Paediatric Cardiology

Decreased kidney function increases cardiovascular risk and predicts poor survival. Estimated glomerular filtration rate (eGFR) by creatinine may theoretically be less accurate in the critically ill. This observational study compares long-term cardiovascular mortality risk by the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) creatinine equation; Caucasian, Asian, paediatric and adult cohort (CAPA) cystatin C equation and […]

Necrotizing enterocolitis (NEC) is a common devastating inflammatory gastrointestinal disease and frequently occurs in premature infants. Here, we reported a case of late-onset NEC in a term neonate with good outcome after surgery for long-term follow-up.Ten-week-old male came to emergency unit due to prolonged diarrhea and abdominal distention. He was born at gestational age of […]

In the pediatric population, exercise capacity differs between females and males and the gap widens through adolescence. However, specific age- and sex-based changes in adolescents with congenital heart disease and Fontan palliation have not been reported. The purpose of the current study is to identify age- and sex-specific changes in exercise performance at peak and […]

Radiation reduction in the pediatric cardiac catheterization laboratory is well-suited for targeted quality improvement (QI) interventions. Transcatheter atrial septal defect (ASD) closure was chosen for this QI project based on a homogenous procedural population and inter-operator variability in radiation usage, with the aim to reduce radiation exposure during ASD device closure by 50% over 1 […]

There has been little exploration of acute myocarditis trends in children despite notable advancements in care over the past decade.  We explored trends in pediatric hospitalizations for acute myocarditis from 2007-2016 in the United States (US). This was a retrospective, serial cross-sectional study of the National Inpatient Sample database from 2007 to 2016, identifying patients […]

ABO-incompatible heart transplantation increases donor availability in young children and is evolving into standard of care in children younger than 2 years. Previous smaller studies suggest similar outcomes to ABO-compatible heart transplantation, but persisting alterations of the immune system in ABO-incompatible recipients might increase the risk of some infections or benefit the graft owing to […]

Multisystem inflammatory syndrome in children (MIS-C) is a newly identified and serious health condition associated with SARS-CoV-2 infection. Clinical manifestations vary widely among patients with MIS-C, and the aim of this study was to investigate factors associated with severe outcomes.In this retrospective surveillance study, patients who met the US Centers for Disease Control and Prevention […]

Hypertension is a major modifiable cardiovascular disease risk factor and its presence in childhood is associated with the presence and burden of atherosclerosis. Moreover, hypertension tracks from childhood to adulthood and is associated with adverse cardiac changes and vascular damage that in turn are associated with premature cardiovascular disease in adulthood. Therefore, the early identification […]

Indigenous children and young peoples live with an inequitable burden of acute rheumatic fever and rheumatic heart disease. In this Review, we focus on the epidemiological burden and lived experience of these conditions for Indigenous young peoples in Australia, New Zealand, and Canada. We outline the direct and indirect drivers of rheumatic heart disease risk […]

Our 2011 report, reflecting data from 2007-2008, demonstrated that, for many pediatric subspecialties, pursuing fellowship training was a negative financial decision when compared with practicing as a general pediatrician. We provide an updated analysis on the financial impact of pediatric fellowship training and model interventions that can influence the results.We estimated the financial returns a […]

The COVID-19 pandemic disproportionately affects females in the home and workplace. This study aimed to acquire information regarding the gender-specific effects of the COVID-19 lockdown on aspects of professional and personal lives of a subset of pediatric cardiologists. We sent an online multiple-choice survey to a listserv of Pediatric Cardiologists. Data collected included demographics, dependent […]

Strategies to prevent sudden cardiac death (SCD) among young athletes have become topical worldwide and unrecognized cardiac pathology has been identified as a leading cause. Black ethnicity has been reported as an independent predictor of abnormal electrocardiography (ECG) findings among athletes and the frequency and significance of training-related ECG findings versus findings suggestive of an underlying […]

Absent pulmonary valve syndrome (APV) is a rare condition usually associated with tetralogy of Fallot (TOF). Some infants develop respiratory failure from bronchial compression and the long-term neurodevelopmental outcome is unknown. We aimed to investigate the outcomes of APV and the need for long-term ventilation (LTV).Retrospective single-centre review of patients diagnosed with APV between 2007 […]

The transition from intrauterine life to extrauterine existence encompasses significant cardiorespiratory adaptations. These include rapid lung aeration and increase in pulmonary blood flow (PBF). Perinatal asphyxia and fetal growth restriction can severely hamper this transition. Hypoxia is the common denominator in these 2 disease states, with the former characterized by acute insult and the latter […]

Updated knowledge on the rates and causes of death among children with severe congenital heart defects (CHDs) is needed to further improve treatment and survival. This study investigated nationwide mortality rates in children with severe CHDs with an emphasis on unexpected mortality unrelated to cardiac intervention.Data on all pregnancies and live-born children in Norway from […]

Pregnancy may be a key window to optimize cardiovascular health (CVH) for the mother and influence lifelong CVH for her child.To examine associations between maternal gestational CVH and offspring CVH.This cohort study used data from the Hyperglycemia and Adverse Pregnancy Outcome (HAPO) Study (examinations: July 2000-April 2006) and HAPO Follow-Up Study (examinations: February 2013-December 2016). […]

With evidence of benefits of pediatric palliative care (PPC) integration, we sought to characterize subspecialty PPC referral patterns and end of life (EOL) care in pediatric advanced heart disease (AHD).In this retrospective cohort study, we compared inpatient pediatric (<21 years) deaths due to AHD in 2 separate 3-year epochs: 2007-2009 (early) and 2015-2018 (late). Demographics, […]

Because of the absence of a specific diagnostic test and pathognomonic clinical features, physicians must rely on the presence of specific clinical criteria and laboratory data that support the diagnosis of KD. To help clinicians distinguish KD, KDSS, septic shock, and TSS earlier, we suggest differential diagnosis and treatment guideline.Medical records of immunocompetent patients who […]

Current nutritional management of infants born very preterm results in significant deficiency of the essential fatty acids (FAs) arachidonic acid (ARA) and docosahexaenoic acid (DHA). The impact of this deficit on brain maturation and inflammation mediated neonatal morbidities are unknown. The aim of this study is to determine whether early supply of ARA and DHA […]

Upper endoscopy is an essential tool for diagnosing pediatric gastrointestinal issues. This study aimed to assess the indications, diagnostic yields, concordance between histopathological and endoscopic findings and suitability of upper endoscopies performed at a tertiary university hospital in Jordan.Hospital records of children who underwent upper endoscopy were retrospectively reviewed. Demographics, endoscopic details (e.g., indications, findings […]

N-methyl-D-aspartate (NMDA) receptors are ligand-gated ion channels that mediate excitatory synaptic transmission in the central nervous system. The functional NMDA receptors are heterotetramers consisting mainly of two GluN1 and two GluN2 subunits. GluN2 is encoded by the GRIN2D gene. A few case series have shown that GRIN2D variants are linked to developmental and epileptic encephalopathy. […]

Data on anxiety, depression, and attention-deficit/hyperactivity disorder (ADHD) are lacking for youth with congenital heart disease (CHD), particularly those with simple CHD. This study aims to characterize these disorders in youth with CHD compared to those without CHD.A comparative cross-sectional study was conducted by using the electronic medical records of a large tertiary care hospital […]

Granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody-associated vasculitis that primarily affects small vessels. The disease typically affects the respiratory tract and kidneys but has also been known to involve the gastrointestinal tract, genitourinary tract, thyroid, and liver. Cardiac involvement is rare. Coronary artery aneurysms (CAAs) are an extremely uncommon finding, with only 1 reported […]

Bicuspid aortic valve (BAV) aortopathy is defined by dilation of the aortic root (AoRt) and/or ascending aorta (AsAo), and increases risk for aortic aneurysm and dissection. The effects of medical prophylaxis on aortic growth rates in moderate to severe bicuspid aortopathy have not yet been evaluated. This was a single-center retrospective study of young patients […]

Aortic Z-score (Z-score) is utilized in clinical trials to monitor the effect of medications on aortic dilation rate in Marfan (MFS) patients. Z-scores are reported in relation to body surface area (BSA) and therefore are a function of height and weight. However, an information void exists regarding natural, non-pharmacological changes in Z-scores as children age. […]

It is proven that adverse intrauterine environment results in ‘early life programming,’ alterations in metabolism and physiological development of the fetus, often termed as ‘Developmental Origins of Health and Disease’ (DOHaD) resulting in a smaller size at birth, greater non-communicable diseases (NCD) risk factors during childhood and adolescence, and cardiometabolic disorders in adulthood. Nevertheless, very […]

Bazett formula is frequently used in paediatric screening for the long QT syndrome (LQTS) and proposals exist that using standing rather than supine electrocardiograms (ECG) improves the sensitivity of LQTS diagnosis. Nevertheless, compared to adults, children have higher heart rates (especially during postural provocations) and Bazett correction is also known to lead to artificially prolonged […]

Pulmonary hypertension is a deadly complication of bronchopulmonary dysplasia, the most common pulmonary morbidity of prematurity. Despite these catastrophic consequences, no evidence-based therapies are available for the prevention of pulmonary hypertension in this population. Sildenafil is a potent pulmonary vasodilator approved by the US Food and Drug Administration for the treatment of pulmonary hypertension in […]

To establish novel facial characteristics unique to congenital rubella syndrome (CRS) as prediagnostic criteria to supplement disease diagnosis in patients with or without a history of maternal rubella infection.An analysis of 115 CRS case series (2018-2020) based on the presence of any of the triad features.Outpatient department of a tertiary care referral cardiac hospital in […]

Necrotizing enterocolitis (NEC) is a devastating disease that is relatively frequently diagnosed in term infants with congenital heart disease (CHD), compared with term infants without CHD, in whom NEC is rare. The exact pathogenesis of NEC in term infants with CHD is unknown, but it is hypothesized that ischemia of the intestines plays a pivotal […]

Kangaroo mother care (KMC) is an effective intervention for preterm and low birth weight infants. Effective implementation of KMC relies on a multidisciplinary team centering on the newborn’s caregiver, who delivers care with support from health care workers. This study explored the experiences of caregivers on the implementation of KMC.We conducted a descriptive qualitative study […]

Cardiovascular anomalies are the largest group of congenital anomalies and the major cause of death in young children, with various data linking rising atrial septal defect incidence (ASDI) with prenatal cannabis exposure. Objectives / Hypotheses. Is cannabis associated with ASDI in USA? Is this relationship causal?Geospatiotemporal cohort study, 1991-2016. Census populations of adults, babies, congenital […]

Although continuous subcutaneous insulin infusion therapy (ie, insulin pump therapy) is associated with improved metabolic control compared with multiple daily insulin injections in children with type 1 diabetes, it is unclear when it is best to start it after diagnosis. In this study, we aimed to compare the outcomes between early and delayed start of […]

Galectin-3 is a new biomarker, which plays an important role in tissue inflammation, cardiac remodeling, and fibrosis. It can be readily measured in the circulation to detect early heart failure (HF). This study aimed to assess the value of galectin-3 assay in early diagnosis of children with heart failure secondary to congenital heart disease (CHD) […]

Hemiscrotal agenesis (HSA) is an exceedingly rare congenital anomaly in scrotal development. It is characterized by unilateral absence of scrotal skin with intact midline raphe. In the English literature, only seven patients were diagnosed with HSA. Herein, we report a 14-month-old boy with HSA, unilateral cryptorchidism and a perineal skin tag. Additionally, the patient had […]

Parents of adolescents and young adults (AYAs) with serious illness experience enormous stress as they navigate their child’s illness. In this study, we aimed to elucidate AYA parental perspectives on the advanced cancer experience, including what parents find challenging and their sources of strength.Parents of AYAs aged 14 to 24 years old being treated for […]

Changes in left ventricular structure and function have been previously described in children with obstructive sleep apnea (OSA). We aimed to determine if these structural and functional cardiac changes are reversible after treatment of OSA with adenotonsillectomy. Children aged 5-13 years with OSA and matched healthy controls were recruited. Adenotonsillectomy occurred within 1 month after […]

Noonan syndrome is a multiorgan system disorder mediated by genetic defects along the rat sarcoma-mitogen-activated protein kinase (RAS-MAPK) pathway known as RASopathies. It is the second most common syndromic cause of congenital heart disease and, in ∼20% of the cases, is associated with severe lymphatic disorders, including chylothorax and protein-losing enteropathy. Recently, we reported on […]

With improvements in survival rates in newborns with congenital heart defects (CHDs), focus has now shifted toward enhancing neurodevelopmental outcomes across their life span.To systematically review the prevalence and extent of motor difficulties in infants, children, and adolescents with CHD requiring open-heart surgery.Data sources included Embase, Medline and the Cumulative Index to Nursing and Allied […]

Multisystem inflammatory syndrome in children (MIS-C) has spread through the pediatric population during the coronavirus disease 2019 pandemic. Our objective for the study was to report the prevalence of conduction anomalies in MIS-C and identify predictive factors for the conduction abnormalities.We performed a single-center retrospective cohort study of pediatric patients <21 years of age presenting […]

Prevalence of hypertension (both primary and secondary) in children and adolescents in the US ranges from 3% to 4%. Primary hypertension in children and adolescents occurs primarily in children older than 13 years and has no known cause but is associated with several risk factors, including family history and higher body mass index. Secondary hypertension […]

We describe characteristics and outcomes of contemporary pediatric hypertrophic cardiomyopathy (PHC) patients. We studied 398 consecutive pediatric HC patients (<18 years, median 14 years, 65% boys) seen at our center between 2002-2018. Baseline clinical and pediatric echocardiographic data was collected. Left ventricular outflow tract gradient (LVOTG), LV fractional shortening and Z-score for left ventricular (LV) […]

Our aim was to assess the risk of cardiovascular disease (CVD) mortality in US 5-year survivors of adolescent and young adult (AYA) cancer compared with those of the general population and contemporaneous 5-year survivors of childhood cancer.A total of 160 834 5-year AYA cancer survivors (aged 15-39 years at diagnosis) were included, representing 2 239 […]

Women with Turner syndrome (TS) have high prevalence of cardiovascular anomalies. Literature suggests pregnancy is associated with a higher dissection risk, presumably preceded by aortic dilatation. Whether the aortic diameter truly changes during pregnancy in TS is not well investigated. This study aims to evaluate ascending aortic diameter change during pregnancy and reports on cardiac […]

Pediatric patients with genetic disorders have a higher incidence of pulmonary arterial hypertension (PAH) regardless of their heart defects. Filamin A (FLNA) mutation is recently recognized to be associated with pediatric pulmonary disorders, however, the clinical courses of PAH related to the mutation were reported in limited cases. Here, we presented a case and pooled […]

A double aortic arch (DAA) is increasingly identified before birth; however, there are no published data describing the postnatal outcome of a large prenatal cohort.To describe the associations, symptoms and impact of prenatally diagnosed DAA.Retrospective review of consecutive cases seen at two fetal cardiology units from 2014 to 2019. Clinical records including symptoms and assessment […]

This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention.This was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 […]

Postnatal ductal closure is stimulated by rising oxygen tension and withdrawal of vasodilatory mediators (prostaglandins, nitric oxide, adenosine) and by vasoconstrictors (endothelin-1, catecholamines, contractile prostanoids), ion channels, calcium flux, platelets, morphologic maturity, and a favorable genetic predisposition. A persistently patent ductus arteriosus (PDA) in preterm infants can have clinical consequences. Decreasing pulmonary vascular resistance, especially […]

Acute flaccid myelitis (AFM) is characterised by rapid onset of limb weakness with spinal cord grey-matter abnormalities on MRI scan. We aimed to assess whether detection of enterovirus in respiratory or other specimens can help predict prognosis in children with AFM.In this nationwide, longitudinal study, we evaluated the significance of detection of enterovirus in any […]

To determine ifVery low dose mydriatic eye microdrop regimen sufficiently dilates the pupil (above 4.1 mm) compared with the currently used low dose mydriatic eye microdrop regimen.Cardiovascular, gastrointestinal and respiratory adverse effects occur following eye drop instillation.Seventeen premature infants were recruited into this prospective, randomised controlled pilot trial in January 2017 to November 2018. Data were […]

X-linked lymphoproliferative disease (XLP) is a rare inherited X-linked primary immunodeficiency diseases (PID). One such disease, X-linked inhibitor of apoptosis protein (XIAP) deficiency, is characterized by Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH). However, EBV-HLH with coronary artery dilation and acute renal injury (AKI) in children is unusual.We report the case of a young boy aged 17 months […]

Ninety percent of intrapartum-related neonatal deaths are attributable to respiratory depression, with the vast majority of these deaths occurring in low- and lower-middle-income countries. Neonatal resuscitation training with Helping Babies Breathe (HBB) decreases mortality from respiratory depression. Cardiorespiratory monitoring in conjunction with HBB can provide valuable resuscitation feedback for both training and bedside purposes. In […]

Bystander training in cardiopulmonary resuscitation (CPR) is crucial to improve the victims’ survival and quality of life after sudden cardiac arrest. This quasi-experimental study aimed to determine the success rate of two different programs of CPR-training for children, adolescents, and adults in school communities. We assessed the development and acquisition of the following CPR-skills: checking […]

Cardiac T2* MRI (T2*CMR), for accurate estimation of myocardial siderosis, was introduced as part of a QI collaborative to optimise chelation therapy in order to improve cardiac morbidity in transfusion dependent thalassaemia (TDT) patients. We report the impact of this QI initiative from two thalassaemia centres from this collaborative.A key driver based quality initiative was […]

Childhood obesity represents a serious global health crisis. Apelin and its receptor system are widely distributed throughout the central nervous system and have been demonstrated to serve a role modulating feeding behaviour and energy homeostasis. The purposes of this study were to examine apelin concentrations and anthropometric-cardiometabolic parameters in obese and non-obese children and to […]

Young children and those with chronic medical conditions are at risk for complications of influenza including cardiopulmonary compromise. Here we aim to examine risks of mortality, clinical complications in children with congenital heart disease (CHD) hospitalized for influenza.We analyzed data from in-hospital pediatric patients from 2003, 2006, 2009, 2012 and 2016 using the nationally representative […]

The Paediatric Admission Guidance in the Emergency Department (PAGE) score is an assessment tool currently in development that helps predict hospital admission using components including patient characteristics, vital signs (heart rate, temperature, respiratory rate and oxygen saturation) and clinical features (eg, breathing, behaviour and nurse judgement). It aims to assist in safe admission and discharge […]

The identification of life-threatening infection in febrile children presenting to the emergency department (ED) remains difficult. The quick Sequential Organ Failure Assessment (qSOFA) was only derived for adult populations, implying an urgent need for pediatric scores. We developed and validated a novel, adapted qSOFA score (Liverpool quick Sequential Organ Failure Assessment [LqSOFA]) and compared its […]

Low socioeconomic status (SES) has emerged as an important risk factor for higher short-term mortality and neurodevelopmental outcomes in children with hypoplastic left heart syndrome and related anomalies; yet little is known about how SES affects these outcomes over the long-term.We linked data from the Single Ventricle Reconstruction trial to US Census Bureau data to […]

Haemoptysis is an uncommon presenting symptom in children and is usually caused by acute lower respiratory tract infection or foreign body aspiration. We report a rare case of right unilateral pulmonary vein atresia (PVA) as the underlying aetiology of recurrent haemoptysis in a child.A 4 years old girl presented with history of recurrent haemoptysis. Bronchoscopic evaluation […]