with the role of the children themselves and of course with their families. How involved were children or their families in an era widely known as the golden age of medicine?
Paediatric cardiomyopathy is a progressive, often lethal disorder and the most common cause of heart failure in children. Despite its severe outcomes, the genetic aetiology is still poorly characterised. High-throughput sequencing offers a great opportunity for a better understanding of the genetic causes of cardiomyopathy.
The current study aimed to elucidate the genetic background of cardiomyopathy in Egyptian children.
This hospital-based study involved 68 patients; 58 idiopathic primary dilated cardiomyopathy and 10 left ventricular noncompaction cardiomyopathy. Cardiomyopathy-associated genes were investigated using targeted next-generation sequencing.
Consanguinity was positive in 53 and 70% of dilated cardiomyopathy and left ventricular noncompaction cardiomyopathy patients, respectively. Positive family history of cardiomyopathy was present in 28% of dilated cardiomyopathy and 10% of the left ventricular noncompaction cardiomyopathy patients. In 25 patients, 29 rare variants were detected; 2 likely pathogenic variants in TNNI3 and TTN and 27 variants of uncertain significance explaining 2.9% of patients.
The low genetic detection rate suggests that novel genes or variants might underlie paediatric cardiomyopathy in Egypt, especially with the high burden of consanguinity. Being the first national and regional report, our study could be a reference for future genetic testing in Egyptian cardiomyopathy children. Genome-wide tests (whole exome/genome sequencing) might be more suitable than the targeted sequencing to investigate the primary cardiomyopathy patients. Molecular characterisation of cardiomyopathies in different ethnicities will allow for global comparative studies that could result in understanding the pathophysiology and heterogeneity of cardiomyopathies.
Anomalous origin of the left coronary artery from the pulmonary artery is associated with high mortality if not timely surgery. We reviewed our experience with anomalous origin of the left coronary artery from the pulmonary artery to assess the preoperative variables predictive of outcome and post-operative recovery of left ventricular function.
A retrospective review was conducted and collected data from patients who underwent anomalous origin of the left coronary artery from the pulmonary artery repair at our institute from April 2005 to December 2019. Left ventricular function was assessed by ejection fraction and the left ventricular end-diastolic dimension index. The outcomes of reimplantation repair were analysed.
A total of 30 consecutive patients underwent anomalous origin of the left coronary artery from the pulmonary artery repair, with a median age of 14.7 months (range, 1.5–59.6 months), including 14 females (46.67%). Surgery was performed with direct coronary reimplantation in 12 patients (40%) and the coronary lengthening technique in 18 (60%). Twelve patients had concomitant mitral annuloplasty. There were two in-hospital deaths (6.67%), no patients required mechanical support, and no late deaths occurred. Follow-up echocardiograms demonstrated significant improvement between the post-operative time point and the last follow-up in ejection fraction (49.43%±19.92% vs 60.21%±8.27%, p < 0.01) and in moderate or more severe mitral regurgitation (19/30 vs 5/28, p < 0.01). The left ventricular end-diastolic dimension index decreased from 101.91 ± 23.07 to 65.06 ± 12.82 (p < 0.01).
Surgical repair of anomalous origin of the left coronary artery from the pulmonary artery has good mid-term results with low mortality and reintervention rates. The coronary lengthening technique has good operability and leads to excellent cardiac recovery. The decision to concomitantly correct mitral regurgitation should be flexible and be based on the pathological changes of the mitral valve and the degree of mitral regurgitation.
Cow’s milk is a naturally nutrient-dense foodstuff. A significant source of many essential nutrients, its inclusion as a component of a healthy balanced diet has been long recommended. Beyond milk’s nutritional value, an increasing body of evidence illustrates cow’s milk may confer numerous benefits related to health. Evidence from adult populations suggests that cow’s milk may have a role in overall dietary quality, appetite control, hydration and cognitive function. Although evidence is limited compared to the adult literature, these benefits may be echoed in recent paediatric studies. This article, therefore, reviews the scientific literature to provide an evidence-based evaluation of the associated health benefits of cow’s milk consumption in primary-school aged children (4-11 years). We focus on seven key areas related to nutrition and health comprising nutritional status, hydration, dental and bone health, physical stature, cognitive function, and appetite control. The evidence consistently demonstrates cow’s milk (plain and flavoured) improves nutritional status in primary-school aged children. With some confidence, cow’s milk also appears beneficial for hydration, dental and bone health and beneficial to neutral concerning physical stature and appetite. Due to conflicting studies, reaching a conclusion has proven difficult concerning cow’s milk and cognitive function therefore a level of caution should be exercised when interpreting these results. All areas, however, would benefit from further robust investigation, especially in free-living school settings, to verify conclusions. Nonetheless, when the nutritional-, physical- and health-related impact of cow’s milk avoidance is considered, the evidence highlights the importance of increasing cow’s milk consumption.
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.
Many children born with congenital heart defects are faced with cognitive deficits and psychological challenges later in life. The mechanisms behind are suggested to be multifactorial and are explained as an interplay between innate and modifiable risk factors. The aim was to assess whether there is a relationship between mean arterial pressure during surgery of a septal defect in infancy or early childhood and intelligence quotient scores in adulthood.
In a retrospective study, patients were included if they underwent surgical closure of a ventricular septal defect or an atrial septal defect in childhood between 1988 and 2002. Every patient completed an intelligence assessment upon inclusion, 14–27 years after surgery, using the Wechsler Adult Intelligence Scale Version IV.
A total of 58 patients met the eligibility criteria and were included in the analyses. No statistically significant correlation was found between blood pressure during cardiopulmonary bypass and intelligence quotient scores in adulthood (r = 0.138; 95% CI−0.133–0.389). Although amongst patients with mean arterial pressure < 40 mmHg during cardiopulmonary bypass, intelligence quotient scores were significantly lower (91.4; 95% CI 86.9–95.9) compared to those with mean arterial pressure > 40 mmHg (99.8; 95% CI 94.7–104.9).
Mean arterial pressure during surgery of ventricular septal defects or atrial septal defects in childhood does not correlate linearly with intelligence quotient scores in adulthood. Although there may exist a specific cut-off value at which low blood pressure becomes harmful. Larger studies are warranted in order to confirm this, as it holds the potential of partly relieving CHD patients of their cognitive deficits.
The gluten-free (GF) diet is the only treatment for coeliac disease (CD). While the GF diet can be nutritious, increased reliance on processed and packaged GF foods can result in higher fat/sugar and lower micronutrient intake in children with CD. Currently, there are no evidence-based nutrition guidelines that address the GF diet. The objective of this cross-sectional study was to describe the methodological considerations in forming a GF food guide for Canadian children and youth (4–18 years) with CD. Food guide development occurred in three phases: (1) evaluation of nutrient intake and dietary patterns of children on the GF diet, (2) pre-guide stakeholder consultations with 151 health care professionals and 383 community end users and (3) development of 1260 GF diet simulations that addressed cultural preferences and food traditions, diet patterns and diet quality. Stakeholder feedback identified nutrient intake and food literacy as important topics for guide content. Except for vitamin D, the diet simulations met 100 % macronutrient and micronutrient requirements for age–sex. The paediatric GF plate model recommends intake of >50 % fruits and vegetables (FV), <25 % grains and 25 % protein foods with a stronger emphasis on plant-based sources. Vitamin D-fortified fluid milk/unsweetened plant-based alternatives and other rich sources are important to optimise vitamin D intake. The GF food guide can help children consume a nutritiously adequate GF diet and inform policy makers regarding the need for nutrition guidelines in paediatric CD.
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.
Previous echocardiographic studies failed to show residual alterations of heart function in paediatric patients that have received treatment for Chagas disease. While the echocardiogram is the fundamental front-line tool for evaluating heart function, the appearance of new techniques allows a more detailed analysis. We aimed to evaluate systolic and diastolic function with new techniques in a paediatric population with Chagas disease several years after treatment completion.
Material and methods:
Echocardiograms were obtained from 84 Chagas disease patients (48 female) and 27 healthy controls. All patients had received treatment concluding on average 10 years prior to the study. The prospective analysis considered cardiac dimensions and cardiac function using two-dimensional, M-mode, Doppler and tissue Doppler imaging with emphasis on measuring longitudinal strain in the left ventricle by speckle tracking. Ejection fraction was measured with three-dimensional echocardiography.
Patients had an age of 14.2 ± 5.7 years (6–33) at the time of evaluation. Global and segmental motility of the left ventricle was normal in all patients. Ejection fraction was 59.2 ± 6.5 and 57.4 ± 6.5% (p = 0.31) in patients and controls respectively. Left ventricular global longitudinal systolic strain was −19 ± 2.4% in patients and −19 ± 3.6% (p = 0.91) in controls. No significant differences were found in remaining systolic and diastolic function measurements.
Paediatric patients that have received treatment for Chagas disease, evaluated with either conventional techniques or new tools, do not show significant long-term alterations of ventricular function.
Although cardiac catheterisation (cath) is the diagnostic test for pulmonary hypertension, it is an invasive procedure. Echocardiography (echo) is commonly used for the non-invasive diagnosis of pulmonary hypertension but maybe limited by lack of adequate signals. Therefore, emphasis has been placed on biomarkers as a potential diagnostic tool. No prior paediatric studies have simultaneously compared N-terminal pro-B-type-natriuretic peptide (NTproBNP) with cath/echo as a potential diagnostic tool. The aim of this study was to determine if NTproBNP was a reliable diagnostic tool for pulmonary hypertension in this population.
Patients were divided into Study (echo evidence/established diagnosis of pulmonary hypertension undergoing cath) and Control (cath for small atrial septal defect/patent ductus arteriosus and endomyocardial biopsy post cardiac transplant) groups. NTproBNP, cath/echo data were obtained.
Thirty-one patients met inclusion criteria (10 Study, 21 Control). Median NTproBNP was significantly higher in the Study group. Echo parameters including transannular plane systolic excursion z scores, pulmonary artery acceleration time and right ventricular fractional area change were lower in the Study group and correlated negatively with NTproBNP. Receiver operation characteristic curve analysis demonstrated NTproBNP > 389 pg/ml was 87% specific for the diagnosis of pulmonary hypertension with the addition of pulmonary artery acceleration time improving the specificity.
NTproBNP may be a valuable adjunctive diagnostic tool for pulmonary hypertension in the paediatric population. Echo measures of transannular plane systolic excursion z score, pulmonary artery acceleration time and right ventricular fractional area change had negative correlations with NTproBNP. The utility of NTproBNP as a screening tool for pulmonary hypertension requires validation in a population with unknown pulmonary hypertension status.
Retrospectively apply criteria from Center to Advance Palliative Care to a cohort of children treated in a cardiac ICU and compare children who received a palliative care consultation to those who were eligible for but did not receive one.
Medical records of children admitted to a cardiac ICU between January 2014 and June 2017 were reviewed. Selected criteria include cardiac ICU length of stay >14 days and/or ≥ 3 hospitalisations within a 6-month period.
Measurements and Results:
A consultation occurred in 17% (n = 48) of 288 eligible children. Children who received a consult had longer cardiac ICU (27 days versus 17 days; p < 0.001) and hospital (91 days versus 35 days; p < 0.001) lengths of stay, more complex chronic conditions at the end of first hospitalisation (3 versus1; p < 0.001) and the end of the study (4 vs.2; p < 0.001), and higher mortality (42% versus 7%; p < 0.001) when compared with the non-consulted group. Of the 142 pre-natally diagnosed children, only one received a pre-natal consult and 23 received it post-natally. Children who received a consultation (n = 48) were almost 2 months of age at the time of the consult.
Less than a quarter of eligible children received a consultation. The consultation usually occurred in the context of medical complexity, high risk of mortality, and at an older age, suggesting potential opportunities for more and earlier paediatric palliative care involvement in the cardiac ICU. Screening criteria to identify patients for a consultation may increase the use of palliative care services in the cardiac ICU.
We report a case of a newborn infant with coarctation of the aorta and hypoplastic transverse aortic arch who was found to have a circumaortic double left innominate vein on echocardiography. This exceedingly rare finding was important for surgical planning and was confirmed during congenital heart surgery.
Extra-corporeal membrane oxygenation is a life-saving modality to support the cardiac and/or pulmonary system as a form of life support in resuscitation, post-cardiotomy, as a bridge to cardiac transplantation and in respiratory failure. Its use in the paediatric and neonatal population has proven incredibly useful. However, extra-corporeal membrane oxygenation is also associated with a greater rate of mortality and complications, particularly in those with co-morbidities. As a result, interventions such as ventricular assist devices have been trialled in these patients. In this review, we provide a comprehensive analysis of the current literature on extra-corporeal membrane oxygenation for cardiac support in the paediatric and neonatal population. We evaluate its effectiveness in comparison to other forms of mechanical circulatory support and focus on areas for future development.
Children with congenital heart disease and cardiomyopathy are a unique patient population. Different therapies continue to be introduced with large practice variability and questionable outcomes. The purpose of this study is to determine the impact of various medications on intensive care unit length of stay, total length of stay, billed charges, and mortality for admissions with congenital heart disease and cardiomyopathy.
Materials and methods:
We identified admissions of paediatric patients with cardiomyopathy using the Pediatric Health Information System database. The admissions were then separated into two groups: those with and without inpatient mortality. Univariate analyses were conducted between the groups and the significant variables were entered as independent variables into the regression analyses.
A total of 10,376 admissions were included these analyses. Of these, 904 (8.7%) experienced mortality. Comparing patients who experienced mortality with those who did not, there was increased rate of acute kidney injury with an odds ratio (OR) of 5.0 [95% confidence interval (CI) 4.3 to 5.8, p < 0.01], cardiac arrest with an OR 7.5 (95% CI 6.3 to 9.0, p < 0.01), and heart transplant with an OR 0.3 (95% CI 0.2 to 0.4, p < 0.01). The medical interventions with benefit for all endpoints after multivariate regression analyses in this cohort are methylprednisolone, captopril, enalapril, furosemide, and amlodipine.
Diuretics, steroids, angiotensin-converting enzyme inhibitors, calcium channel blockers, and beta blockers all appear to offer beneficial effects in paediatric cardiomyopathy admission outcomes. Specific agents within each group have varying effects.
Percutaneous closure of atrial septal defect is recognised as a safe and effective procedure, however, in some patients complications may occur. Although chest pain has been sporadically reported, its exact aetiology has been poorly studied. Herein, a 14-year-old female with an atypical and long-lasting chest pain after percutaneous atrial septal defect closure is described.
In low-income countries, prospective data on combined effects of in utero teratogen exposure are lacking and necessitates new research. The aim of the present study was to explore the effect of in utero teratogen exposure on the size of the kidneys and pancreas 5 years after birth in a low-income paediatric population. Data was collected from 500 mother–child pairs from a low-income setting. Anthropometric measurements included body weight, (BW) body height, mid-upper arm and waist circumference (WC). Clinical measurements included blood pressure (BP), mean arterial pressure and heart rate. Ultrasound measurements included pancreas, and kidney measurements at age 5 years. The main outcome of interest was the effect of maternal smoking and alcohol consumption on ultrasound measurements of organ size at age 5 years. Left and right kidney length measurements were significantly lower in smoking exposed children compared to controls (p = 0.04 and p = 0.03). Pancreas body measurements were significantly lower in smoking exposed children (p = 0.04). Multiple regression analyses were used to examine the associations between the independent variables (IDVs), maternal age, body mass index (BMI), mid-upper arm circumference (MUAC) and BW of the child, on the dependent variables (DVs) kidney lengths and kidney volumes. Also, the association between in utero exposure to alcohol and nicotine and pancreas size. WC was strongest (r = 0.28; p < 0.01) associated with pancreas head [F (4, 454) = 13.44; R2 = 0.11; p < 0.01] and tail (r = 0.30; p < 0.01) measurements at age 5 years, with in utero exposure, sex of the child and BMI as covariates. Kidney length and pancreas body measurements are affected by in utero exposure to nicotine at age 5 years and might contribute to cardiometabolic risk in later life. Also, findings from this study report on ultrasound reference values for kidney and pancreas measurements of children at age 5 years from a low-income setting.
We describe a previously asymptomatic 7-year-old girl with a sudden cardiac arrest during elective pacemaker revision. Later imaging identified epicardial pacemaker lead strangulation of the left anterior descending and left circumflex coronary arteries. Anaesthetic induction led to a reduction in myocardial perfusion, precipitating the arrest. Extreme care should be taken during anaesthesia if cardiac strangulation is suspected.
Infants with single ventricle following stage I palliation are at risk for poor nutrition and growth failure. We hypothesise a standardised enteral feeding protocol for these infants that will result in a more rapid attainment of nutritional goals without an increased incidence of gastrointestinal co-morbidities.
Materials and methods:
Single-centre cardiac ICU, prospective case series with historical comparisons. Feeding cohort consisted of consecutive patients with a single ventricle admitted to cardiac ICU over 18 months following stage I palliation (n = 33). Data were compared with a control cohort and admitted to the cardiac ICU over 18 months before feeding protocol implementation (n = 30). Feeding protocol patients were randomised: (1) protocol with cerebro-somatic near-infrared spectroscopy feeding advancement criteria (n = 17) or (2) protocol without cerebro-somatic near-infrared spectroscopy feeding advancement criteria (n = 16).
Median time to achieve goal enteral volume was significantly higher in the control compared to feeding cohort. There were no significant differences in enteral feeds being held for feeding intolerance or necrotising enterocolitis between cohorts. Feeding cohort had significant improvements in discharge nutritional status (weight, difference admit to discharge weight, weight-for-age z score, volume, and caloric enteral nutrition) and late mortality compared to the control cohort. No infants in the feeding group with cerebro-somatic near-infrared spectroscopy developed necrotising enterocolitis versus 4/16 (25%) in the feeding cohort without cerebro-somatic near-infrared spectroscopy (p = 0.04).
A feeding protocol is a safe and effective means of initiating and advancing enteral nutrition in infants following stage I palliation and resulted in improved nutrition delivery, weight gain, and nourishment status at discharge without increased incidence of gastrointestinal co-morbidities.
The combination of pediatric cardiology being both a perceptual and a cognitive subspecialty demands a complex decision-making model which makes artificial intelligence a particularly attractive technology with great potential. The prototypical artificial intelligence system would autonomously impute patient data into a collaborative database that stores, syncs, interprets and ultimately classifies the patient’s profile to specific disease phenotypes to compare against a large aggregate of shared peer health data and outcomes, the current medical body of literature and ongoing trials to offer morbidity and mortality prediction, drug therapy options targeted to each patient’s genetic profile, tailored surgical plans and recommendations for timing of sequential imaging. The focus of this review paper is to offer a primer on artificial intelligence and paediatric cardiology by briefly discussing the history of artificial intelligence in medicine, modern and future applications in adult and paediatric cardiology across selected concentrations, and current barriers to implementation of these technologies.