Alarming rate of liver cirrhosis after the small conduit extracardiac Fontan: A comparative analysis with the lateral tunnel.

Background: The association between the prevalence of cirrhosis and the types of Fontan operations remains unclear.

Methods: We conducted a retrospective chart review of 332 patients who underwent the Fontan procedure at our institution. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis.

Association Between Remote Monitoring and Interstage Morbidity and Death in Patients With Single-Ventricle Heart Disease Across Socioeconomic Groups.

Background: Despite improvements in survival over time, the mortality rate for infants with single-ventricle heart disease remains high. Infants of low socioeconomic status (SES) are particularly vulnerable. We sought to determine whether use of a novel remote monitoring program, the Cardiac High Acuity Monitoring Program, mitigates differences in outcomes by SES.

Alterations in diastolic function and cardiac geometry in children: a longitudinal study across the spectrum of dialysis and transplant.

Background: Children with kidney failure have increased risk for cardiovascular morbidities before and after transplantation. Ejection fraction is often preserved, masking cardiac dysfunction until severe. Data on longitudinal changes in diastolic function and cardiac geometry are limited.

Effect of levocarnitine supplementation on myocardial strain in children with acute kidney injury receiving continuous kidney replacement therapy: a pilot study.

Background: Carnitine plays a key role in energy production in the myocardium and is efficiently removed by continuous kidney replacement therapy (CKRT). Effects of levocarnitine supplementation on myocardial function in children receiving CKRT have not been investigated.

Methods: This controlled pilot cohort study of 48 children investigated effects of levocarnitine supplementation on myocardial strain in children receiving CKRT for acute kidney injury (AKI).

Effectiveness and Safety of Statin Therapy in Children: A Real-World Clinical Practice Experience.

Background: Statin use for hypercholesterolemia in children is predominantly reported from short-term clinical trials. In this study, we assess the efficacy and safety of statin treatment in clinical pediatric practice.

Methods: Records of all patients who began statin treatment at age <18 years and remained on statins for >6 months from 5 pediatric lipid clinics were reviewed.

Children with Heterozygous Familial Hypercholesterolemia in the United States: Data from the Cascade Screening for Awareness and Detection-FH Registry.

Objective: To describe enrollment characteristics of youth in the Cascade Screening for Awareness and Detection of FH Registry.

Study Design: This is a cross-sectional analysis of 493 participants aged <18 years with heterozygous familial hypercholesterolemia recruited from US lipid clinics (n = 20) between April 1, 2014, and January 12, 2018. At enrollment, some were new patients and some were already in care.

Update on Preventive Cardiology.

Although progress had been made in reducing cardiovascular disease (CVD) mortality, the positive trend has reversed in recent years, and CVD remains the most common cause of mortality in US women and men. Youth represent the future of CVD prevention; emerging evidence suggests exposure to risk factors in children contributes to atherosclerosis and results in vascular changes and increased CVD events. The contributors to CVD include those commonly seen in adults.

Circulating Donor Specific Antibodies and Carotid Intima-media Thickness in Pediatric Kidney Transplant Recipients, A Pilot Study.

The presence of circulating donor specific anti-HLA antibodies (dnDSA) has been implicated in an immune-mediated form of accelerated systemic arteriosclerosis in adult heart and kidney transplant recipients, however this has not been previously investigated in pediatric kidney transplant recipients. Carotid intima-media thickness (CIMT) is a reliable method for detection of arteriosclerosis. We hypothesized that children who develop dnDSA after kidney transplant would have increased CIMT compared with those who remain dnDSA negative.

Prevalence of unsuspected abnormal echocardiograms in adolescents with down syndrome.

The purpose of this article is to describe the prevalence of cardiac disease previously undiagnosed in healthy asymptomatic children and adolescents with Down syndrome (DS). Subjects with DS ages 10-20 years were recruited from two sites, the Children's Hospital of Philadelphia (Philadelphia, PA) and Children's National Health System (Washington, DC) for a cross-sectional study of body composition and cardiometabolic risk. Echocardiographic and clinical data were collected from patients enrolled in the parent study of cardiometabolic risk.

Relationships of Body Composition to Cardiac Structure and Function in Adolescents With Down Syndrome are Different than in Adolescents Without Down Syndrome.

Median survival in Down syndrome (DS) is 60 years, but cardiovascular disease risk and its markers such as left ventricular mass (LVM) have received limited attention. In youth, LVM is typically scaled to height as a surrogate for lean body mass (LBM), the strongest predictor of LVM, but whether this algorithm applies to DS, a condition which features short stature, is unknown. To examine the relationships of LVM and function with height, LBM, and moderate-to-vigorous physical activity(MVPA) in DS, DS youth aged 10-20 years, and age-, sex-, BMI-, race-matched nonDS controls underwent echocardiography for LVM, ejection fraction (EF), and left ventricular diastolic function (measured as E/E'); dual-energy X-ray absorptiometry (DXA)-measured LBM; accelerometry for MVPA.

Comparison of BMI, waist circumference, and waist-to-height ratio for identification of subclinical cardiovascular risk in pediatric kidney transplant recipients.

Background: Kidney transplant recipients are at high risk for CV morbidity. However, the measure of obesity that best predicts CV risk has not been established.

Objective: A prospective, controlled study was conducted to compare the ability of BMI, WC, and WHr to identify CV risk in pediatric kidney transplant recipients.

Community study to uncover the full spectrum of rheumatic heart disease in Uganda.

Objective: Estimates of the prevalence of rheumatic heart disease (RHD) in many endemic countries are limited to samples of children attending schools, which generate an incomplete picture of disease burden in communities. The present study conducted household-based RHD screening in a representative community in Gulu district, Uganda.

Methods: Members of households identified through a two-stage cluster-sampling approach between the ages of 5 years and 50 years were invited to undergo limited cardiac testing with a handheld echocardiogram to assess for the presence of RHD.

Recurrent Cardiology Evaluation for Innocent Heart Murmur: Echocardiogram Utilization.

We conducted a retrospective study to identify electrocardiogram (ECG) and echocardiogram utilization among patients presenting for a follow-up cardiology evaluation with innocent heart murmur between 2012 and 2014. The 2014 echocardiogram Appropriate Use Criteria was applied. We observed high rates of ordering ECGs and echocardiograms on follow-up visits (79% and 36%); only 1 patient had an appropriate indication for echocardiogram while the rest had rarely appropriate indication.

Correction to: Effects of obesity and metabolic syndrome on cardiovascular outcomes in pediatric kidney transplant recipients: a longitudinal study.

As originally published, this article contained errors owing to oversights in typesetting. The article has now been amended accordingly.

Cardiovascular effects of metabolic syndrome after transplantation: convergence of obesity and transplant-related factors.

Children are at increased risk of developing metabolic syndrome (MS) after kidney transplantation, which contributes to long-term cardiovascular (CV) morbidities and decline in allograft function. While MS in the general population occurs due to excess caloric intake and physical inactivity, additional chronic kidney disease and transplant-related factors contribute to the development of MS in transplant recipients. Despite its significant health consequences, the interplay of the individual components in CV morbidity in pediatric transplant recipients is not well understood.

Increased carotid intima-media thickness in African American pediatric kidney transplant recipients.

Early signs of subclinical CV dysfunction can be detected by ultrasound for CIMT. Although A-A are at high risk for CV disease, CIMT of A-A kidney transplant recipients has not been previously investigated. The aim of this prospective, controlled, longitudinal study was to investigate determinants of CIMT in a multiracial pediatric kidney transplant population, with a focus on A-A.

Treatment Strategies for Hypercholesterolemia.

Background: Atherosclerotic disease is a leading cause of morbidity and mortality in adults and is generally thought of as only affecting adults. However, the pathologic changes in vessels leading to atherosclerosis, and an increased risk of cardiovascular disease, have been shown to begin in early adolescence.

Objectives: There is a growing body of literature suggesting that earlier treatment, through lifestyle changes and pharmacotherapy, can help reduce this risk.

Effects of obesity and metabolic syndrome on cardiovascular outcomes in pediatric kidney transplant recipients: a longitudinal study.

Background: Obesity and metabolic syndrome (MS) are common after kidney transplantation, but their contribution to adverse cardiovascular (CV) outcomes in children are not well known. A prospective, controlled, longitudinal cohort study was conducted to investigate the effects of obesity and MS on left ventricular hypertrophy (LVH) and myocardial strain in pediatric kidney transplant recipients.

Methods: Transplant recipients (n = 42) had anthropometrics [body mass index (BMI), waist circumference, waist-to-height ratio], biochemical parameters (fasting glucose, lipid panel, HbA1c%), and echocardiogram with speckle tracking analysis for strain measured at 1, 18, and 30 months post-transplant.

The Impact of a Designated Cardiology Team Involving Telemedicine Home Monitoring on the Care of Children with Single-Ventricle Physiology After Norwood Palliation.

We evaluated the effect of an interdisciplinary single-ventricle task force (SVTF) that utilizes a family-driven, telemedicine home monitoring program on clinical outcomes of stage II admissions and its acceptance by parents and cardiologists. Study population was divided into two cohorts, one with Norwood surgery dates before the SVTF (pre-SVTF) and one interventional (post-SVTF). Post-SVTF data also included surveys of parents and cardiologists on the efficacy of the SVTF.

Quality improvement through collaboration: the National Pediatric Quality improvement Collaborative initiative.

Purpose Of Review: The National Pediatric Quality Improvement Collaborative (NPCQIC) was established to improve outcomes and quality of life in children with hypoplastic left heart syndrome and other single ventricle lesions requiring a Norwood operation. The NPCQIC consists of a network of providers and families collecting longitudinal data, conducting research, and using quality improvement science to decrease variations in care, develop and spread best practices, and decrease mortality.

Recent Findings: Initial descriptive investigation of the collaborative data found interstage care process variations, different surgical strategies, diverse feeding practices, and variable ICU approaches between centers and within sites.

Utility of hand-held echocardiography in outpatient pediatric cardiology management.

Adult patient series have shown hand-held echocardiography (echo) units (HHE) to be accurate for rapid diagnosis and triage. This is the first study to evaluate the ability of HHE to inform decision making in outpatient pediatric cardiology. New pediatric cardiology patients in outpatient clinics staffed by six pediatric cardiologists (experience 1-17 years) were prospectively enrolled if an echocardiogram (echo) was ordered during their initial visit.

Abnormal cardiac strain in children and young adults with HIV acquired in early life.

Background: Traditional measures of cardiac function are now often normal in adolescents and young adults treated with antiretroviral therapy for human immunodeficiency virus (HIV) infection. There is, however, evidence of myocardial abnormalities in adults with HIV. Cardiac strain analysis may detect impairment in cardiac function that may be missed by conventional measurements in this population.

High rate of coronary artery abnormalities in adolescents and young adults infected with human immunodeficiency virus early in life.

We completed a cross-sectional study of individuals infected with human immunodeficiency virus in early childhood using cardiac magnetic resonance imaging and magnetic resonance angiography. Coronary artery abnormality (CAA) was defined by the presence of luminal narrowing and irregularity of the coronary vessel wall. More than 50% of participants (14/27) had evidence of CAA.

Ezetimibe treatment of pediatric patients with hypercholesterolemia.

Objective: To review the efficacy of ezetimibe monotherapy for treatment of hypercholesterolemia in pediatric patients.

Study Design: This is a retrospective review of all pediatric patients who received ezetimibe monotherapy as treatment for hypercholesterolemia and for whom follow-up clinical and lipid results were available. Of 36 identified patients, 26 had lipoprotein profiles suggestive of familial hypercholesterolemia (FH), and 10 had profiles suggestive of familial combined hyperlipidemia (FCHL).

Phenotype and course of Hutchinson-Gilford progeria syndrome.

Background: Hutchinson-Gilford progeria syndrome is a rare, sporadic, autosomal dominant syndrome that involves premature aging, generally leading to death at approximately 13 years of age due to myocardial infarction or stroke. The genetic basis of most cases of this syndrome is a change from glycine GGC to glycine GGT in codon 608 of the lamin A (LMNA) gene, which activates a cryptic splice donor site to produce abnormal lamin A; this disrupts the nuclear membrane and alters transcription.

Methods: We enrolled 15 children between 1 and 17 years of age, representing nearly half of the world's known patients with Hutchinson-Gilford progeria syndrome, in a comprehensive clinical protocol between February 2005 and May 2006.