Although a life-preserving surgery for children with single ventricle physiology, the Fontan palliation is associated with striking morbidity and mortality with advancing age. Our primary objective was to evaluate the impact of non-invasive, external, thoraco-abdominal ventilation on pulmonary blood flow (PBF) and cardiac output (CO) as measured by cardiovascular magnetic resonance (CMR) imaging in adult […]
Congenital diaphragmatic hernia is a deficiency of the fetal diaphragm resulting in herniation of the abdominal viscera into the thoracic cavity. The best method of respiratory management of congenital diaphragmatic hernia is unclear, but high frequency oscillatory ventilation is often used as the initial ventilator mode for severe congenital diaphragmatic hernia. When it becomes impossible […]
Death is defined biologically as the irreversible loss of the functioning of the organism as a whole, which typically occurs after the loss of cardiorespiratory function. In 1968, a Harvard committee proposed that death could also be defined neurologically as the irreversible loss of brain function. Brain death has been considered to be equivalent to […]
Background Increasing numbers of neonates are undergoing painful procedures in low-income and middle-income countries, with adequate analgesia seldom used. In collaboration with a multi-disciplinary team in Kenya, we aimed to establish the first evidence-based guidelines for the management of routine procedure-related neonatal pain that consider low-resource hospital settings.We did a systematic review by searching MEDLINE, […]
Cardiopulmonary exercise test (CPET) provides accurate evaluation of physical capacity and disease severity in children with congenital heart disease (CHD). However, full participation to obtain optimal measure of VO2max may be difficult. As an alternative, the oxygen uptake efficiency slope (OUES) is a reproducible and reliable parameter measured during CPET, which does not require a […]
Background Increasing numbers of neonates are undergoing painful procedures in low-income and middle-income countries, with adequate analgesia seldom used. In collaboration with a multi-disciplinary team in Kenya, we aimed to establish the first evidence-based guidelines for the management of routine procedure-related neonatal pain that consider low-resource hospital settings.We did a systematic review by searching MEDLINE, […]
Cardiopulmonary exercise test (CPET) provides accurate evaluation of physical capacity and disease severity in children with congenital heart disease (CHD). However, full participation to obtain optimal measure of VO2max may be difficult. As an alternative, the oxygen uptake efficiency slope (OUES) is a reproducible and reliable parameter measured during CPET, which does not require a […]
We aimed to assess the risk factors for left ventricle (LV) enlargement in children with idiopathic frequent ventricular premature complexes (VPC) and discuss the clinical features and treatment strategies. Children diagnosed with idiopathic frequent VPC at Xinhua Hospital affiliated to the Shanghai Jiao Tong University during 2013 to 2019 were retrospectively evaluated. Gender, age, body […]
We sought to evaluate the association between low birth weight (LBW) and outcomes following neonatal cardiac surgery in a low-income and middle-income country setting where LBW prevalence is high and its impact on surgical outcomes is undefined.Single-centre retrospective cohort study.Referral paediatric hospital in Southern India PATIENTS: All neonatal cardiac surgical cases between January 2011 and […]
Paediatric Early Warning Scores (PEWSs) are being used increasingly in hospital wards to identify children at risk of clinical deterioration, but few scores exist that were designed for use in emergency care settings. To improve the prioritisation of children in the emergency department (ED), we developed and validated an ED-PEWS.The TrIAGE project is a prospective […]
Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. Despite early detection of CHD and major advances in paediatric cardiac surgery, ES is still prevalent and requires a multidisciplinary approach by adult CHD experts in tertiary centres. […]
Cardiorespiratory and pulse oximetry monitoring in children who are hospitalized should balance benefits of detecting deterioration with potential harms of alarm fatigue. We developed recommendations for monitoring outside the ICU on the basis of available evidence and expert opinion.We conducted a comprehensive literature search for studies addressing the utility of cardiorespiratory and pulse oximetry monitoring […]
Hereditary hemorrhagic telangiectasia (HHT) can be clinically diagnosed, but children often lack characteristic features. We report a family with homozygous growth differentiation factor 2 (GDF2)-related HHT diagnosed by genetic testing. A boy aged 5 years and 2 months presented with isolated hypoxemia. He was the product of a consanguineous marriage; his parents were second cousins. […]
To assess and compare associations between childhood maltreatment and cardiovascular disease (CVD) in men and women in the UK. In secondary analyses, we also explored possible age differences and associations with early onset CVD (<50 years).We included 157 311 participants from the UK Biobank who had information on physical, sexual or emotional abuse, emotional or physical […]
In April, 2020, clinicians in the UK observed a cluster of children with unexplained inflammation requiring admission to paediatric intensive care units (PICUs). We aimed to describe the clinical characteristics, course, management, and outcomes of patients admitted to PICUs with this condition, which is now known as paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 […]
Highly reliable identification of adults with hypertrophic cardiomyopathy (HC) at risk for sudden death (SD) has been reported. A significant controversy remains, however, regarding the most reliable risk stratification methodology for children and adolescents with HC. The present study assesses the accuracy of SD prediction and prevention with prophylactic implantable cardioverter-defibrillators (ICDs) in young HC […]
Currently, car seat tolerance screens (CSTSs) are recommended for all infants born prematurely in the United States. Although many late-preterm infants are cared for exclusively in newborn nurseries (NBNs), data on implementation of CSTS in nurseries are limited. Our objective for this study was to determine management strategies and potential variation in practice of CSTS […]
Preterm birth is associated with incident heart failure in children and young adults.To determine the effect size of preterm birth on cardiac remodeling from birth to young adulthood.Data sources include Medline, Embase, Scopus, Cochrane databases, and clinical trial registries (inception to March 25, 2020).Studies in which cardiac phenotype was compared between preterm individuals born at […]
To examine how overweight and obesity at specific ages and overall BMI growth patterns throughout childhood predict cardiometabolic phenotypes at 11 to 12 years.In a population-based sample of 5107 infants, BMI was measured every 2 years between ages 2 to 3 and 10 to 11 years. We identified 5 BMI trajectories using growth curve models. […]
13q33-q34 microdeletions are rare chromosomal aberrations associated with a high risk of developmental disability, facial dysmorphism, cardiac defects and other malformation of organs. It is necessary to collect and report evidence of this rare chromosome mutation to improve the prognosis of this rare disease.We report a patient harboring an 11.56 Mb microdeletion at 13q33.1-34 region, which […]
More than 50% of newborns with congenital heart disease (CHD) are unrecognized at birth; however, the use of echocardiogram (Echo) for diagnosing CHD in newborns with asymptomatic, non-syndromic cardiac murmurs (ANCM), has not been systematically reviewed yet. We aimed to identify the incidence of CHD diagnosed with Echo and systematically review whether Echo should be […]
Alveolar capillary dysplasia (ACD) is a rare cause of severe pulmonary hypertension and respiratory failure in neonates. The onset of ACD is usually preceded by a short asymptomatic period. The condition is refractory to all available therapies as it irreversibly affects development of the capillary bed in the lungs. The diagnosis of ACD is based on […]
Despite substantial variation of streptococcal antibody titres among global populations, there is no data on normal values in sub-Saharan Africa. The objective of this study was to establish normal values for antistreptolysin O (ASO) and antideoxyribonuclease B (ADB) antibodies in Uganda.This was an observational cross-sectional study.This study was conducted at Mulago National Referral Hospital, which […]
Understanding the epidemiology and clinical course of multisystem inflammatory syndrome in children (MIS-C) and its temporal association with coronavirus disease 2019 (Covid-19) is important, given the clinical and public health implications of the syndrome.We conducted targeted surveillance for MIS-C from March 15 to May 20, 2020, in pediatric health centers across the United States. The […]
A multisystem inflammatory syndrome in children (MIS-C) is associated with coronavirus disease 2019. The New York State Department of Health (NYSDOH) established active, statewide surveillance to describe hospitalized patients with the syndrome.Hospitals in New York State reported cases of Kawasaki’s disease, toxic shock syndrome, myocarditis, and potential MIS-C in hospitalized patients younger than 21 years […]
Sleep-disordered breathing (SDB) is a common syndrome in children, related to their immune responses, cardiovascular function, and neurocognitive function. This study aimed to determine the prevalence of SDB among children in Wuxi, China, and to evaluate the protective and risk factors of SDB in children.A cross-sectional study was conducted on children attending different schools across […]
The prevalence of child and adolescent obesity and severe obesity continues to increase despite decades of policy and research aimed at prevention. Obesity strongly predicts cardiovascular and metabolic disease risk; both begin in childhood. Children who receive intensive behavioral interventions can reduce body mass index (BMI) and reverse disease risk. However, delivering these interventions with […]
Corticosteroids are widely used in pediatric cardiac surgery to blunt systemic inflammatory response and to reduce complications; nevertheless, their clinical efficacy is uncertain.To determine whether intraoperative administration of dexamethasone is more effective than placebo for reducing major complications and mortality during pediatric cardiac surgery.The Intraoperative Dexamethasone in Pediatric Cardiac Surgery was an investigator-initiated, double-blind, multicenter […]
Flecainide acetate is a Vaughan-Williams class IC antiarrhythmic drug prescribed for the treatment of supraventricular arrhythmias. It has a narrow therapeutic index and proarrhythmic effects even at therapeutic doses. Flecainide is metabolized by a CYP2D6 enzyme that exhibits polymorphism. In this case report, we present, to our best knowledge, the first case of toxicity contributed […]
Pheochromocytomas are rare tumors originating in chromaffin cells which predominantly are located in adrenal glands. Sustained or paroxysmal hypertension (HT) is the most frequent sign of pheochromocytoma. In some cases, it is associated with the classic triad including episodic headaches, sudoresis, and tachycardia; however, we present a case of pheochromocytoma with first presentation of cardiomyopathy.The […]
Neonatal-perinatal medicine (NPM) fellowship programs must provide adequate delivery room (DR) experience to ensure that physicians can independently provide neonatal resuscitation to very low birth weight (VLBW) infants. The availability of learning opportunities is unknown.The number of VLBW (≤1500 g) and extremely low birth weight (ELBW) (<1000 g) deliveries, uses of continuous positive airway pressure, […]
Psychological and behavioral correlates are considered important in the development and persistence of obesity in both adults and youth. This study aimed to identify such features in youth with severe obesity (BMI ≥ 120% of 95thpercentile of sex-specific BMI-for-age) compared to those with overweight or non-severe obesity.Youth with BMI ≥ 85th percentile were invited to participate in a prospective […]
Hypertension is highly prevalent in pediatric kidney transplant recipients and contributes to cardiovascular death and graft loss. Improper blood pressure (BP) measurement limits the ability to control hypertension in this population. Here, we report multicenter efforts from the Improving Renal Outcomes Collaborative (IROC) to standardize and improve appropriate BP measurement in transplant patients.Seventeen centers participated […]
Viral respiratory infections (VRIs) are a frequent cause of hospitalization in children under 24 months of age. A history of prematurity or heart disease may be a risk factor for complications in patients hospitalized for VRI. The objective was to describe epidemiological data for children hospitalized for IRV and aged 1 to 24 months and to identify […]
In communities with high rates of coronavirus disease 2019, reports have emerged of children with an unusual syndrome of fever and inflammation.To describe the clinical and laboratory characteristics of hospitalized children who met criteria for the pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (PIMS-TS) and compare these characteristics […]
Patients are surviving decades after congenital heart surgery (CHS), raising the importance of postoperative quality of life as an outcome measure. We determined the long-term social outcomes after CHS performed during childhood.Between 1953 and 2009, 10 635 patients underwent surgery for congenital heart defects at <15 years of age in Finland. We obtained 4 control subjects […]
Seven years after its addition to the US Recommended Uniform Screening Panel, newborn screening for critical congenital heart disease (CCHD) using pulse oximetry became mandatory in the United States. Although CCHD newborn screening reduces morbidity and mortality, there remain important opportunities to improve. An expert panel convened for a 1-day meeting in September 2018, including […]
To describe the methodology undertaken to provide guidance on the appropriateness, as well as inappropriateness, of vascular access device selection, characteristics, and insertion technique for pediatric patients.The RAND Corporation-University of California, Los Angeles Appropriateness Method was used. After definition of key terms and scope, a systematic review of the pediatric vascular access literature was undertaken. […]
A previously healthy 15-year-old boy presented with 3 months of progressive psychosis, insomnia, back and groin pain, and hyperhidrosis. On examination, the patient was disheveled, agitated, and soaked with sweat, with systolic blood pressure in the 160s and heart rate in the 130s. Aside from occasional auditory and visual hallucinations, his neurologic examination was normal. […]
Methylmalonic Aciduria and Homocystinemia, cobalamin C (cblC) is an inherited disease of vitamin B12 metabolism with a wide spectrum of clinical manifestations. cblC presenting with pulmonary hypertension (PH) as leading sympotom is rare and easily misdiagnosed because of limited awareness. Timely diagnosis is crucial by the relentless progression without appropriate treatment.We reported a 12-year-old girl […]
Anthracycline-induced cardiotoxicity can lead to clinical and subclinical heart failure. Decrease of global longitudinal strain is a predictor for heart failure. Early detection of subclinical cardiotoxicity is crucial for timely intervention and prevention of further progression. Cardiac function of 41 survivors of childhood acute lymphoblastic leukemia (ALL) was assessed. Values of cardiac troponin T, N-terminal-pro-brain […]
Childhood obesity is associated with a multitude of co-morbidities, including hypertension, hyperlipidaemia, cardiovascular disease and type 2 diabetes. Childhood obesity can also affect a young person’s social, emotional and mental health if they encounter negative prejudice and social marginalisation. Given the prevalence of overweight and obese children globally, it is imperative that effective interventions are […]
Sickle cell anaemia (SCA) is characterized by attendant ischemia-reperfusion injury especially to the heart.The aim of this work is to compare the right ventricular function of children with SCA in steady state (subjects) with those with haemoglobin AA genotype (controls), using echocardiography. It is a cross-sectional study, which echocardiographic measurements to assess right ventricular function […]
Newborn resuscitation relies on accurate heart rate (HR) assessment, which, during auscultation, is prone to error. We investigated if a 6 s visual timer (VT) could improve HR assessment accuracy during newborn simulation.Prospective observational study of newborn healthcare professionals.Three-phase developmental approach: phase I: HR auscultation during newborn simulation using a standard clock timer (CT) or […]
Advances in paediatric care mean that more children with complex medical problems (heart disease, neurodevelopmental problems and so on) are surviving their early years. This has important implications for the design and delivery of healthcare given their extensive multidisciplinary requirements and susceptibility to poor outcomes when not optimally managed. Importantly, their medical needs must also […]
The study aimed to investigate the risk factors of malnutrition in children with congenital heart defect (CHD) in China.This cohort study was performed at the biggest pediatric heart center in China; 3252 patients with CHD who underwent cardiac surgeries in 2013 were included. Anthropometric measurements included weight for age Z score (WAZ), weight for height […]
Hypertensive disorders of pregnancy (HDPs) affect 5%-10% of pregnancies and have been associated with excess maternal cardiovascular disease (CVD) risk. The primary aim of this study was to reliably estimate absolute and relative risks of CVD after HDP.A retrospective cohort of women who had singleton pregnancies in New South Wales, Australia, between 2002 and 2016 […]
Children with congenital heart disease have complex medical and neurodevelopmental needs. We aimed to develop a multi-professional consensus-based referral pathway applicable to action the results of the brief developmental assessment (BDA), a validated early recognition tool, that categorises the neurodevelopmental status as green (appropriate for age), amber (equivocal) or red (delayed) in children aged between […]
Use of simulation in neonatal resuscitation (NR) training programmes has increased throughout low-income and middle-income countries. Many of such programmes have demonstrated a positive impact on NR knowledge and skill acquisition along with reduction of early neonatal mortality and fresh stillbirth rates. However, NR skill retention after simulation programmes remains a challenge.This study assessed facility […]
Kawasaki Disease (KD) is considered a major acquired heart disease in children under the age of 5. Coronary artery aneurysm (CAA) can occur in serious cases despite extreme therapy efforts. Previous studies have reported low serum albumin level was associated with disease outcome, but no further investigation was addressed yet.This retrospective (case-control) study randomly included […]
There is only limited experience with wearable cardioverter-defibrillators (WCD) in pediatric patients. We report on the successful application of a WCD in an adolescent patient with hypertrophic cardiomyopathy and myocardial bridging.A 15-year-old girl presented with a history of recurrent syncope, dyspnea, and vertigo with exercise. Diagnostic work-up revealed non-obstructive hypertrophic cardiomyopathy and signs of myocardial […]
Monosomy 1p36 is the most common terminal deletion syndrome with an autosomal dominant pattern of inheritance. This syndrome is defined by an extremely wide spectrum of characteristics; however, developmental delay and intellectual disability of various degree are present in all patients and about the 90% of patients have a severe intellectual disability. Dental agenesis or […]
The World Health Organization recommends tummy time for infants because of the benefits of improved motor development and reduced likelihood of plagiocephaly. Because of poor uptake of these recommendations, the association of tummy time with other health outcomes requires further investigation.To review existing evidence regarding the association of tummy time with a broad and specific […]
Primordial and primary prevention is the cornerstone for cardiometabolic health. In the randomised, controlled Special Turku Coronary Risk Factor Intervention Project (STRIP; n=1116), a 20-year dietary counselling intervention was given to children biannually from infancy, and cardiometabolic health benefits had been observed among the participants in the intervention group. Here, we report on the key […]
US national guidelines discourage the use of continuous pulse oximetry monitoring in hospitalized children with bronchiolitis who do not require supplemental oxygen.Measure continuous pulse oximetry use in children with bronchiolitis.A multicenter cross-sectional study was performed in pediatric wards in 56 US and Canadian hospitals in the Pediatric Research in Inpatient Settings Network from December 1, […]
During childhood and adolescence leading behavioural risk factors for the development of cardiometabolic diseases include poor diet quality and sedentary lifestyle. The aim of this study was to determine the feasibility and effect of a real-world group-based multidisciplinary intervention on cardiorespiratory fitness, diet quality and self-concept in sedentary children and adolescents aged 9 to 15 years.Project […]
US national guidelines discourage the use of continuous pulse oximetry monitoring in hospitalized children with bronchiolitis who do not require supplemental oxygen.Measure continuous pulse oximetry use in children with bronchiolitis.A multicenter cross-sectional study was performed in pediatric wards in 56 US and Canadian hospitals in the Pediatric Research in Inpatient Settings Network from December 1, […]
High-quality cardiopulmonary resuscitation (CPR) increases the likelihood of survival of pediatric out-of-hospital cardiac arrest (OHCA). Maintenance of high-quality CPR during transition of care between prehospital and pediatric emergency department (PED) providers is challenging. Our objective for this initiative was to minimize pauses in compressions, in alignment with American Heart Association recommendations, for patients with OHCA […]
Our study was to apply the 2015 American Heart Association/American College of Cardiology Athletic Participation Guidelines to a group of otherwise healthy school age children and young adults with bicuspid aortic valve (BAV) and describe the potential competitive sports restriction as they age. We performed a retrospective chart review of children and young adults aged […]
Anomalous aortic origin of the left coronary artery (AAOLCA) from the right aortic sinus is a rare congenital anomaly associated with significantly increased risk of myocardial ischemia, arrhythmias and sudden cardiac death. This refers specifically to AAOLCA associated with interarterial and/or intramural course. Much less is known about anomalous origin of the left coronary artery […]
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