Journal ArticleJ Heart Lung Transplant · March 2024
BACKGROUND: Heart failure results in significant morbidity and mortality for young children with hypoplastic left heart syndrome (HLHS) following the Norwood procedure. The trajectory in later childhood is not well described. METHODS: We studied the outcom ...
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Journal ArticleJAMA · January 2, 2024
IMPORTANCE: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until ...
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ConferenceJ Heart Lung Transplant · December 2022
BACKGROUND: Black race is associated with worse outcomes across solid organ transplantation. Augmenting immunosuppression through antithymocyte globulin (ATG) induction may mitigate organ rejection and graft loss. We investigated whether racial and socioec ...
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Journal ArticleJ Heart Lung Transplant · January 2022
BACKGROUND: There is wide variability in the timing of heart transplant (HTx) after pediatric VAD implant. While some centers wait months before listing for HTx, others accept donor heart offers within days of VAD surgery. We sought to determine if HTx wit ...
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Journal ArticleJ Heart Lung Transplant · December 2021
BACKGROUND: Freedom from rejection in pediatric heart transplant recipients is highly variable across centers. This study aimed to assess the center variation in methods used to diagnose rejection in the first-year post-transplant and determine the impact ...
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Journal ArticleJ Am Heart Assoc · September 7, 2021
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 of sudden unexpected death in epilepsy, the pathophysiologic ...
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Journal ArticleNeurology · November 24, 2020
OBJECTIVE: To define the risks and consequences of cardiac abnormalities in ATP1A3-related syndromes. METHODS: Patients meeting clinical diagnostic criteria for rapid-onset dystonia-parkinsonism (RDP), alternating hemiplegia of childhood (AHC), and cerebel ...
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ConferenceCirculation · November 17, 2020
Introduction:
There is wide variability in the timing of heart transplant (HT) listing and consideration of organ offers after ventricular assist device (VAD) implant in children. While some centers w ...
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Journal ArticlePediatr Transplant · August 2019
NDT is a well-defined complication after solid organ transplantation. Little has been published describing the incidence, risk factors, and effect on outcome after pediatric heart transplantation. We performed a retrospective evaluation of pediatric patien ...
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Journal ArticleCatheter Cardiovasc Interv · December 1, 2018
OBJECTIVES: To evaluate differences in radiation dose and image quality across institutions, fluoroscope vendors and generations of fluoroscopes for pediatric cardiac catheterization. BACKGROUND: Increased recognition of the potentially harmful effects of ...
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Journal ArticleJAMA Cardiol · July 1, 2017
IMPORTANCE: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a potentially lethal genetic arrhythmia syndrome characterized by polymorphic ventricular tachycardia with physical or emotional stress, for which current therapy with β-blockers i ...
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Journal ArticleCirculation · November 11, 2016
INTRODUCTION: Increased recognition of the potentially harmful effects of ionizing radiation has spurred technological advances to reduce exposure during fluoroscopy. However there is currently little understanding of the dose-image quality (IQ) relationsh ...
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Journal ArticleAnn Neurol · December 2015
We report 2 patients with drug-resistant epilepsy caused by KCNT1 mutations who were treated with quinidine. Both mutations manifested gain of function in vitro, showing increased current that was reduced by quinidine. One, who had epilepsy of infancy with ...
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Journal ArticleJ Heart Lung Transplant · September 2015
BACKGROUND: Post-Fontan protein-losing enteropathy (PLE) is associated with significant morbidity and mortality. Although heart transplantation (HTx) can be curative, PLE may increase the risk of morbidity before and after HTx. This study analyzed the infl ...
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ConferenceJ Heart Lung Transplant · September 2015
BACKGROUND: Cardiac allograft vasculopathy is an important cause of long-term graft loss. In adults, percutaneous revascularization procedures (PRPs) have variable success with high restenosis rates and little impact on graft survival. Limited data exist i ...
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Journal ArticleJ Am Coll Cardiol · August 4, 2015
BACKGROUND: Recent guidelines recommend assessment of systolic function and filling pressures to augment angiographic grading of cardiac allograft vasculopathy (CAV); however, no data exist on the utility of these guidelines. OBJECTIVES: The aims of this s ...
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Journal ArticleCirc Arrhythm Electrophysiol · August 2014
BACKGROUND: Pediatric patients with persistent arrhythmias may require mechanical cardiopulmonary support. We sought to classify the population, spectrum, and success of current treatment strategies. METHODS AND RESULTS: A multicenter retrospective chart r ...
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Journal ArticlePacing Clin Electrophysiol · March 2013
INTRODUCTION: Patients with congenital heart disease (CHD) and implanted cardiac rhythm management devices have a high rate of endocardial lead issues requiring lead extraction. Laser lead extraction (LLE) is a promising modality for lead management though ...
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Journal ArticleJ Am Soc Echocardiogr · August 2012
BACKGROUND: Clinical trials often rely on echocardiographic measures of left ventricular size and function as surrogate end points. However, the quantitative impact of factors that affect the reproducibility of these measures is unknown. To address this is ...
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Journal ArticleCirculation · January 3, 2012
BACKGROUND: Brugada syndrome is a potentially serious channelopathy that usually presents in adulthood and has only rarely been described in infancy. In the absence of metabolic or structural cardiac disease, rapid ventricular tachycardia (>200 bpm) and pr ...
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Journal ArticlePediatr Cardiol · August 2011
Long QT syndrome is a well-described entity in infants. Its presentation in the context of congenital heart disease is rare and is almost exclusively diagnosed postoperatively. For patients undergoing surgical intervention, preoperative knowledge of the di ...
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Journal ArticleMuscle Nerve · November 2010
LMNA-related congenital muscular dystrophy (L-CMD) is a recently described disorder characterized by infantile-onset myopathy due to mutations in the lamin A/C (LMNA) gene. We report the genetic and clinical characteristics of two unrelated L-CMD patients. ...
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Journal ArticleJ Cardiovasc Electrophysiol · February 2009
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BACKGROUND: The presence of a single left superior vena cava in the absence of complex congenital heart disease is uncommon, and, in the absence of hemodynamic consequences, it would not be expected to result in cardiovascular signs or symptoms. Single cas ...
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Journal ArticleGenet Med · May 2006
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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 ...
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Journal ArticleHeart Rhythm · January 2006
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BACKGROUND: The incidence of atrial tachycardia following Fontan surgery is high, but access to the pulmonary venous atrium, a frequent site of arrhythmia origin, is limited. OBJECTIVES: The purpose of this study is to report our results with a novel trans ...
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Journal ArticleJ Membr Biol · September 2005
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Voltage- and/or conformation-dependent association and dissociation of local anesthetic-class drugs from a putative receptor site in domain IV S6 of the sodium channel and slow conformation transitions of the drug-associated channel have been proposed as m ...
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Journal ArticlePediatr Cardiol · 2005
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Barth syndrome is an X-linked disorder characterized by dilated cardiomyopathy, cyclic neutropenia, skeletal myopathy, abnormal mitochondria, and growth deficiency. The primary defect is a mutation in the TAZ gene on the X chromosome at Xq28, resulting in ...
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Journal ArticlePacing Clin Electrophysiol · July 2004
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The purpose of the study was to investigate the results of endocardial lead implantation, lead performance, and follow-up in young patients after the Fontan procedure. A retrospective study was conducted with patients who had endocardial atrial pacing for ...
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Journal ArticleJ Clin Invest · October 2002
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The function of the 12 positive charges in the 53-residue III/IV interdomain linker of the cardiac Na(+) channel is unclear. We have identified a four-generation family, including 17 gene carriers with long QT syndrome, Brugada syndrome, and conduction sys ...
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Journal ArticleBiophys J · December 2000
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The role of inactivation as a central mechanism in blockade of the cardiac Na(+) channel by antiarrhythmic drugs remains uncertain. We have used whole-cell and single channel recordings to examine the block of wild-type and inactivation-deficient mutant ca ...
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Journal ArticleJ Am Coll Cardiol · February 2000
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OBJECTIVES: The purpose of this study was to determine the efficacy and risks of radiofrequency ablation of various forms of supraventricular tachycardia after Mustard and Senning operations for d-transposition of the great arteries. BACKGROUND: In this pa ...
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Journal ArticleCardiol Rev · 2000
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Pediatric cardiologists and cardiologists who examine adults frequently receive referrals of adolescents and young adults with chest pain. Chest pain in this age group is most likely noncardiac in origin, which often creates a diagnostic dilemma for the co ...
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Journal ArticlePediatr Cardiol · 1997
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Persistent patency of the ductus arteriosus (PDA), common in premature infants, is associated with severe respiratory distress. The likelihood and significance of finding PDA in premature infants beyond the second week of life is unknown. We retrospectivel ...
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