Jennifer Shiunroh Li

Journal Article medRxiv · February 12, 2025 BACKGROUND: Prophylactic steroids are often used to reduce the systemic inflammatory response to cardiopulmonary bypass in infants undergoing heart surgery. The STRESS trial found that the likelihood of a worse outcome did not differ between infants random ... Full text Link to item Cite

Using Parks and Recreation Providers to Enhance Obesity Treatment: A Randomized Controlled Trial.

Journal Article Pediatrics · February 10, 2025 OBJECTIVE: Intensive health behavior and lifestyle treatment (IHBLT) is recommended for children aged 6-18 years with obesity. The objective was to evaluate the effectiveness of Fit Together, a health care and parks and recreation partnership to deliver IH ... Full text Link to item Cite

Racial and Educational Isolation are Associated with Worse Outcomes in Congenital Heart Disease.

Journal Article Pediatr Cardiol · January 15, 2025 Sociodemographic factors influence outcomes in children with congenital heart disease (CHD). We predict an association between measures of social isolation and outcomes in infants with complex CHD. These measures, racial (RI) and educational (EI) isolation ... Full text Link to item Cite

Correlating Severity of Pulmonary Hypertension by Echocardiogram with Mortality in Premature Infants with Bronchopulmonary Dysplasia.

Journal Article Am J Perinatol · December 2024 OBJECTIVE: Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth. Infants with BPD are at increased risk for pulmonary hypertension (PH). Cardiac catheterization is the gold standard for diagnosing PH, but cardiac catheterizati ... Full text Link to item Cite

Novel linkage of the Society of Thoracic Surgeons database and hospital electronic health records.

Journal Article Cardiol Young · December 2024 BACKGROUND: Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital heart surgery database worldwide but does not provide information beyond primary episode of care. Linkage to hospital electronic health records would captu ... Full text Link to item Cite

Boys With Duchenne Muscular Dystrophy Have Diastolic Dysfunction Based on CMR.

Journal Article Circ Cardiovasc Imaging · December 2024 BACKGROUND: Cardiomyopathy is the leading cause of death in boys with Duchenne muscular dystrophy (DMD). While cardiac magnetic resonance (CMR) is routinely used to assess fibrosis and left ventricular (LV) ejection fraction, CMR measures of LV filling and ... Full text Link to item Cite

Identifying novel data-driven subgroups in congenital heart disease using multi-modal measures of brain structure.

Journal Article Neuroimage · August 15, 2024 Individuals with congenital heart disease (CHD) have an increased risk of neurodevelopmental impairments. Given the hypothesized complexity linking genomics, atypical brain structure, cardiac diagnoses and their management, and neurodevelopmental outcomes, ... Full text Link to item Cite

The Association of Race and Ethnicity with Mortality in Pediatric Patients with Congenital Heart Disease: a Systematic Review.

Journal Article J Racial Ethn Health Disparities · August 2024 CONTEXT: Congenital heart disease (CHD) is a common condition with high morbidity and mortality and is subject to racial and ethnic health disparities. OBJECTIVE: To conduct a systematic review of the literature to identify differences in mortality in pedi ... Full text Link to item Cite

Cardiovascular disease risk factors in congenital heart disease survivors are associated with heart failure.

Journal Article Pediatr Res · July 5, 2024 BACKGROUND: Despite advances in treatment and survival, individuals with congenital heart defects (CHD) have a higher risk of heart failure (HF) compared to the general population. OBJECTIVE: To evaluate comorbidities associated with HF in patients with CH ... Full text Link to item Cite

Racial and Ethnic Disparities in Health Care Usage and Death by Neighborhood Poverty Among Individuals With Congenital Heart Defects, 4 US Surveillance Sites, 2011 to 2013.

Journal Article J Am Heart Assoc · June 4, 2024 BACKGROUND: Socioeconomic factors may lead to a disproportionate impact on health care usage and death among individuals with congenital heart defects (CHD) by race, ethnicity, and socioeconomic factors. How neighborhood poverty affects racial and ethnic d ... Full text Link to item Cite

Risk Factors and Outcomes Associated with Gaps in Care in Children with Congenital Heart Disease.

Conference Pediatr Cardiol · June 2024 Adults with congenital heart disease (CHD) benefit from cardiology follow-up at recommended intervals of ≤ 2 years. However, benefit for children is less clear given limited studies and unclear current guidelines. We hypothesize there are identifiable risk ... Full text Link to item Cite

Evaluating registry-based trial economics: Results from the STRESS clinical trial.

Journal Article Contemp Clin Trials Commun · April 2024 BACKGROUND: Registry-based trials have the potential to reduce randomized clinical trial (RCT) costs. However, observed cost differences also may be achieved through pragmatic trial designs. A systematic comparison of trial costs across different designs h ... Full text Link to item Cite

Physical activity and sleep changes among children during the COVID-19 pandemic.

Journal Article NPJ Digit Med · March 16, 2024 Daily routines, including in-person school and extracurricular activities, are important for maintaining healthy physical activity and sleep habits in children. The COVID-19 pandemic significantly disrupted daily routines as in-person school and activities ... Full text Link to item Cite

Coronary Artery Aneurysm Rupture in Kawasaki Disease and SARS-CoV-2 Infection.

Journal Article CASE (Phila) · February 2024 • CAA rupture is a rare, life-threatening complication of KD. • SARS-CoV-2 and KD may have a synergistic effect resulting in severe inflammation. • Recognizing specific echocardiogram findings is necessary to diagnosis CAA rupture. ... Full text Open Access Link to item Cite

Development and Temporal Validation of a Machine Learning Model to Predict Clinical Deterioration.

Journal Article Hosp Pediatr · January 1, 2024 OBJECTIVES: Early warning scores detecting clinical deterioration in pediatric inpatients have wide-ranging performance and use a limited number of clinical features. This study developed a machine learning model leveraging multiple static and dynamic clin ... Full text Link to item Cite

Distinguishing between translational science and translational research in CTSA pilot studies: A collaborative project across 12 CTSA hubs.

Journal Article J Clin Transl Sci · 2024 INTRODUCTION: The institutions (i.e., hubs) making up the National Institutes of Health (NIH)-funded network of Clinical and Translational Science Awards (CTSAs) share a mission to turn observations into interventions to improve public health. Recently, th ... Full text Link to item Cite

Improving Outcomes for Infants After Cardiopulmonary Bypass Surgery for Congenital Heart Disease: A Commentary on Recent Randomized Controlled Trials.

Journal Article Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies · November 2023 Full text Cite

Impact of Prenatal Diagnosis of Critical Congenital Heart Disease on Preoperative and Postoperative Outcomes.

Journal Article Pediatr Cardiol · October 2023 The objective of this study was to assess the relationship of prenatal diagnosis of critical congenital heart disease (CHD) to preoperative and postoperative patient findings. Retrospective analysis of neonates with critical CHD who underwent cardiothoraci ... Full text Link to item Cite

Risk of in-hospital Deterioration for Children with Single Ventricle Physiology.

Journal Article Pediatr Cardiol · August 2023 Children with single ventricle physiology (SV) are at high risk of in-hospital morbidity and mortality. Identifying children at risk for deterioration may allow for earlier escalation of care and subsequently decreased mortality.We conducted a retrospectiv ... Full text Link to item Cite

American Heart Association's Children's Strategically Focused Research Network Experience.

Journal Article J Am Heart Assoc · April 4, 2023 The American Heart Association's Strategically Focused Children's Research Network started in July 2017 with 4 unique programs at Children's National Hospital in Washington, DC; Duke University in Durham, North Carolina; University of Utah in Salt Lake Cit ... Full text Link to item Cite

Methylprednisolone for Heart Surgery in Infants. Reply.

Journal Article N Engl J Med · March 9, 2023 Full text Link to item Cite

Third-Grade Academic Performance and Episodes of Cardiac Care Among Children with Congenital Heart Defects.

Conference Pediatr Cardiol · February 2023 Children with congenital heart defects (CHDs) are at risk for poor academic performance. The degree to which receipt of health care services is associated with adverse academic outcomes is not known. We examined the association between episodes of cardiac ... Full text Link to item Cite

Association of Potentially Damaging De Novo Gene Variants With Neurologic Outcomes in Congenital Heart Disease.

Journal Article JAMA Netw Open · January 3, 2023 IMPORTANCE: Neurodevelopmental disabilities are commonly associated with congenital heart disease (CHD), but medical and sociodemographic factors explain only one-third of the variance in outcomes. OBJECTIVE: To examine whether potentially damaging de novo ... Full text Link to item Cite

Changes in Drug Development Regulations and Their Impact on Clinical Trials

Chapter · January 1, 2023 Regulatory changes in the United States and Europe have stimulated major pediatric clinical trials of >18 different antihypertensive agents over the last two decades. As pediatric hypertension trials have become more common, trial designs have been refined ... Full text Cite

Effect Of The Covid-19 Pandemic On Activity And Sleep In Children And Adolescents With Obesity

Conference MEDICINE & SCIENCE IN SPORTS & EXERCISE · 2023 Cite

Methylprednisolone for Heart Surgery in Infants - A Randomized, Controlled Trial.

Journal Article N Engl J Med · December 8, 2022 BACKGROUND: Although perioperative prophylactic glucocorticoids have been used for decades, whether they improve outcomes in infants after heart surgery with cardiopulmonary bypass is unknown. METHODS: We conducted a multicenter, prospective, randomized, p ... Full text Link to item Cite

Ferumoxytol-Enhanced Cardiac Magnetic Resonance Angiography and 4D Flow: Safety and Utility in Pediatric and Adult Congenital Heart Disease

Journal Article Children · December 1, 2022 Cardiac magnetic resonance imaging and angiography have a crucial role in the diagnostic evaluation and follow up of pediatric and adult patients with congenital heart disease. Although much of the information required of advanced imaging studies can be pr ... Full text Cite

Disparities in Loss to Follow-Up Among Adults With Congenital Heart Disease in North Carolina.

Journal Article World J Pediatr Congenit Heart Surg · November 2022 BACKGROUND: The AHA/ACC Adult Congenital Heart Disease guidelines recommend that most adults with congenital heart disease (CHD) follow-up with CHD cardiologists every 1 to 2 years because longer gaps in care are associated with adverse outcomes. This stud ... Full text Link to item Cite

Health Care Usage Among Adolescents With Congenital Heart Defects at 5 Sites in the United States, 2011 to 2013.

Journal Article J Am Heart Assoc · September 20, 2022 Background We sought to characterize health care usage for adolescents with congenital heart defects (CHDs) using population-based multisite surveillance data. Methods and Results Adolescents aged 11 to 18 years with ≥1 CHD-related diagnosis code and resid ... Full text Link to item Cite

How Well Do ICD-9-CM Codes Predict True Congenital Heart Defects? A Centers for Disease Control and Prevention-Based Multisite Validation Project.

Journal Article Journal of the American Heart Association · August 2022 Background The Centers for Disease Control and Prevention's Surveillance of Congenital Heart Defects Across the Lifespan project uses large clinical and administrative databases at sites throughout the United States to understand population-based congenita ... Full text Cite

Association of Acute Anti-inflammatory Treatment With Medium-term Outcomes for Coronary Artery Aneurysms in Kawasaki Disease.

Journal Article CJC Pediatr Congenit Heart Dis · August 2022 BACKGROUND: The impact of adjunctive anti-inflammatory treatment on outcomes for patients with Kawasaki disease (KD) and coronary artery aneurysms (CAAs) is unknown. METHODS: Using data from the International KD Registry in patients with ≥ medium CAA we ev ... Full text Link to item Cite

Cumulative In-Hospital Costs Associated With Single-Ventricle Palliation.

Journal Article JACC Adv · June 2022 BACKGROUND: In the SVR (Single Ventricle Reconstruction) Trial, 1-year survival in recipients of right ventricle to pulmonary artery shunts (RVPAS) was superior to that in those receiving modified Blalock-Taussig-Thomas shunts (MBTTS), but not in subsequen ... Full text Link to item Cite

Dosing Regimen Prediction and Confirmation With Rivaroxaban for Thromboprophylaxis in Children After the Fontan Procedure: Insights From the Phase III UNIVERSE Study.

Journal Article J Clin Pharmacol · February 2022 Thrombosis remains an important complication for children with single-ventricle physiology following the Fontan procedure, and effective thromboprophylaxis is an important unmet medical need. To obviate conventional dose-finding studies and expedite clinic ... Full text Link to item Cite

Clinically Suspected Myocarditis Temporally Related to COVID-19 Vaccination in Adolescents and Young Adults: Suspected Myocarditis After COVID-19 Vaccination.

Journal Article Circulation · February 2022 BACKGROUND: Understanding the clinical course and short-term outcomes of suspected myocarditis after the coronavirus disease 2019 (COVID-19) vaccination has important public health implications in the decision to vaccinate youth. METHODS: We retrospectivel ... Full text Link to item Cite

Variation in Pharmacologic Management of Patients with Kawasaki Disease with Coronary Artery Aneurysms.

Journal Article J Pediatr · January 2022 OBJECTIVE: To evaluate practice variation in pharmacologic management in the International Kawasaki Disease Registry (IKDR). STUDY DESIGN: Practice variation in intravenous immunoglobulin (IVIG) therapy, anti-inflammatory agents, statins, beta-blockers, an ... Full text Link to item Cite

A systematic review of the evidence supporting post-operative antithrombotic use following cardiopulmonary bypass in children with CHD.

Journal Article Cardiol Young · January 2022 OBJECTIVES: To determine the optimal antithrombotic agent choice, timing of initiation, dosing and duration of therapy for paediatric patients undergoing cardiac surgery with cardiopulmonary bypass. METHODS: We used PubMed and EMBASE to systematically revi ... Full text Link to item Cite

The NHLBI Study on Long-terM OUtcomes after the Multisystem Inflammatory Syndrome In Children (MUSIC): Design and Objectives.

Journal Article American heart journal · January 2022 BackgroundThe Long-terM OUtcomes after the Multisystem Inflammatory Syndrome In Children (MUSIC) study aims to characterize the frequency and time course of acute and long-term cardiac and non-cardiac sequelae in multisystem inflammatory syndrome ... Full text Cite

Association of Racial and Educational Isolation on Outcomes in Infants With Congenital Heart Disease

Conference CIRCULATION · 2022 Cite

Risk Factors for Sudden Infant Death in North Carolina

Conference PEDIATRICS · 2022 Cite

Steroids to Reduce Systemic Inflammation After Infant Heart Surgery: The STRESS Trial

Conference CIRCULATION · 2022 Cite

Thromboprophylaxis for Children Post-Fontan Procedure: Insights From the UNIVERSE Study.

Journal Article Journal of the American Heart Association · November 2021 Background Patients with single-ventricle physiology who undergo the Fontan procedure are at risk for thrombotic events associated with significant morbidity and mortality. The UNIVERSE Study evaluated the efficacy and safety of a novel liquid rivaroxaban ... Full text Cite

Weight-Related Behaviors of Children with Obesity during the COVID-19 Pandemic.

Journal Article Childhood obesity (Print) · September 2021 Background: During the coronavirus disease 2019 (COVID-19) pandemic, children and families have had to adapt their daily lives. The purpose of this study was to describe changes in the weight-related behaviors of children with obesity after t ... Full text Cite

Causes of Death in Infants and Children with Congenital Heart Disease.

Journal Article Pediatr Cardiol · August 2021 With improved surgical outcomes, infants and children with congenital heart disease (CHD) may die from other causes of death (COD) other than CHD. We sought to describe the COD in youth with CHD in North Carolina (NC). Patients from birth to 20 years of ag ... Full text Link to item Cite

Individuals aged 1-64 years with documented congenital heart defects at healthcare encounters, five U.S. surveillance sites, 2011-2013.

Journal Article Am Heart J · August 2021 BACKGROUND: Many individuals born with congenital heart defects (CHD) survive to adulthood. However, population estimates of CHD beyond early childhood are limited in the U.S. OBJECTIVES: To estimate the percentage of individuals aged 1-to-64 years at five ... Full text Link to item Cite

Risk Factors for Sudden Infant Death in North Carolina.

Journal Article Front Pediatr · 2021 Background: Sudden infant death syndrome (SIDS) is the sudden, unexplained death of infants <1 year old. SIDS remains a leading cause of death in US infants. We aim to identify associations between SIDS and race/ethnicity, birth weight/gestational age, and ... Full text Open Access Link to item Cite

Low-Molecular-Weight Heparin vs Warfarin for Thromboprophylaxis in Children With Coronary Artery Aneurysms After Kawasaki Disease: A Pragmatic Registry Trial.

Conference Can J Cardiol · October 2020 BACKGROUND: The substantial risk of thrombosis in large coronary artery aneurysms (CAAs) (maximum z-score ≥ 10) after Kawasaki disease (KD) mandates effective thromboprophylaxis. We sought to determine the effectiveness of anticoagulation (low-molecular-we ... Full text Link to item Cite

A Randomized, Controlled Pharmacokinetic and Pharmacodynamics Trial of Ambrisentan After Fontan Surgery.

Journal Article Pediatr Crit Care Med · September 2020 OBJECTIVES: To determine the pharmacokinetics, pharmacodynamics, and safety of the hepatically metabolized endothelin receptor antagonist, ambrisentan in children after Fontan surgery. DESIGN: Prospective, randomized, double-blind, placebo-controlled pharm ... Full text Link to item Cite

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From the International Kawasaki Disease Registry.

Journal Article J Am Heart Assoc · August 4, 2020 Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought ... Full text Link to item Cite

Overcoming underpowering: Trial simulations and a global rank end point to optimize clinical trials in children with heart disease.

Journal Article Am Heart J · August 2020 BACKGROUND: Randomized controlled trials (RCTs) in children with heart disease are challenging and therefore infrequently performed. We sought to improve feasibility of perioperative RCTs for this patient cohort using data from a large, multicenter clinica ... Full text Link to item Cite

Clinical and socio-economic predictors of work participation in adult CHD patients.

Journal Article Cardiol Young · August 2020 BACKGROUND: Adults with CHD have reduced work participation rates compared to adults without CHD. We aimed to quantify employment rate among adult CHD patients in a population-based registry and to describe factors and barriers associated with work partici ... Full text Link to item Cite

Flattening the (BMI) Curve: Timing of Child Obesity Onset and Cardiovascular Risk.

Journal Article Pediatrics · August 2020 Full text Link to item Cite

Causes of Death and Cardiovascular Comorbidities in Adults With Congenital Heart Disease.

Journal Article J Am Heart Assoc · July 21, 2020 Background Little is known about the contemporary mortality experience among adults with congenital heart disease (CHD). The objectives of this study were to assess the age at death, presence of cardiovascular comorbidities, and most common causes of death ... Full text Link to item Cite

Variants in ADRB1 and CYP2C9: Association with Response to Atenolol and Losartan in Marfan Syndrome.

Journal Article J Pediatr · July 2020 OBJECTIVE: To test whether variants in ADRB1 and CYP2C9 genes identify subgroups of individuals with differential response to treatment for Marfan syndrome through analysis of data from a large, randomized trial. STUDY DESIGN: In a subset of 250 white, non ... Full text Link to item Cite

Rationale and design of "Hearts & Parks": study protocol for a pragmatic randomized clinical trial of an integrated clinic-community intervention to treat pediatric obesity.

Journal Article BMC Pediatr · June 26, 2020 BACKGROUND: 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. Ch ... Full text Link to item Cite

Pulmonary Valve Endocarditis: The Potential Utility of Multimodal Imaging Prior to Surgery.

Journal Article World journal for pediatric & congenital heart surgery · March 2020 BackgroundThe presence of echocardiographic (echo) evidence is a major criterion for the diagnosis of infective endocarditis (IE) by modified Duke criteria. Pulmonary valve (PV) IE, however, can be challenging to identify by echo. We sought to eva ... Full text Cite

Rationale and design of the STeroids to REduce Systemic inflammation after infant heart Surgery (STRESS) trial.

Journal Article Am Heart J · February 2020 For decades, physicians have administered corticosteroids in the perioperative period to infants undergoing heart surgery with cardiopulmonary bypass (CPB) to reduce the postoperative systemic inflammatory response to CPB. Some question this practice becau ... Full text Link to item Cite

The Bayley-III scale may underestimate neurodevelopmental disability after cardiac surgery in infants.

Journal Article Eur J Cardiothorac Surg · January 1, 2020 OBJECTIVES: Neurodevelopmental disability is the most common complication among congenital heart surgery survivors. The Bayley scales are standardized instruments to assess neurodevelopment. The most recent edition (Bayley Scales of Infant and Toddler Deve ... Full text Link to item Cite

Distribution Of Cardiorespiratory Fitness Among Children In The Hearts And Parks Study

Conference MEDICINE & SCIENCE IN SPORTS & EXERCISE · 2020 Cite

Changes In Cardiorespiratory Fitness Among Children In The Hearts And Parks Heathy Lifestyle Intervention

Conference MEDICINE & SCIENCE IN SPORTS & EXERCISE · 2020 Cite

Behavior and Quality of Life at 6 Years for Children With Hypoplastic Left Heart Syndrome.

Journal Article Pediatrics · November 2019 OBJECTIVES: We measured behavioral, quality of life (QoL), and functional status outcomes for 6-year-old children with hypoplastic left heart syndrome enrolled in the Single Ventricle Reconstruction Trial. We sought to compare these outcomes with those in ... Full text Link to item Cite

Facilitating translational team science: The project leader model.

Journal Article J Clin Transl Sci · August 2019 Project management expertise is employed across many professional sectors, including clinical research organizations, to ensure that efforts undertaken by the organization are completed on time and according to specifications and are capable of achieving t ... Full text Link to item Cite

Rivaroxaban, a direct Factor Xa inhibitor, versus acetylsalicylic acid as thromboprophylaxis in children post-Fontan procedure: Rationale and design of a prospective, randomized trial (the UNIVERSE study).

Journal Article Am Heart J · July 2019 BACKGROUND: The Fontan procedure is the final step of the 3-stage palliative procedure commonly performed in children with single ventricle physiology. Thrombosis remains an important complication in children after this procedure. To date, guideline recomm ... Full text Link to item Cite

Acid Suppression Therapy and Symptom Improvement (or Lack Thereof) in Children.

Journal Article Pediatrics · July 2019 Full text Link to item Cite

Research Gaps in Primary Pediatric Hypertension.

Journal Article Pediatrics · May 2019 Hypertension affects >40% of the US population and is a major contributor to cardiovascular-related morbidity and mortality. Although less common among children and adolescents, hypertension affects 1% to 5% of all youth. The 2017 Clinical Practice Guideli ... Full text Link to item Cite

Agreement of an echocardiogram-based diagnosis of pulmonary hypertension in infants at risk for bronchopulmonary dysplasia among masked reviewers.

Journal Article J Perinatol · February 2019 OBJECTIVE: To evaluate the agreement of an echocardiogram-based pulmonary hypertension diagnosis in premature infants at risk for bronchopulmonary dysplasia (BPD). STUDY DESIGN: Echocardiograms from infants born ≤28 weeks post menstrual age were retrospect ... Full text Link to item Cite

Health-Related Quality of Life in Children and Young Adults with Marfan Syndrome.

Journal Article J Pediatr · January 2019 OBJECTIVE: To assess health-related quality of life (HRQOL) in a large multicenter cohort of children and young adults with Marfan syndrome participating in the Pediatric Heart Network Marfan Trial. STUDY DESIGN: The Pediatric Quality of Life Inventory (Pe ... Full text Link to item Cite

Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome.

Journal Article Pediatr Cardiol · October 2018 Few data exist regarding predictors of rapid aortic root dilation and referral for aortic surgery in Marfan syndrome (MFS). To identify independent predictors of the rate of aortic root (AoR) dilation and referral for aortic surgery, we investigated the da ... Full text Link to item Cite

Severe Cardiac Involvement Is Rare in Patients with Late-Onset Pompe Disease and the Common c.-32-13T>G Variant: Implications for Newborn Screening.

Journal Article The Journal of pediatrics · July 2018 Based on a review of a large patient cohort, published literature, and 3 newborn screening cohorts, we concluded that children diagnosed through newborn screening with late-onset Pompe disease and the common heterozygous c.-32-13T>G variant require frequen ... Full text Cite

Transplant-Free Survival and Interventions at 6 Years in the SVR Trial.

Conference Circulation · May 22, 2018 BACKGROUND: In the SVR trial (Single Ventricle Reconstruction), 1-year transplant-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock-Taussig shunt in patients with hypo ... Full text Link to item Cite

Recommendations to Enhance Pediatric Cardiovascular Drug Development: Report of a Multi-Stakeholder Think Tank.

Journal Article J Am Heart Assoc · February 10, 2018 Full text Link to item Cite

Effect of renal function on antihypertensive drug safety and efficacy in children.

Journal Article Pediatr Nephrol · January 2018 BACKGROUND: Hypertension and chronic kidney disease (CKD) are common comorbidities. Guidelines recommend treating hypertension in children with CKD because it is a modifiable risk factor for subsequent cardiovascular disease. Children with CKD are frequent ... Full text Link to item Cite

Changes in drug development regulations and their impact on clinical trials

Chapter · January 1, 2018 Regulatory changes in the United States and Europe have stimulated major pediatric clinical trials of over 15 different antihypertensive agents over the last two decades. With increased pediatric hypertension trial experience, trial designs have been refin ... Full text Cite

A Double Blind, Randomized, Controlled Pharmacokinetic and Pharmacodynamics Trial of Ambrisentan After Fontan Surgery

Conference CIRCULATION · 2018 Cite

Causes of Death in Adults With Congenital Heart Disease Differ by Lesion Severity

Conference CIRCULATION · 2018 Cite

Overcoming Underpowering: Simulation Study to Evaluate an STS Global Rank Endpoint for Trials in Congenital Heart Surgery

Conference CIRCULATION · November 14, 2017 Link to item Cite

Efficacy and safety of aspirin combined with warfarin after acute coronary syndrome : A meta-analysis.

Journal Article Herz · May 2017 A comprehensive meta-analysis was performed to investigate whether the combination of high-/low-dose of aspirin and various intensities of warfarin (W) offer greater benefit than aspirin (ASA) alone. A total of 14 randomized clinical trials (RCTs) having 2 ... Full text Link to item Cite

Impact of imaging approach on radiation dose and associated cancer risk in children undergoing cardiac catheterization.

Journal Article Catheter Cardiovasc Interv · April 2017 OBJECTIVES: To quantify the impact of image optimization on absorbed radiation dose and associated risk in children undergoing cardiac catheterization. BACKGROUND: Various imaging and fluoroscopy system technical parameters including camera magnification, ... Full text Link to item Cite

Congenital Heart Disease in Premature Infants 25-32 Weeks' Gestational Age.

Journal Article J Pediatr · February 2017 OBJECTIVE: To determine the birth prevalence of congenital heart defects (CHDs) across the spectrum of common defects in very/extremely premature infants and to compare mortality rates between premature infants with and without CHDs. STUDY DESIGN: The Kids ... Full text Link to item Cite

Severe Cardiomyopathy as the Isolated Presenting Feature in an Adult with Late-Onset Pompe Disease: A Case Report.

Journal Article JIMD Rep · 2017 Many inborn errors of metabolism can cause cardiomyopathy. Cardiomyopathy associated with glycogen storage includes PRKAG2-associated glycogen storage disease (GSD), Danon disease, infantile-onset Pompe disease (GSD II), GSD III, GSD IV, and phosphofructok ... Full text Link to item Cite

Respiratory syncytial virus hospitalisation trends in children with haemodynamically significant heart disease, 1997-2012.

Journal Article Cardiol Young · January 2017 OBJECTIVE: The aim of the study was to evaluate the trends in respiratory syncytial virus-related hospitalisations and associated outcomes in children with haemodynamically significant heart disease in the United States of America. Study design The Kids' I ... Full text Link to item Cite

Childhood Hypertension: An Underappreciated Epidemic?

Journal Article Pediatrics · December 2016 Full text Link to item Cite

Utility of Multi-modal Imaging in the Diagnosis of Pulmonary Valve Endocarditis

Conference CIRCULATION · November 11, 2016 Link to item Cite

Cardiopulmonary resuscitation in hospitalized infants.

Journal Article Early Hum Dev · October 2016 BACKGROUND: Hospitalized infants requiring cardiopulmonary resuscitation (CPR) represent a high-risk group. Recent data on risk factors for mortality following CPR in this population are lacking. AIMS: We hypothesized that infant demographic characteristic ... Full text Link to item Cite

Impact of Operative and Postoperative Factors on Neurodevelopmental Outcomes After Cardiac Operations.

Conference Ann Thorac Surg · September 2016 BACKGROUND: Neurodevelopmental disability is common after operations for congenital heart defects. We previously showed that patient and preoperative factors, center, and calendar year of birth explained less than 30% of the variance for the Psychomotor De ... Full text Link to item Cite

Maladaptive aortic properties after the Norwood procedure: An angiographic analysis of the Pediatric Heart Network Single Ventricle Reconstruction Trial.

Journal Article J Thorac Cardiovasc Surg · August 2016 OBJECTIVES: Aortic arch reconstruction in children with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe neoaortic anatomy and physiology, risk fa ... Full text Link to item Cite

Clinicians' Adherence to Guidelines on Evaluation of Hypertension in Children and Adolescents.

Journal Article World J Pediatr Congenit Heart Surg · July 2016 BACKGROUND: Hypertension is increasingly prevalent among children. We sought to review provider adherence to the National High Blood Pressure Education Program (NHBPEP) recommendations at a single academic medical center. METHODS: We identified children 3- ... Full text Link to item Cite

Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation.

Journal Article J Pediatr · March 2016 OBJECTIVES: To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan. STUDY DESIGN: Cross-sectional. The Pediatric Quality of Life Inventory (PedsQL) was administered to 408 survivors of Fo ... Full text Link to item Cite

Pharmacokinetics of intravenous sildenafil in children with palliated single ventricle heart defects: effect of elevated hepatic pressures.

Journal Article Cardiol Young · February 2016 UNLABELLED: Aims Sildenafil is frequently prescribed to children with single ventricle heart defects. These children have unique hepatic physiology with elevated hepatic pressures, which may alter drug pharmacokinetics. We sought to determine the impact of ... Full text Link to item Cite

Quality of Vitamin K Antagonist Control and 1-Year Outcomes in Patients with Atrial Fibrillation: A Global Perspective from the GARFIELD-AF Registry.

Journal Article PLoS One · 2016 AIMS: Vitamin K antagonists (VKAs) need to be individually dosed. International guidelines recommend a target range of international normalised ratio (INR) of 2.0-3.0 for stroke prevention in atrial fibrillation (AF). We analysed the time in this therapeut ... Full text Link to item Cite

Epidemiology and Mortality of Very and Extremely Preterm Infants With Congenital Heart Defects

Journal Article CIRCULATION · November 10, 2015 Link to item Cite

Task Force 8: Pediatric Cardiology Fellowship Training in Research and Scholarly Activity. SPCTPD/ACC/AAP/AHA.

Journal Article Circulation · August 11, 2015 Full text Link to item Cite

Task Force 8: Pediatric Cardiology Fellowship Training in Research and Scholarly Activity.

Journal Article J Am Coll Cardiol · August 11, 2015 Full text Link to item Cite

Paediatric cardiovascular clinical trials: an analysis of ClinicalTrials.gov and the Food and Drug Administration Pediatric Drug Labeling Database.

Conference Cardiol Young · August 2015 Recent regulatory initiatives in the United States of America and Europe have transformed the paediatric clinical trials landscape by significantly increasing capital investment and paediatric trial volume. The purpose of this manuscript was to review the ... Full text Link to item Cite

Neurodevelopmental outcomes after cardiac surgery in infancy.

Journal Article Pediatrics · May 2015 BACKGROUND: Neurodevelopmental disability is the most common complication for survivors of surgery for congenital heart disease (CHD). METHODS: We analyzed individual participant data from studies of children evaluated with the Bayley Scales of Infant Deve ... Full text Link to item Cite

Response to letters regarding article, "Cumulative radiation exposure and cancer risk estimation in children with heart disease".

Journal Article Circulation · April 21, 2015 Full text Link to item Cite

Implementation of management strategies for diabetes and hypertension: from local to global health in cardiovascular diseases.

Journal Article Glob Heart · March 2015 Diabetes and hypertension are chronic conditions that are growing in prevalence as major causal factors of cardiovascular disease (CVD). The need for chronic-illness surveillance, population-risk management, and successful treatment interventions are cruci ... Full text Link to item Cite

Maladaptive Aortic Properties after the Norwood Procedure: an Angiographic Analysis of the Pediatric Heart Network Single Ventricle Reconstruction Trial

Journal Article CIRCULATION · November 25, 2014 Link to item Cite

Comparative effectiveness of digoxin and propranolol for supraventricular tachycardia in infants.

Conference Pediatr Crit Care Med · November 2014 OBJECTIVES: Supraventricular tachycardia is the most common arrhythmia in infants, and antiarrhythmic medications are frequently used for prophylaxis. The optimal prophylactic antiarrhythmic medication is unknown, and prior randomized trials have been unde ... Full text Link to item Cite

Excess costs associated with complications and prolonged length of stay after congenital heart surgery.

Journal Article Ann Thorac Surg · November 2014 BACKGROUND: While there is an increasing emphasis on both optimizing quality of care and reducing health care costs, there are limited data regarding how to best achieve these goals for common and resource-intense conditions such as congenital heart diseas ... Full text Link to item Cite

Pediatric exclusivity and other contemporary regulatory initiatives: aligning financial incentives with the needs of our patients

Journal Article Clinical Investigation · November 2014 Full text Cite

Challenges and priorities for research: a report from the National Heart, Lung, and Blood Institute (NHLBI)/National Institutes of Health (NIH) Working Group on thrombosis in pediatric cardiology and congenital heart disease.

Journal Article Circulation · September 30, 2014 Full text Link to item Cite

Cumulative radiation exposure and cancer risk estimation in children with heart disease.

Journal Article Circulation · July 8, 2014 BACKGROUND: Children with heart disease are frequently exposed to imaging examinations that use ionizing radiation. Although radiation exposure is potentially carcinogenic, there are limited data on cumulative exposure and the associated cancer risk. We ev ... Full text Link to item Cite

Longitudinal assessment of growth in hypoplastic left heart syndrome: results from the single ventricle reconstruction trial.

Journal Article J Am Heart Assoc · June 23, 2014 BACKGROUND: We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. METHODS AND RESULTS: We performed a secondary analysis using the Single Ventricle Reconstruction ... Full text Link to item Cite

Characteristics of pediatric cardiovascular clinical trials registered on ClinicalTrials.gov.

Journal Article Am Heart J · June 2014 BACKGROUND: ClinicalTrials.gov is an National Institutes of Health-sponsored registry of federally and privately funded trials. We sought to determine fundamental characteristics of registered pediatric cardiovascular trials (PCVTs). METHODS: A data set in ... Full text Link to item Cite

Variation in congenital heart surgery costs across hospitals.

Journal Article Pediatrics · March 2014 BACKGROUND: A better understanding of costs associated with common and resource-intense conditions such as congenital heart disease has become increasingly important as children's hospitals face growing pressure to both improve quality and reduce costs. We ... Full text Link to item Cite

Sildenafil exposure and hemodynamic effect after Fontan surgery.

Journal Article Pediatr Crit Care Med · January 2014 OBJECTIVE: Determine sildenafil exposure and hemodynamic effect in children after Fontan single-ventricle surgery. DESIGN: Prospective dose-escalation trial. SETTING: Single-center pediatric catheterization laboratory. PATIENTS: Nine children post Fontan s ... Full text Link to item Cite

Radiation Exposure and Estimated Lifetime Attributable Risk of Cancer From Cardiac Catheterization in Children

Conference CIRCULATION · November 26, 2013 Link to item Cite

Excess Costs Attributable to Complications and Prolonged Length of Stay Following Congenital Heart Surgery

Conference CIRCULATION · November 26, 2013 Link to item Cite

Diagnosis and Management of Infective Endocarditis

Chapter · October 28, 2013 Cite

Intervention for recoarctation in the single ventricle reconstruction trial: incidence, risk, and outcomes.

Journal Article Circulation · August 27, 2013 BACKGROUND: Recoarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt or a ri ... Full text Link to item Cite

Hospital variation in postoperative infection and outcome after congenital heart surgery.

Journal Article Ann Thorac Surg · August 2013 BACKGROUND: Several initiatives aim to reduce postoperative infection across a variety of surgical patients as a means to improve overall quality of care and reduce variation across centers. However, the association of infection rates with hospital-level o ... Full text Link to item Cite

Sildenafil exposure and hemodynamic effect after stage II single-ventricle surgery.

Journal Article Pediatr Crit Care Med · July 2013 OBJECTIVES: To determine sildenafil exposure and hemodynamic effect in children after stage II single-ventricle surgery. DESIGN: Prospective, dose escalation trial. SETTING: Single-center, pediatric catheterization laboratory. PATIENTS: Twelve children pos ... Full text Link to item Cite

Clopidogrel in infants with systemic-to-pulmonary-artery shunts.

Journal Article N Engl J Med · June 20, 2013 BACKGROUND: Infants with cyanotic congenital heart disease palliated with placement of a systemic-to-pulmonary-artery shunt are at risk for shunt thrombosis and death. We investigated whether the addition of clopidogrel to conventional therapy reduces mort ... Full text Link to item Cite

HOSPITAL COSTS FOR PEDIATRIC HEART SURGERY VARY WIDELY ACROSS INSTITUTIONS

Conference Journal of the American College of Cardiology · March 2013 Full text Cite

Approaches to the prevention and management of childhood obesity: the role of social networks and the use of social media and related electronic technologies: a scientific statement from the American Heart Association.

Journal Article Circulation · January 15, 2013 Full text Link to item Cite

Conducting Clinical Trials in Developing and Emerging Countries: Review and Case Study

Chapter · January 1, 2013 This chapter focuses on the trends, benefits, challenges and ethical considerations involved in the globalization of pediatric clinical trials and, more specifically, the conduct of these trials in developing countries. These considerations are discussed i ... Full text Cite

Changes in pediatric food and drug administration written requests and regulations: Impact on clinical trial of hypertension trials in children

Chapter · January 1, 2013 Regulatory changes in the United States and Europe have stimulated major pediatric clinical trials of > 15 different antihypertensive agents over the last decade. With increased pediatric hypertension trial experience, trial designs have been refined and w ... Full text Cite

Differential case ascertainment in clinical registry versus administrative data and impact on outcomes assessment for pediatric cardiac operations.

Journal Article Ann Thorac Surg · January 2013 BACKGROUND: Administrative datasets are often used to assess outcomes and quality of pediatric cardiac programs; however their accuracy regarding case ascertainment is unclear. We linked patient data (2004-2010) from the Society of Thoracic Surgeons Congen ... Full text Link to item Cite

Sildenafil Pharmacokinetics, Hemodynamic Efficacy and Safety after Stage II Single Ventricle Surgery

Journal Article CIRCULATION · November 20, 2012 Link to item Cite

Intervention for Re-coarctation in the Single Ventricle Reconstruction Trial: Incidence, Risk and Outcomes

Journal Article CIRCULATION · November 20, 2012 Link to item Cite

Cancer Risk Estimation from Cardiac Catheterization in Children with Heart Disease

Journal Article CIRCULATION · November 20, 2012 Link to item Cite

Status of the pediatric clinical trials enterprise: an analysis of the US ClinicalTrials.gov registry.

Journal Article Pediatrics · November 2012 BACKGROUND AND OBJECTIVES: Clinical trials are the gold standard for generating evidence-based knowledge in medicine. Recent legislation requiring trials to be registered at ClinicalTrials.gov has enabled evaluation of the clinical trial enterprise as a wh ... Full text Link to item Cite

Pediatric cardiovascular safety: challenges in drug and device development and clinical application.

Journal Article Am Heart J · October 2012 Development of pediatric medications and devices is complicated by differences in pediatric physiology and pathophysiology (both compared with adults and within the pediatric age range), small patient populations, and practical and ethical challenges to de ... Full text Link to item Cite

Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association.

Journal Article Circulation · August 28, 2012 BACKGROUND: The goal of this statement was to review the available literature on surveillance, screening, evaluation, and management strategies and put forward a scientific statement that would comprehensively review the literature and create recommendatio ... Full text Link to item Cite

Use of the complete blood cell count in early-onset neonatal sepsis.

Journal Article Pediatr Infect Dis J · August 2012 BACKGROUND: Early-onset sepsis (EOS) is an important cause of morbidity and mortality in neonates, and its diagnosis remains challenging. The complete blood cell count and differential have been previously evaluated as diagnostic tools for EOS in small, si ... Full text Link to item Cite

Use of the complete blood cell count in late-onset neonatal sepsis.

Journal Article Pediatr Infect Dis J · August 2012 BACKGROUND: Late-onset sepsis is an important cause of morbidity and mortality in infants. Diagnosis of late-onset sepsis can be challenging. The complete blood cell count and differential have been previously evaluated as diagnostic tools for late-onset s ... Full text Link to item Cite

Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: risk factors and their interaction with shunt type.

Journal Article J Thorac Cardiovasc Surg · July 2012 OBJECTIVE: The study objective was to identify factors associated with death and cardiac transplantation in infants undergoing the Norwood procedure and to determine differences in associations that might favor the modified Blalock-Taussig shunt or a right ... Full text Link to item Cite

Relative impact of surgeon and center volume on early mortality after the Norwood operation.

Journal Article Ann Thorac Surg · June 2012 BACKGROUND: Previous studies suggest center volume is associated with outcome after the Norwood operation; however, the impact of surgeon volume is less clear. We evaluated the relative impact of surgeon and center volume on mortality in a large Norwood co ... Full text Link to item Cite

The complex relationship between center volume and outcome in patients undergoing the Norwood operation.

Journal Article Ann Thorac Surg · May 2012 BACKGROUND: Norwood outcomes vary across centers, and a relationship between center volume and outcome has been previously described. It is unclear whether this volume-outcome relationship exists across all levels of patient risk or holds true for all cent ... Full text Link to item Cite

Sepsis in young infants with congenital heart disease.

Journal Article Early Hum Dev · May 2012 BACKGROUND: We sought to describe the incidence, pathogen distribution, and mortality associated with blood culture-proven sepsis in young infants with congenital heart disease (CHD) admitted to a neonatal intensive care unit (NICU). METHODS: Cohort study ... Full text Link to item Cite

Safety of cardiac devices in children

Journal Article Progress in Pediatric Cardiology · May 1, 2012 Implanted cardiac devices have improved and prolonged the lives of countless children. Some of these devices have undergone vigorous pre-market assessment and are US Food and Drug Administration (FDA) approved for use in children; however, many devices are ... Full text Cite

Introduction

Journal Article Progress in Pediatric Cardiology · May 1, 2012 Full text Cite

Trends in endocarditis hospitalizations at US children's hospitals: impact of the 2007 American Heart Association Antibiotic Prophylaxis Guidelines.

Journal Article Am Heart J · May 2012 BACKGROUND: In 2007, the American Heart Association recommended cessation of antibiotic prophylaxis for infective endocarditis (IE) before dental procedures for all but those at highest risk for adverse outcomes from IE. The impact of these guidelines is u ... Full text Link to item Cite

A rare coronary collateral in pulmonary atresia and intact septum with coronary sinusoids.

Journal Article World J Pediatr Congenit Heart Surg · April 1, 2012 A neonate with pulmonary atresia and intract ventricular septum, ventriculocoronary sinusoids, bilateral coronary ostial atresia, and a rare collateral vessel between the descending thoracic aorta and the coronary system is described. The clinical course i ... Full text Link to item Cite

Comparative analysis of antifibrinolytic medications in pediatric heart surgery.

Journal Article J Thorac Cardiovasc Surg · March 2012 OBJECTIVES: Recent studies suggest adverse events associated with aprotinin in adults may not occur in children, and there is interest in further pediatric study of aprotinin. However, there are limited contemporary data comparing aprotinin with other avai ... Full text Link to item Cite

Perioperative methylprednisolone and outcome in neonates undergoing heart surgery.

Journal Article Pediatrics · February 2012 BACKGROUND: Recent studies have called into question the benefit of perioperative corticosteroids in children undergoing heart surgery, but have been limited by the lack of placebo control, limited power, and grouping of various steroid regimens together i ... Full text Link to item Cite

Association of center volume with mortality and complications in pediatric heart surgery.

Journal Article Pediatrics · February 2012 OBJECTIVE: Previous analyses have suggested center volume is associated with outcome in children undergoing heart surgery. However, data are limited regarding potential mediating factors, including the relationship of center volume with postoperative compl ... Full text Link to item Cite

Pharmacodynamic effects of clopidogrel in pediatric cardiac patients: a comparative study of platelet aggregation response.

Journal Article Platelets · 2012 Little data on pediatric percent platelet aggregation (%PA) exist in the literature, particularly in cardiac patients and in response to clopidogrel. The objectives were to estimate the %PA range expected in pediatric patients and to measure the clopidogre ... Full text Link to item Cite

Opportunities and challenges in linking information across databases in pediatric cardiovascular medicine.

Journal Article Prog Pediatr Cardiol · January 2012 Multicenter databases are increasingly utilized in pediatric cardiovascular research. In this review, we discuss the rational for using these types of data sources, provide several examples of how large datasets have been utilized in clinical research, and ... Full text Link to item Cite

Timing of Perioperative Methylprednisolone and Outcome in Infants Undergoing Heart Surgery

Journal Article CIRCULATION · November 22, 2011 Link to item Cite

Risk Factors for Mortality in the Single Ventricle Reconstruction Trial: Beyond Shunt Type

Journal Article CIRCULATION · November 22, 2011 Link to item Cite

Complications after the Norwood operation: an analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

Journal Article Ann Thorac Surg · November 2011 BACKGROUND: Limited multicenter data exist regarding the prevalence of postoperative complications after the Norwood operation and their associated mortality risk. METHODS: We evaluated infants in The Society of Thoracic Surgeons Congenital Heart Surgery D ... Full text Link to item Cite

Pediatric cardiovascular drug dosing in critically ill children and extracorporeal membrane oxygenation.

Journal Article J Cardiovasc Pharmacol · August 2011 Cardiovascular disease in children is common and results in significant morbidity and mortality. The sickest children with cardiovascular disease may require support with extracorporeal membrane oxygenation (ECMO), which provides life-saving assistance for ... Full text Link to item Cite

Pediatric cardiovascular drug trials, lessons learned.

Journal Article J Cardiovasc Pharmacol · July 2011 Few drugs have been labeled for pediatric cardiovascular indications, and many children with cardiac disease are prescribed drugs off-label. Recent initiatives have narrowed this gap, and as a result, there are an increasing number of cardiology trials in ... Full text Link to item Cite

Cardiovascular abnormalities in late-onset Pompe disease and response to enzyme replacement therapy.

Journal Article Genet Med · July 2011 PURPOSE: We evaluated the prevalence of cardiovascular abnormalities and the efficacy and safety of enzyme replacement therapy in patients with late-onset Pompe disease. METHODS: Ninety patients were randomized 2:1 to enzyme replacement therapy or placebo ... Full text Link to item Cite

Care models and associated outcomes in congenital heart surgery.

Journal Article Pediatrics · June 2011 OBJECTIVE: Recently, there has been a shift toward care of children undergoing heart surgery in dedicated pediatric cardiac intensive care units (CICU). The impact of this trend on patient outcomes is unclear. We evaluated postoperative outcomes associated ... Full text Link to item Cite

Renin-angiotensin-aldosterone genotype influences ventricular remodeling in infants with single ventricle.

Journal Article Circulation · May 31, 2011 BACKGROUND: We investigated the effect of polymorphisms in the renin-angiotensin-aldosterone system (RAAS) genes on ventricular remodeling, growth, renal function, and response to enalapril in infants with single ventricle. METHODS AND RESULTS: Single vent ... Full text Link to item Cite

Center variation in patient age and weight at Fontan operation and impact on postoperative outcomes.

Journal Article Ann Thorac Surg · May 2011 BACKGROUND: The impact of age and weight on outcomes after the Fontan operation is unclear. Previous analyses have suggested that lower weight-for-age z-score is an important predictor of poor outcome in patients undergoing bidirectional Glenn. We evaluate ... Full text Link to item Cite

Center variation in hospital costs for patients undergoing congenital heart surgery.

Journal Article Circ Cardiovasc Qual Outcomes · May 2011 BACKGROUND: Congenital heart disease consumes significant health care resources; however, there are limited data regarding factors affecting resource utilization. The purpose of this study was to evaluate variation between centers in total hospital costs f ... Full text Link to item Cite

Evaluation of Kawasaki disease risk-scoring systems for intravenous immunoglobulin resistance.

Journal Article J Pediatr · May 2011 OBJECTIVES: To assess the performance of 3 risk scores from Japan that were developed to predict, in children with Kawasaki disease, resistance to intravenous immunoglobulin (IVIG) treatment. STUDY DESIGN: We used data from a randomized trial of pulsed ste ... Full text Link to item Cite

Celiac artery flow pattern in infants with single right ventricle following the Norwood procedure with a modified Blalock-Taussig or right ventricle to pulmonary artery shunt.

Journal Article Pediatr Cardiol · April 2011 A potential advantage of the right ventricle to pulmonary artery versus modified Blalock-Taussig shunt in patients undergoing the Norwood procedure is limitation of diastolic runoff from the systemic to pulmonary circulation. We evaluated mesenteric flow p ... Full text Link to item Cite

PATIENT RISK STATUS, CENTER VOLUME AND OUTCOME FOLLOWING THE NORWOOD OPERATION

Conference Journal of the American College of Cardiology · April 2011 Full text Cite

PREVALENCE AND MORTALITY RISK ASSOCIATED WITH POST-OPERATIVE COMPLICATIONS FOLLOWING THE NORWOOD OPERATION: ANALYSIS OF A NATIONAL CLINICAL DATABASE

Conference Journal of the American College of Cardiology · April 2011 Full text Cite

Lessons learned from a pediatric clinical trial: the Pediatric Heart Network angiotensin-converting enzyme inhibition in mitral regurgitation study.

Journal Article Am Heart J · February 2011 BACKGROUND: Mitral regurgitation is the most common indication for reoperation in children following repair of atrioventricular septal defect (AVSD). We hypothesized that angiotensin-converting enzyme inhibitor therapy would decrease the severity of mitral ... Full text Link to item Cite

Noncoronary cardiac abnormalities are associated with coronary artery dilation and with laboratory inflammatory markers in acute Kawasaki disease.

Journal Article J Am Coll Cardiol · January 4, 2011 OBJECTIVES: We explored the association of noncoronary cardiac abnormalities with coronary artery dilation and with laboratory inflammatory markers early after Kawasaki disease (KD) diagnosis. BACKGROUND: Left ventricular (LV) dysfunction, mitral regurgita ... Full text Link to item Cite

Pediatric Antihypertensive Clinical Trials

Chapter · 2011 Full text Cite

In reply

Journal Article Pediatrics · January 1, 2011 Full text Cite

Clinical research careers: reports from a NHLBI pediatric heart network clinical research skills development conference.

Journal Article Am Heart J · January 2011 BACKGROUND: Wyman W. Lai, MD, MPH, and Victoria L. Vetter, MD, MPH. The Pediatric Heart Network (PHN), funded under the U.S. National Institutes of Health-National Heart, Lung, and Blood Institute (NIH-NHLBI), includes two Clinical Research Skills Developm ... Full text Link to item Cite

Predictors of coronary artery visualization in Kawasaki disease.

Journal Article J Am Soc Echocardiogr · January 2011 BACKGROUND: Echocardiography is the imaging modality of choice for the evaluation of coronary artery (CA) abnormalities in Kawasaki disease. Small series have established high specificity and sensitivity for detecting abnormalities, yet visualization rates ... Full text Link to item Cite

Oropharyngeal dysphagia in infants and children with infantile Pompe disease.

Journal Article Dysphagia · December 2010 Pompe disease is a rare genetic progressive neuromuscular disorder. The most severe form, infantile Pompe disease, has historically resulted in early mortality, most commonly due to cardiorespiratory failure. Treatment with enzyme replacement therapy (ERT) ... Full text Open Access Link to item Cite

Use of cardiac magnetic resonance imaging to evaluate cardiac structure, function and fibrosis in children with infantile Pompe disease on enzyme replacement therapy.

Journal Article Mol Genet Metab · December 2010 BACKGROUND: Pompe disease (acid α-glucosidase deficiency) is one of several lysosomal storage diseases amenable to treatment with enzyme replacement therapy (ERT). While echocardiography (echo) has been the standard method to evaluate the cardiac response ... Full text Link to item Cite

Linking clinical registry data with administrative data using indirect identifiers: implementation and validation in the congenital heart surgery population.

Journal Article Am Heart J · December 2010 BACKGROUND: The use of clinical registries and administrative data sets in pediatric cardiovascular research has become increasingly common. However, this approach is limited by relatively few existing datasets, each of which contain limited data, and do n ... Full text Link to item Cite

Anthropometric measures after Fontan procedure: implications for suboptimal functional outcome.

Journal Article Am Heart J · December 2010 BACKGROUND: Abnormal height and adiposity are observed after the Fontan operation. These abnormalities may be associated with worse functional outcome. METHODS: We analyzed data from the National Heart, Lung, and Blood Institute Pediatric Heart Network cro ... Full text Link to item Cite

Corticosteroids and outcome in children undergoing congenital heart surgery: analysis of the Pediatric Health Information Systems database.

Journal Article Circulation · November 23, 2010 BACKGROUND: Children undergoing congenital heart surgery often receive corticosteroids with the aim of reducing the inflammatory response after cardiopulmonary bypass; however, the value of this approach is unclear. METHODS AND RESULTS: The Pediatric Healt ... Full text Link to item Cite

Patient, Operative, and Center Factors Impacting Post-operative Outcomes in Patients Undergoing the Fontan Operation: Analysis of a National Clinical Database

Journal Article CIRCULATION · November 23, 2010 Link to item Cite

The Impact of Intensive Care Unit Structure on Post-operative Outcomes Following Congenital Heart Surgery: Analysis of a Multi-institutional Database

Journal Article CIRCULATION · November 23, 2010 Link to item Cite

A Randomized Trial of Clopidogrel to Reduce Mortality and Shunt-Related Morbidity in Infants Palliated with a Systemic to Pulmonary Artery Shunt

Journal Article CIRCULATION · November 23, 2010 Link to item Cite

Letter by Hill et al. regarding article, "Mortality in first 5 years in infants with functional single ventricle born in Texas, 1996 to 2003".

Journal Article Circulation · October 19, 2010 Full text Link to item Cite

Globalization of pediatric research: analysis of clinical trials completed for pediatric exclusivity.

Journal Article Pediatrics · September 2010 OBJECTIVE: We evaluated the setting of published studies conducted under the US Pediatric Exclusivity Provision, which provides economic incentives to pharmaceutical companies to conduct drug studies with children. METHODS: Published studies containing the ... Full text Link to item Cite

Fluticasone propionate/salmeterol combination in children with asthma: Key cardiac and overall safety results

Journal Article Clinical Research and Regulatory Affairs · September 1, 2010 This study studied the safety of fluticasone propionate/salmeterol combination (FSC) 100/50 HFA (2 inhalations of 50/25 mcg) twice daily, compared with fluticasone propionate (FP) 100 HFA (two inhalations of 50 mcg) twice daily, over a 12-week treatment pe ... Full text Cite

The efficacy and safety of the novel aldosterone antagonist eplerenone in children with hypertension: a randomized, double-blind, dose-response study.

Journal Article J Pediatr · August 2010 Featured Publication OBJECTIVES: To determine the efficacy and safety of eplerenone therapy in children with hypertension. STUDY DESIGN: A total of 304 children age 4-16 years with systolic blood pressure (SBP) >or=95th percentile were randomized to low-dose (25 mg daily), mid ... Full text Link to item Cite

Congenital heart surgery outcomes in Down syndrome: analysis of a national clinical database.

Journal Article Pediatrics · August 2010 OBJECTIVE: We describe patient characteristics and postoperative morbidity and mortality rates for patients with Down syndrome undergoing congenital heart disease surgery. METHODS: This retrospective cohort study used the Society of Thoracic Surgeons Conge ... Full text Link to item Cite

Echocardiographic manifestations of Glycogen Storage Disease III: increase in wall thickness and left ventricular mass over time.

Journal Article Genet Med · July 2010 PURPOSE: Glycogen Storage Disease Type III, glycogen debranching enzyme deficiency, causes accumulation of glycogen in liver, skeletal, and cardiac muscle. Some patients develop increased left ventricular thickness by echocardiography, but the rate of incr ... Full text Link to item Cite

Safety of aprotinin in congenital heart operations: results from a large multicenter database.

Journal Article Ann Thorac Surg · July 2010 BACKGROUND: Aprotinin was used frequently in children undergoing congenital heart operations with the aim of reducing bleeding, until it was taken off the market after adult studies reported increased renal failure and death. We evaluated the safety of apr ... Full text Link to item Cite

Cough in pediatric patients receiving angiotensin-converting enzyme inhibitor therapy or angiotensin receptor blocker therapy in randomized controlled trials.

Journal Article Clin Pharmacol Ther · June 2010 Featured Publication The incidence of cough in children receiving antihypertension therapy with angiotensin-converting enzyme inhibitors (ACEis) or angiotensin receptor blockers (ARBs) is unknown. We analyzed patient-level data from eight randomized trials for the treatment of ... Full text Link to item Cite

Paediatric anti-hypertensive clinical trials and various factors influencing trial success or failure

Journal Article · May 21, 2010 Full text Cite

Center variation and outcomes associated with delayed sternal closure after stage 1 palliation for hypoplastic left heart syndrome.

Journal Article J Thorac Cardiovasc Surg · May 2010 OBJECTIVE: There is debate whether primary or delayed sternal closure is the best strategy after stage 1 palliation for hypoplastic left heart syndrome. We describe center variation in delayed sternal closure after stage 1 palliation and associated outcome ... Full text Link to item Cite

Antiplatelet therapy in pediatric cardiovascular patients.

Journal Article Pediatr Cardiol · May 2010 Featured Publication Platelets play a critical role in normal physiologic processes such as hemostasis, wound healing, inflammation, and innate immunity. However, they also play a role in the pathologic process of thrombosis. Although antiplatelet therapy is most commonly used ... Full text Link to item Cite

Variation in models of care delivery for children undergoing congenital heart surgery in the United States.

Journal Article World J Pediatr Congenit Heart Surg · April 2010 Limited data are available regarding contemporary models of care delivery for patients undergoing congenital heart surgery. The purpose of this survey was to evaluate current US practice patterns in this patient population. Cross-sectional evaluation of US ... Full text Link to item Cite

Major infection after pediatric cardiac surgery: a risk estimation model.

Journal Article Ann Thorac Surg · March 2010 Featured Publication BACKGROUND: In pediatric cardiac surgery, infection is a leading cause of morbidity and mortality. We created a model to predict risk of major infection in this population. METHODS: Using the Society of Thoracic Surgeons Congenital Heart Surgery Database, ... Full text Link to item Cite

Partial and transitional atrioventricular septal defect outcomes.

Journal Article Ann Thorac Surg · February 2010 BACKGROUND: Surgical and perioperative improvements permit earlier repair of partial and transitional atrioventricular septal defects (AVSD). We sought to describe contemporary outcomes in a multicenter cohort. METHODS: We studied 87 patients undergoing pr ... Full text Link to item Cite

Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure.

Journal Article Circulation · January 5, 2010 BACKGROUND: Patients after the Fontan procedure are at risk for suboptimal functional health status, and associations with laboratory measures are important for planning interventions and outcome measures for clinical trials. METHODS AND RESULTS: Parents c ... Full text Link to item Cite

Safety of aprotinin in congenital heart operations: Results from a large multicenter database

Other Pediatric Cardiology · January 1, 2010 Full text Cite

Birth weight and prematurity in infants with single ventricle physiology: pediatric heart network infant single ventricle trial screened population.

Journal Article Congenit Heart Dis · 2010 OBJECTIVES: Although congenital heart disease is associated with low birth weight and prematurity, there is little information about these birth outcomes in infants with single ventricle physiology. We describe the birth outcomes (i.e., gestational age and ... Full text Link to item Cite

Cross-reactive immunologic material status affects treatment outcomes in Pompe disease infants.

Journal Article Mol Genet Metab · January 2010 Deficiency of acid alpha glucosidase (GAA) causes Pompe disease, which is usually fatal if onset occurs in infancy. Patients synthesize a non-functional form of GAA or are unable to form native enzyme. Enzyme replacement therapy with recombinant human GAA ... Full text Link to item Cite

Platelet activity associated with concomitant use of clopidogrel and proton pump inhibitors in children with cardiovascular disease.

Journal Article Congenit Heart Dis · 2010 CONTEXT: In adults with acute coronary syndrome, decreased platelet inhibition associated with concomitant use of clopidogrel and proton pump inhibitors (PPI) has been reported. OBJECTIVE: To evaluate platelet activity associated with PPI + clopidogrel vs. ... Full text Link to item Cite

Safety and transparency of pediatric drug trials.

Journal Article Arch Pediatr Adolesc Med · December 2009 Featured Publication OBJECTIVES: To quantify the frequency and type of new safety information arising from studies performed under the auspices of the Pediatric Exclusivity Program, to describe the dissemination of these findings in the peer-reviewed literature and compare thi ... Full text Link to item Cite

Aprotinin is Not Associated With Renal Failure or Increased Mortality in Children Undergoing Congenital Heart Surgery

Conference CIRCULATION · November 3, 2009 Link to item Cite

Corticosteroids Are Associated With Increased Morbidity in Children Undergoing Congenital Heart Surgery

Conference CIRCULATION · November 3, 2009 Link to item Cite

Group B streptococcal meningitis: cerebrospinal fluid parameters in the era of intrapartum antibiotic prophylaxis.

Journal Article Early Hum Dev · October 2009 OBJECTIVE: Describe cerebrospinal fluid parameters in infants with culture-proven Group B streptococcal meningitis in the era of intrapartum antibiotic prophylaxis. STUDY DESIGN: Cohort study of the first lumbar puncture from 13,495 infants cared for at 15 ... Full text Link to item Cite

INFLUENCE OF CROSS-REACTING IMMUNOLOGIC MATERIAL STATUS ON TREATMENT OUTCOMES IN INFANTILE POMPE PATIENTS TREATED WITH RECOMBINANT HUMAN ACID ALPHA-GLUCOSIDASE

Journal Article MOLECULAR GENETICS AND METABOLISM · September 1, 2009 Link to item Cite

Functional state following the Fontan procedure.

Journal Article Cardiol Young · August 2009 BACKGROUND: Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function. METHODS AND RESULTS: We analyzed data ... Full text Link to item Cite

Page Albert Willis Anderson (1942–2008)

Journal Article Cardiology in the Young · June 2009 Full text Cite

Associated symptoms in the ten days before diagnosis of Kawasaki disease.

Journal Article J Pediatr · April 2009 OBJECTIVE: To describe common associated symptoms within the 10 days before diagnosis in subjects enrolled in the Pediatric Heart Network's trial of steroid therapy in Kawasaki disease (KD). STUDY DESIGN: Patients with acute KD were enrolled between days 4 ... Full text Link to item Cite

Rationale and design of a trial of angiotensin-converting enzyme inhibition in infants with single ventricle.

Journal Article Am Heart J · January 2009 Featured Publication BACKGROUND: Angiotensin converting enzyme (ACE) inhibitors are known to improve clinical outcome and ventricular function in adults with heart failure. Infants with single-ventricle physiology show abnormalities in ventricular function as well as poor grow ... Full text Link to item Cite

Global longitudinal cardiac strain and strain rate for assessment of fetal cardiac function: novel experience with velocity vector imaging.

Journal Article Echocardiography · January 2009 BACKGROUND: Cardiac strain and strain rate are new methods to quantitate fetal cardiac function. Doppler-based techniques are regional measurements limited by angle of insonation. Newer feature-tracking algorithms permit angle independent measurements from ... Full text Link to item Cite

Cardiac remodeling after enzyme replacement therapy with acid alpha-glucosidase for infants with Pompe disease.

Journal Article Pediatr Cardiol · November 2008 Featured Publication BACKGROUND: Infantile Pompe disease (glycogen storage disease type 2) is a fatal disorder caused by deficiency of acid alpha-glucosidase. This deficiency results in glycogen accumulation in the lysosomes of many tissues including cardiac muscle. The diseas ... Full text Link to item Cite

Erratum: Racial differences in blood pressure response to angiotensin-converting enzyme inhibitors in children: A meta-analysis (Clinical Pharmacology Therapeutics (2008) 84, (315-319))

Journal Article Clinical Pharmacology and Therapeutics · November 1, 2008 Full text Cite

Prevention of future cardiovascular disease in high-risk pediatric patients: a role for lipid lowering therapy?

Journal Article Circ Cardiovasc Qual Outcomes · November 2008 Full text Link to item Cite

Predictors of Coronary Artery Visualization in Kawasaki Disease in the Pediatric Heart Network (PHN) Multi-Center Study

Journal Article CIRCULATION · October 28, 2008 Link to item Cite

Arrhythmias in patients receiving enzyme replacement therapy for infantile Pompe disease.

Journal Article Genet Med · October 2008 Featured Publication PURPOSE: Enzyme replacement therapy in infants with Pompe disease prolongs survival, decreases cardiomegaly, and improves muscle function. Because ectopy has been previously described in these patients, we sought to determine the prevalence and types of ar ... Full text Link to item Cite

The economic returns of pediatric clinical trials of antihypertensive drugs.

Journal Article Am Heart J · October 2008 Featured Publication BACKGROUND: Congress has authorized the United States Food and Drug Administration (FDA) to provide industry sponsors with a 6-month extension of drug marketing rights under the Pediatric Exclusivity Provision if FDA-requested pediatric drug trials are con ... Full text Link to item Cite

Safety monitoring of drugs receiving pediatric marketing exclusivity.

Journal Article Pediatrics · September 2008 Featured Publication OBJECTIVES: The Food and Drug Administration Modernization Act provided for an additional 6-month period of marketing exclusivity to companies that perform pediatric drug trials in response to a Food and Drug Administration-issued written request. Because ... Full text Link to item Cite

Racial differences in blood pressure response to angiotensin-converting enzyme inhibitors in children: a meta-analysis.

Journal Article Clin Pharmacol Ther · September 2008 Featured Publication Angiotensin-converting enzyme (ACE) inhibitors are frequently used to treat hypertension in children.(1) ACE inhibitors alter the balance between the vasoconstrictive, salt-retentive, and cardiac hypertrophic properties of angiotensin II and the vasodilato ... Full text Link to item Cite

Response to letter regarding article, "dosing of clopidogrel for platelet inhibition in infants and young children: primary results of the platelet inhibition in children on clopidogrel (picolo) trial"

Journal Article Circulation · August 12, 2008 Full text Cite

A cross-sectional study of exercise performance during the first 2 decades of life after the Fontan operation.

Journal Article J Am Coll Cardiol · July 8, 2008 OBJECTIVES: The aim of this study was to describe exercise performance during the first 2 decades of life in Fontan survivors by a cross-sectional study and to identify factors that influence exercise performance. BACKGROUND: Exercise performance after the ... Full text Link to item Cite

Safety of placebo controls in pediatric hypertension trials.

Journal Article Hypertension · April 2008 Featured Publication Many clinical trials, including those in pediatric populations, use a placebo arm for medical conditions for which there are readily available therapeutic interventions. Several short-term efficacy trials of antihypertensive medications performed in respon ... Full text Link to item Cite

Pediatric antihypertensive trial failures: analysis of end points and dose range.

Journal Article Hypertension · April 2008 Featured Publication Historically, drugs prescribed for children have not been studied in pediatric populations. Since 1997, however, a 6-month extension of marketing rights is granted if manufacturers conduct Food and Drug Administration-defined pediatric trials. In nearly ha ... Full text Link to item Cite

Cardiac surgery in infants with low birth weight is associated with increased mortality: analysis of the Society of Thoracic Surgeons Congenital Heart Database.

Journal Article J Thorac Cardiovasc Surg · March 2008 OBJECTIVE: The evaluation of operative mortality risk for cardiac surgery in infants with low weight is limited. To determine whether low weight is a risk factor for increased mortality, we reviewed the experience within the Society of Thoracic Surgeons Co ... Full text Link to item Cite

Dosing of clopidogrel for platelet inhibition in infants and young children: primary results of the Platelet Inhibition in Children On cLOpidogrel (PICOLO) trial.

Journal Article Circulation · January 29, 2008 Featured Publication BACKGROUND: Infants and young children with certain types of heart disease are at increased risk for thromboses. Clopidogrel 75 mg/d is used in adults to prevent thrombotic events. The dose to achieve similar platelet inhibition in children is unknown. The ... Full text Link to item Cite

Delayed diagnosis of Kawasaki disease: what are the risk factors?

Journal Article Pediatrics · December 2007 Featured Publication OBJECTIVE: Because late diagnosis of Kawasaki disease increases the risk for coronary artery abnormalities, we explored the prevalence of and possible risk factors for delayed diagnosis by using the database of the Pediatric Heart Network trial of corticos ... Full text Link to item Cite

Sibling phenotype concordance in classical infantile Pompe disease.

Journal Article Am J Med Genet A · November 1, 2007 Pompe disease (acid-alpha-glucosidase deficiency) encompasses a clinical spectrum, ranging from severe infantile-onset disease with clinical symptoms appearing before 1 year of age with rapid progression to an early death, to late-onset disease with a much ... Full text Link to item Cite

Racial differences in blood pressure response to angiotensin converting enzyme inhibitors in children: An analysis of 6 pediatric hypertension trials

Conference CIRCULATION · October 16, 2007 Link to item Cite

The economic return of performing pediatric anti-hypertensive drug clinical trials

Conference CIRCULATION · October 16, 2007 Link to item Cite

Rationale and design of a randomized clinical trial of beta-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome.

Journal Article Am Heart J · October 2007 BACKGROUND: Cardiovascular disease, including aortic root dilation, dissection, and rupture, is the leading cause of mortality in patients with Marfan syndrome (MFS). The maximal aortic root diameter at the sinuses of Valsalva is considered the best predic ... Full text Link to item Cite

Enzyme-deficiency metabolic cardiomyopathies and the role of enzyme replacement therapy

Journal Article Progress in Pediatric Cardiology · September 1, 2007 Cardiovascular abnormalities are common in some lysosomal storage disorders (LSD) as a result of intracellular accumulation of the storage material. The heart is a major organ of involvement, with a hypertrophic type cardiomyopathy as a cardinal clinical m ... Full text Cite

Cardiac arrhythmias following anesthesia induction in infantile-onset Pompe disease: a case series.

Journal Article Paediatr Anaesth · August 2007 BACKGROUND: Patients with infantile-onset Pompe disease suffer from marked hypertrophic cardiomyopathy and an increased risk of arrhythmia. A noncompliant left ventricle predisposes these infants to diastolic heart failure with elevated left ventricular en ... Full text Link to item Cite

Clinical outcomes of palliative surgery including a systemic-to-pulmonary artery shunt in infants with cyanotic congenital heart disease: does aspirin make a difference?

Journal Article Circulation · July 17, 2007 Featured Publication BACKGROUND: Aspirin (ASA) often is used to prevent thrombosis in infants with congenital heart disease after placement of a systemic-to-pulmonary artery shunt, but its effect on outcomes is unknown. METHODS AND RESULTS: The present multicenter study prospe ... Full text Link to item Cite

Coronary artery involvement in children with Kawasaki disease: risk factors from analysis of serial normalized measurements.

Journal Article Circulation · July 10, 2007 Featured Publication BACKGROUND: Most studies of coronary artery involvement and associated risk factors in Kawasaki disease have used the Japanese Ministry of Health dichotomous criteria. Analysis of serial normalized artery measurements may reveal a broader continuous spectr ... Full text Link to item Cite

Preliminary study of the safety and efficacy of donepezil hydrochloride in children with Down syndrome: a clinical report series.

Journal Article Am J Med Genet A · July 1, 2007 There is growing evidence to support the use of early central cholinergic enhancement to improve cognitive functioning in individuals with Down syndrome (DS). This report summarizes preliminary safety and cognitive efficacy data for seven children (8-13 ye ... Full text Link to item Cite

Age at bidirectional glenn procedure and mortality: A multi-center, international registry

Conference JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY · March 6, 2007 Link to item Cite

Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease.

Journal Article N Engl J Med · February 15, 2007 Featured Publication BACKGROUND: Treatment of acute Kawasaki disease with intravenous immune globulin and aspirin reduces the risk of coronary-artery abnormalities and systemic inflammation, but despite intravenous immune globulin therapy, coronary-artery abnormalities develop ... Full text Link to item Cite

Economic return of clinical trials performed under the pediatric exclusivity program.

Journal Article JAMA · February 7, 2007 Featured Publication CONTEXT: In 1997, Congress authorized the US Food and Drug Administration (FDA) to grant 6-month extensions of marketing rights through the Pediatric Exclusivity Program if industry sponsors complete FDA-requested pediatric trials. The program has been pra ... Full text Open Access Link to item Cite

Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease.

Journal Article Neurology · January 9, 2007 BACKGROUND: Pompe disease is a progressive metabolic neuromuscular disorder resulting from deficiency of lysosomal acid alpha-glucosidase (GAA). Infantile-onset Pompe disease is characterized by cardiomyopathy, respiratory and skeletal muscle weakness, and ... Full text Link to item Cite

Major complications associated with transcatheter atrial septal occluder implantation: a review of the medical literature and the manufacturer and user facility device experience (MAUDE) database.

Journal Article Congenit Heart Dis · 2007 OBJECTIVE: To summarize major complications and outcome for patients receiving percutaneous closure of atrial septal communications. DESIGN: The Medline database and the United States Food and Drug Administration manufacturer and user facility device exper ... Full text Link to item Cite

Safety and efficacy of rivastigmine in adolescents with Down syndrome: a preliminary 20-week, open-label study.

Journal Article J Child Adolesc Psychopharmacol · December 2006 Individuals with Down syndrome (DS) exhibit a cholinergic deficiency similar to that found in Alzheimer's disease. Cholinesterase inhibitors, used to treat Alzheimer's disease, may improve cognitive function in individuals with DS. This is the first invest ... Full text Link to item Cite

Optimal dose of clopidogrel for platelet inhibition in children: Primary results of the PICOLO trial

Journal Article CIRCULATION · October 31, 2006 Link to item Cite

Peer-reviewed publication of clinical trials completed for pediatric exclusivity.

Journal Article JAMA · September 13, 2006 Featured Publication CONTEXT: Much of pediatric drug use is off-label because appropriate pediatric studies have not been conducted and the drugs have not been labeled by the US Food and Drug Administration (FDA) for use in children. In 1997, Congress authorized the FDA to gra ... Full text Link to item Cite

The use of TP10, soluble complement receptor 1, in cardiopulmonary bypass.

Journal Article Expert Rev Cardiovasc Ther · September 2006 Featured Publication Cardiopulmonary bypass (CPB) for cardiac surgery or lung transplantation initiates a systemic inflammatory response characterized by increased vascular permeability, generalized edema, abnormal lung function and oxygenation and impaired ventricular functio ... Full text Link to item Cite

Clinical trials in children with Down syndrome: issues from a cognitive research perspective.

Journal Article Am J Med Genet C Semin Med Genet · August 15, 2006 Clinical and translational research play a key role in the transition of basic research discoveries to effective therapies. In Down syndrome (DS), these research approaches are not well utilized or developed to test new therapies to improve cognitive and/o ... Full text Link to item Cite

Racial differences are seen in blood pressure response to fosinopril in hypertensive children.

Journal Article Am Heart J · August 2006 Featured Publication BACKGROUND: Few antihypertensive therapies have been systematically studied in children and dosages for many agents are either extrapolated from adult studies or obtained from small homogenous pediatric populations. It is well established that adult patien ... Full text Link to item Cite

Electrocardiographic response to enzyme replacement therapy for Pompe disease.

Journal Article Genet Med · May 2006 Featured Publication PURPOSE: Electrocardiogram (ECG) abnormalities are universal in infantile Pompe disease or glycogen storage disease type II, a fatal genetic muscle disorder caused by deficiency of acid alpha-glucosidase (GAA). Hallmarks of this disease include a shortened ... Full text Link to item Cite

Ambulatory electrocardiogram analysis in infants treated with recombinant human acid alpha-glucosidase enzyme replacement therapy for Pompe disease.

Journal Article Genet Med · May 2006 Featured Publication PURPOSE: Infantile Pompe disease is caused by deficiency of lysosomal acid alpha-glucosidase. Trials with recombinant human acid alpha-glucosidase enzyme replacement therapy (ERT) show a decrease in left ventricular mass and improved function. We evaluated ... Full text Link to item Cite

Neonatal meningitis: what is the correlation among cerebrospinal fluid cultures, blood cultures, and cerebrospinal fluid parameters?

Journal Article Pediatrics · April 2006 BACKGROUND: Meningitis is a substantial cause of morbidity and mortality in neonates. Clinicians frequently use the presence of positive blood cultures to determine whether neonates should undergo lumbar puncture. Abnormal cerebrospinal fluid (CSF) paramet ... Full text Link to item Cite

Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure.

Journal Article Circulation · February 28, 2006 Featured Publication BACKGROUND: After the Fontan procedure, patients are at risk for suboptimal health status related to their complex healthcare experience, physiological limitations, medical complications, and guarded long-term prognosis. METHODS AND RESULTS: In the Pediatr ... Full text Link to item Cite

A comparison of neonatal Gram-negative rod and Gram-positive cocci meningitis.

Journal Article J Perinatol · February 2006 OBJECTIVE: Neonatal meningitis is an illness with potentially devastating consequences. Early identification of potential risk factors for Gram-negative rod (GNR) infections versus Gram-positive cocci (GPC) infection prior to obtaining final culture result ... Full text Link to item Cite

Randomized trial of pulse steroid therapy in Kawasaki disease

Conference CIRCULATION · October 25, 2005 Link to item Cite

Influence of aspirin use on mortality in infants with cyanotic congenital heart disease palliated with a systemic-to-pulmonary artery shunt: A multi-center, international registry

Conference CIRCULATION · October 25, 2005 Link to item Cite

Clinical outcome of palliative surgery including a systemic-to-pulmonary artery shunt in infants with cyanotic congenital heart disease: Results of a multi-center, international registry

Conference CIRCULATION · October 25, 2005 Link to item Cite

A meta-analysis of medical versus surgical therapy for Candida endocarditis.

Journal Article J Infect · October 2005 OBJECTIVES: The optimal management of Candida infective endocarditis (IE) is unknown. METHODS: We reviewed all 879 cases of Candida IE reported from 1966-2002 in the peer-reviewed literature to better understand the role of medical and surgical therapies. ... Full text Link to item Cite

Frequency of infective endocarditis among infants and children with Staphylococcus aureus bacteremia.

Journal Article Pediatrics · January 2005 Featured Publication PURPOSE: The prevalence of infective endocarditis (IE) among children with Staphylococcus aureus bacteremia (SAB) is unknown. The objective of this study was to determine prospectively the prevalence of IE among pediatric patients with SAB in a large terti ... Full text Link to item Cite

Electrocardiographic response to enzyme replacement therapy for infantile Pompe disease

Conference CIRCULATION · October 26, 2004 Link to item Cite

Cardiac remodeling and clinical effects of recombinant human acid alpha-glucosidase enzyme replacement therapy for infantile glycogen storage disease type II

Conference CIRCULATION · October 26, 2004 Link to item Cite

The relationship of medical and patient characteristics to health-related quality of life in adolescents after the Fontan procedure

Conference CIRCULATION · October 26, 2004 Link to item Cite

Is the extrapolated adult dose of fosinopril safe and effective in treating hypertensive children?

Journal Article Hypertension · September 2004 Featured Publication We evaluated the efficacy, safety, and dose-response relationship of fosinopril in children aged 6 to 16 years with hypertension or high-normal blood pressure with an associated medical condition requiring treatment. The study was a prospective, double-bli ... Full text Link to item Cite

1114-205 Racial differences in blood pressure response in children with hypertension treated with fosinopril

Conference Journal of the American College of Cardiology · March 2004 Full text Cite

1085-167 Dose-response study of the efficacy and safety of fosinopril in children with hypertension: A randomized, double-blind, placebo-controlled, multicenter clinical trial

Conference Journal of the American College of Cardiology · March 2004 Full text Cite

Pharmacokinetics and safety of TP10, soluble complement receptor 1, in infants undergoing cardiopulmonary bypass.

Journal Article Am Heart J · January 2004 Featured Publication BACKGROUND: Increase in vascular permeability and multiorgan dysfunction after cardiopulmonary bypass (CPB) are barriers to successful cardiac surgery in infants. Complement inhibition with TP10, a C3/C5 convertase inhibitor (AVANT Immunotherapeutics, Need ... Full text Link to item Cite

Measures of cardiac repolarization and body position in infants.

Journal Article Clin Pediatr (Phila) · 2003 Sudden Infant Death Syndrome (SIDS) is the most common cause of death in children between 1 and 6 months of age. Recent data suggest that a prolonged QTc interval on the 12-lead electrocardiogram (ECG) is associated with SIDS. Prone body position during sl ... Full text Link to item Cite

Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries: a meta-analysis.

Journal Article Circulation · November 12, 2002 BACKGROUND: Prior studies of coronary pattern and outcome after arterial switch operation (ASO) for transposition of the great arteries (TGA) have been hindered by limited statistical power. This meta-analysis assesses the effect of coronary anatomy on pos ... Full text Link to item Cite

Coronary artery pattern and mortality following arterial switch operation for transposition of the great arteries: A meta-analysis

Conference CIRCULATION · November 5, 2002 Link to item Cite

Eight-channel multidetector computed tomography: unique potential for pediatric chest computed tomography angiography.

Journal Article J Thorac Imaging · October 2002 This case report of a possible aortic pseudoaneurysm after coarctectomy in a 12-month-old boy illustrates the unique considerations when performing pediatric cardiovascular CT angiography in young children. With newer (8-channel) multidetector technology, ... Full text Link to item Cite

Oral antihypertensive trial design and analysis under the pediatric exclusivity provision.

Journal Article Am Heart J · October 2002 Featured Publication BACKGROUND: The Best Pharmaceuticals for Children Act provides a 6-year extension to the economic incentives offered to drug manufacturers under the Pediatric Exclusivity Provision, for providing requested information on drugs used in children, which inclu ... Full text Link to item Cite

Racial and geographic disparities in timing of bidirectional Glenn and Fontan stages of single-ventricle palliation.

Journal Article J Natl Med Assoc · October 2002 OBJECTIVE: In adults, race-based disparity in access to cardiovascular care has been documented. Racial differences in cardiac care for children have not been evaluated previously. METHODOLOGY: We analyzed timing of single-ventricle palliation as a functio ... Link to item Cite

Racial and geographic disparities in timing of bidirectional Glenn and Fontan stages of single-ventricle palliation

Journal Article JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION · October 1, 2002 Link to item Cite

Real-time transmission of pediatric echocardiograms using a single ISDN line.

Journal Article Comput Biol Med · September 2002 Featured Publication We tested the adequacy of a videoconferencing system using a single integrated systems digital network (ISDN) line (128 kilobits per second) for the remote diagnosis of children with suspected congenital heart disease (CHD). Real-time echocardiogram interp ... Full text Link to item Cite

Cost implications of closure of atrial septal defect.

Journal Article Catheter Cardiovasc Interv · January 2002 We sought to evaluate the relative cost of surgical and device closure of atrial septal defect. Device closure for atrial septal defects is becoming an alternative to surgical closure. We examined the hospital-generated cost data in 13 patients who underwe ... Full text Link to item Cite

Bacterial endocarditis in infants and children.

Journal Article Pediatr Infect Dis J · August 2001 Featured Publication Full text Link to item Cite

Racial and geographic disparities in timing of bidirectional Glenn (BDG) and Fontan stages of single-ventricle palliation

Journal Article JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY · February 1, 2001 Link to item Cite

Real-time volumetric echocardiography: the technology and the possibilities.

Journal Article Echocardiography · November 2000 The heart is a dynamic organ with complexities in its shape. As such, it places special demands on three-dimensional techniques for reconstruction. Real-time volumetric echocardiography, which is based on phased array and parallel processing principles to ... Full text Link to item Cite

Pharmacokinetics, efficacy, and safety of TP10, soluble complement receptor 1, in infants undergoing cardiopulmonary bypass

Conference CIRCULATION · October 31, 2000 Link to item Cite

Clinical information determines the impact of transesophageal echocardiography on the diagnosis of infective endocarditis by the duke criteria.

Journal Article Am Heart J · June 2000 BACKGROUND: Although transesophageal echocardiography (TEE) is more sensitive than transthoracic echocardiography (TTE) in detecting echocardiographic evidence of infective endocarditis (IE), the impact of TEE on the clinical diagnosis of IE has not been c ... Full text Link to item Cite

Clinical auscultation skills in pediatric residents.

Journal Article Pediatrics · June 2000 OBJECTIVE: The aim of this study is to determine the level of clinical auscultation skills in pediatric residents at Duke University Medical Center. METHODS: Forty-seven residents from pediatrics and joint medicine/pediatrics training programs at Duke Univ ... Full text Link to item Cite

Proposed modifications to the Duke criteria for the diagnosis of infective endocarditis.

Journal Article Clin Infect Dis · April 2000 Featured Publication Although the sensitivity and specificity of the Duke criteria for the diagnosis of infective endocarditis (IE) have been validated by investigators from Europe and the United States, several shortcomings of this schema remain. The Duke IE database contains ... Full text Link to item Cite

Prospective analysis of Staphylococcus aureus bacteremia in nonneutropenic adults with malignancy.

Journal Article J Clin Oncol · March 2000 PURPOSE: To determine the primary sources and secondary complications of Staphylococcus aureus bacteremia (SAB) in cancer patients, as well as predictors of outcome in cancer patients with SAB. PATIENTS AND METHODS: Fifty-two patients at Duke University Me ... Full text Link to item Cite

Real-time 3-dimensional echocardiography evaluation of congenital heart disease.

Journal Article J Am Soc Echocardiogr · March 2000 We evaluated the ability of real-time 3-dimensional (RT3D) echocardiography to diagnose congenital heart defects and its potential for presenting structural abnormalities in novel views. Seventy-five patients with suspected congenital heart defects were ex ... Full text Link to item Cite

A comparison of transesophageal and intracardiac induction of atrial fibrillation in children: Implications in Wolff-Parkinson-White syndrome

Journal Article JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY · February 1, 2000 Link to item Cite

Staphylococcus aureus bacteremia in the surgical patient: a prospective analysis of 73 postoperative patients who developed Staphylococcus aureus bacteremia at a tertiary care facility.

Journal Article J Am Coll Surg · January 2000 BACKGROUND: Staphylococcus aureus is a frequent cause of infection and bacteremia in the postoperative patient. Unfortunately, there have been no prospective studies evaluating these patients, so the incidence of complications, subsequent treatment algorit ... Full text Link to item Cite

Cost-effectiveness of transesophageal echocardiography to determine the duration of therapy for intravascular catheter-associated Staphylococcus aureus bacteremia.

Journal Article Ann Intern Med · May 18, 1999 BACKGROUND: The appropriate duration of therapy for catheter-associated Staphylococcus aureus bacteremia is controversial. Conventional practice dictates that all patients receive prolonged courses of intravenous antibiotics. Some clinicians recommend abbr ... Full text Link to item Cite

Echocardiographic characteristics of successful deployment of the Das AngelWings atrial septal defect closure device: initial multicenter experience in the United States.

Journal Article Am J Cardiol · April 15, 1999 The AngelWings device is a newer transcatheter device used for closure of secundum atrial septal defects (ASD) and patent foramen ovale (PFO), which consists of a self-centering, 2-disk system. Transesophageal echocardiography (TEE) plays a pivotal role in ... Full text Link to item Cite

Infective endocarditis due to Staphylococcus aureus: 59 prospectively identified cases with follow-up.

Journal Article Clin Infect Dis · January 1999 Fifty-nine consecutive patients with definite Staphylococcus aureus infective endocarditis (IE) by the Duke criteria were prospectively identified at our hospital over a 3-year period. Twenty-seven (45.8%) of the 59 patients had hospital-acquired S. aureus ... Full text Link to item Cite

Three-dimensional echocardiography in congenital heart disease.

Journal Article Curr Opin Cardiol · January 1999 Recent advances in transducer technology and image processing have led to the development of two techniques for three-dimensional (3-D) echocardiography: 1) 3-D reconstruction and 2) real-time 3-D (RT3-D) volumetric imaging. 3-D reconstruction creates a 3- ... Full text Link to item Cite

Abnormal left ventricular filling after neonatal repair of congenital heart disease: association with increased mortality and morbidity.

Journal Article Am Heart J · December 1998 BACKGROUND: The presence of mid-diastolic flow reversal on the mitral valve Doppler inflow indicates abnormal left ventricular filling. To determine whether mid-diastolic flow reversal predicts outcome in patients undergoing repair or palliation of neonata ... Full text Link to item Cite

Intraoperative transesophageal echocardiography in congenital heart disease.

Journal Article Semin Thorac Cardiovasc Surg · October 1998 This article reviews the use of intraoperative echocardiography during repair of congenital heart defects. Although initial experience was generated using epicardial transducers, there has been a trend in recent years toward the use of transesophageal echo ... Full text Link to item Cite

Evaluation of Ventricular Outflow Tract Abnormalities Using Real-time Three-dimensional Echocardiography

Journal Article Journal of the American College of Cardiology · February 1998 Full text Cite

An evaluation of the Duke criteria in 25 pathologically confirmed cases of prosthetic valve endocarditis.

Journal Article Clin Infect Dis · December 1997 Six studies have compared the sensitivity of the Duke criteria with that of the Beth Israel criteria for the diagnosis of infective endocarditis without attempting to distinguish between native valve and prosthetic valve cases. After reviewing clinical dat ... Full text Link to item Cite

Evaluation of atrioventricular valve abnormalities using real-time three-dimensional echocardiography

Journal Article CIRCULATION · October 21, 1997 Link to item Cite

Role of echocardiography in evaluation of patients with Staphylococcus aureus bacteremia: experience in 103 patients.

Journal Article J Am Coll Cardiol · October 1997 OBJECTIVES: The purpose of this prospective study was to examine the role of echocardiography in patients with Staphylococcus aureus bacteremia (SAB). BACKGROUND: The reported incidence of infective endocarditis (IE) among patients with SAB varies widely. ... Full text Link to item Cite

Role of right ventricular and pulmonary functional abnormalities in limiting exercise capacity in adults with congenital heart disease.

Journal Article Am J Cardiol · August 1, 1997 This study evaluates right ventricular (RV) and pulmonary function during exercise in adults with congenital heart disease (CHD). Thirty-one patients with CHD involving the right side of the heart underwent symptom-limited bicycle exercise testing with sim ... Full text Link to item Cite

Effect of repair strategy on hospital cost for infants with tetralogy of Fallot.

Journal Article Ann Surg · June 1997 OBJECTIVE: This study compares the total hospital cost (HC) for one-stage versus "two-stage" repair of tetralogy of Fallot (TOF) in infants younger than 1 year of age. SUMMARY BACKGROUND DATA: Total (one-stage) correction of TOF is now being performed with ... Full text Link to item Cite

The role of echocardiography in the practice of respiratory care

Journal Article Respiratory Care · March 1, 1996 The changing critical care environment provides a wonderful opportunity for RCPs to expand their influence in the critical care arena. We hope that a better understanding of one of the new advances in critical care diagnostic techniques, echocardiography, ... Cite

Etiology and danger of pericardial effusion in infants and children

Journal Article Cardiology in the Young · January 1, 1996 Pericardial effusion requires attention because of its underlying pathology and its potential for producing hemodynamic compromise. It was our hypothesis that the etiology of and danger from large pericardial effusions are different at different ages. We r ... Full text Cite

Pericarditis due to Mycobacterium aviumintracellulare in an immunocompetent host

Journal Article Infectious Diseases in Clinical Practice · January 1, 1996 Full text Cite

Intraoperative echocardiography during congenital heart operations: experience from 1,000 cases.

Journal Article Ann Thorac Surg · December 1995 BACKGROUND: This article provides an overview of the application of intraoperative echocardiography during repair of congenital heart defects based on our experience with 1,000 patients. METHODS: The patients in this study all underwent repair of a congeni ... Full text Link to item Cite

DOPPLER DETERMINATION OF LEFT-VENTRICULAR DIASTOLIC DYSFUNCTION PREDICTS OUTCOME IN NEONATAL CONGENITAL HEART-DISEASE FOLLOWING REPAIR OR PALLIATION

Journal Article CIRCULATION · October 15, 1995 Link to item Cite

Pulmonary vein Doppler echocardiography after left atrial operation.

Journal Article Ann Thorac Surg · September 1995 BACKGROUND: Intraoperative transesophageal echocardiography provides the surgical team with important guidance during operations for congenital heart disease. Doppler echocardiography adds hemodynamic information to that provided by two-dimensional imaging ... Full text Link to item Cite

Superior vena cava obstruction after extracorporeal membrane oxygenation.

Journal Article J Pediatr · August 1995 Of 60 neonates who survived extracorporeal membrane oxygenation (ECMO) in our institution between June 1992 and March 1994, seven had either complete or partial superior vena cava (SVC) obstruction. When the patients with SVC obstruction were compared with ... Full text Link to item Cite

Cytomegalovirus pericarditis: a case series and review of the literature.

Journal Article Am J Med Sci · April 1995 Cytomegalovirus (CMV) commonly infects both normal and immunocompromised hosts. Although it usually produces an asymptomatic infection to mild illness, CMV has the potential to significantly injure many different organs. Reports of CMV causing pericardial ... Full text Link to item Cite

Relation of left ventricular mass, volume and mass/volume ratio in children with aortic stenosis and/or insufficiency

Journal Article Cardiology in the Young · January 1, 1995 To determine if symptoms and electrocardiographic abnormalities relate to left ventricular mass, volume, and mass/volume ratio in children with aortic stenosis and/or insufficiency, we examined 42 patients (aged 11±6 years) with cross-sectional and Doppler ... Full text Cite

Adult patients with congenital heart disease.

Journal Article N C Med J · April 1994 Adult patients with congenital heart disease present complex problems that require individualized management. Unfortunately, few physicians who care for adults with congenital heart disease have received adequate specific training to deal with their proble ... Link to item Cite

Mycoplasmal pericarditis: evidence of invasive disease.

Journal Article Clin Infect Dis · August 1993 Although the pathogenic mycoplasmas usually infect the respiratory and urogenital tracts, these organisms also can cause disease in remote sites. Such infections are difficult to diagnose because of both the fastidious nature of the mycoplasmas and the fai ... Full text Link to item Cite