A5037149199- Cardiac Arrhythmias and Treatments
- Cardiac pacing and defibrillation studies
- Cardiac electrophysiology and arrhythmias
- Cardiomyopathy and Myosin Studies
- Congenital Heart Disease Studies
- Cardiac Structural Anomalies and Repair
- Mechanical Circulatory Support Devices
- Atrial Fibrillation Management and Outcomes
- Cardiovascular Function and Risk Factors
- Cardiovascular Effects of Exercise
- Cardiac Arrest and Resuscitation
- Ion Transport and Channel Regulation
- Ion channel regulation and function
- Kawasaki Disease and Coronary Complications
- Cardiovascular Syncope and Autonomic Disorders
- Heart Rate Variability and Autonomic Control
- Transplantation: Methods and Outcomes
- Genetics and Neurodevelopmental Disorders
- Parathyroid Disorders and Treatments
- Cardiac Valve Diseases and Treatments
- Neurological and metabolic disorders
- Metabolism and Genetic Disorders
- Medical Imaging and Pathology Studies
- Genetics and Physical Performance
- ATP Synthase and ATPases Research
Baylor College of Medicine
2019-2025
Texas Children's Hospital
2019-2025
Imperial College Healthcare NHS Trust
2023
Baylor University
2023
University of California, Los Angeles
2023
Boston Children's Hospital
2016-2018
Harvard University
2016-2018
The Ohio State University Wexner Medical Center
2018
The Ohio State University
2018
The University of Texas Medical Branch at Galveston
2012
Hypertrophic cardiomyopathy is the leading cause of sudden cardiac death (SCD) in children and young adults. Our objective was to develop validate a SCD risk prediction model pediatric hypertrophic guide prevention strategies. In an international multicenter observational cohort study, phenotype-positive patients with isolated <18 years age at diagnosis were eligible. The primary outcome variable time from composite events 5-year follow-up: SCD, resuscitated arrest, aborted that is,...
Importance Alternating hemiplegia of childhood (AHC) is a disorder that can result from pathogenic variants in ATP1A3 -encoded sodium-potassium adenosine triphosphatase alpha 3 (ATP1A3). While AHC primarily neurologic disease, some individuals experience sudden unexplained death (SUD) potentially associated with cardiac arrhythmias. Objective To determine the impact on electrophysiology and whether lethal ventricular arrhythmias are SUD patients AHC. Design, Setting, Participants In this...
Transcatheter Leadless Pacemakers (TLP) are a safe and effective option for adults with pacing indications. These devices may be an alternative in pediatric patients congenital heart disease whom repeated sternotomies, thoracotomies, or transvenous systems unfavorable. However, exemption of children from clinical trials has created uncertainty over the indications, efficacy, safety TLP population. The objectives this study to evaluate procedural characteristics, electrical performance,...
Genetic defects in the RAS/mitogen-activated protein kinase pathway are an important cause of hypertrophic cardiomyopathy (RAS-HCM). Unlike primary HCM (P-HCM), risk sudden cardiac death (SCD) and long-term survival RAS-HCM poorly understood.The study's objective was to compare transplant-free survival, incidence SCD, implantable cardioverter-defibrillator (ICD) use between P-HCM patients.In international, 21-center cohort study, we analyzed phenotype-positive pediatric (n = 188) 567)...
There is a paucity of data regarding the impact extracorporeal membrane oxygenation support, adequacy surgical repair, and timing intervention for residual structural lesions in neonates cannulated to after cardiac surgery. Our goal was determine how these factors were associated with survival.Retrospective study.Cardiovascular ICU.Neonates (≤ 28 d old) congenital heart disease surgery during 2006-2013.None.Eighty-four venoarterial Survival discharge 50%. no difference survival based on...
Hypertrophic cardiomyopathy (HCM) can be associated with an abnormal exercise response. In adults HCM, results on stress testing are predictive of heart failure outcomes. Our goal was to determine whether response is adverse outcomes in pediatric patients HCM. international cohort study including 20 centers, phenotype-positive primary HCM who were <18 years age at diagnosis included. Abnormal defined as a blunted blood pressure and new or worsened ST- T-wave segment changes complex...
Heart failure (HF) remains a major source of morbidity and mortality in the US. The multifunctional Ca2+/calmodulin-dependent kinase II (CaMKII) has emerged as critical regulator cardiac hypertrophy failure, although mechanisms remain unclear. Previous studies have established that cytoskeletal protein βIV-spectrin coordinates local CaMKII signaling. Here, we sought to determine role spectrin-CaMKII complex maladaptive remodeling HF. Chronic pressure overload (6 weeks transaortic...
The mechanisms underlying Ca2+/calmodulin-dependent protein kinase II (CaMKII)-induced arrhythmias in ischemia-reperfusion (I/R) are not fully understood. We tested the hypothesis that CaMKII increases late Na+ current ( INa,L) via phosphorylation of Nav1.5 at Ser571 during I/R, thereby increasing arrhythmia susceptibility. To test our hypothesis, we studied isolated, Langendorff-perfused hearts from wild-type (WT) mice and expressing Nav channel variants Nav1.5-Ser571E (S571E)...
The aim of this study was to evaluate cardiac function and clinical outcomes in perioperative pediatric burn patients. Transesophageal echocardiography data were collected on 40 patients from 2004 2007. Of the who received exams, a complete set parameters outcome variables obtained 26 mean age 9.7 ± 0.9 years, TBSA size 64 3%. Patients divided into two groups based systolic function. One group represented with ejection fractions >50% other ≤50%. Clinical then compared among groups. In our...
Extracorporeal membrane oxygenation is an established therapy for cardiac and respiratory failure unresponsive to usual care. mortality remains high, with ongoing risk of death even after successful decannulation. We describe occurrence factors associated in children weaned from extracorporeal oxygenation.Retrospective cohort study.Two hundred five centers reporting the Life Support Organization.Eleven thousand ninety-six patients, less than 18 years, supported during 2007-2013, who achieved...
BACKGROUND: DSP cardiomyopathy is a distinct subset of arrhythmogenic cardiomyopathy, reported primarily in adults, that has predominantly left ventricular involvement and features myocarditis. Clinical characteristics, risk stratification, management pediatric patients with variants are not well known. We sought to identify phenotypic prognosis pathogenic or likely variants. METHODS: Multicenter, retrospective study <21 years age from 6 tertiary hospitals. RESULTS: Thirty-four patients,...
Background Pathogenic variation in the ATP1A3-encoded sodium-potassium ATPase, ATP1A3, is responsible for alternating hemiplegia of childhood (AHC). Although these patients experience a high rate sudden unexpected death epilepsy, pathophysiologic basis this risk remains unknown. The objective was to determine role ATP1A3 genetic variants on cardiac outcomes as determined by QT and corrected (QTc) measurements. Methods Results We analyzed 12-lead ECG recordings from 62 (male subjects=31,...
Ablation for atrioventricular nodal reentrant tachycardia (AVNRT) classically utilizes evaluation of signal morphology within the anatomic region slow pathway (SP), which involves subjectivity. Ripple mapping (RM; CARTO-3© Biosense Webster Inc) displays each electrogram at its three-dimensional coordinate as a bar changing in length according to voltage-time relationship. This allows prolonged, low-amplitude signals be displayed their entirety, helping identify propagation low-voltage areas....