Charles I Berul

Summary

Affiliation: Children's Hospital
Country: USA

Publications

  1. pmc Molecular mechanisms of inherited arrhythmias
    Cordula M Wolf
    Department of Cardiology, Children s Hospital Boston, Department of Pediatrics, Harvard Medical School, Boston, MA, USA
    Curr Genomics 9:160-8. 2008
  2. doi Implantable cardioverter defibrillator criteria for primary and secondary prevention of pediatric sudden cardiac death
    Charles I Berul
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pacing Clin Electrophysiol 32:S67-70. 2009
  3. ncbi Indications and techniques of pediatric cardiac pacing
    Charles I Berul
    Department of Cardiology, Children s Hospital Boston, MA 02115, USA
    Expert Rev Cardiovasc Ther 1:165-76. 2003
  4. doi Results of a multicenter retrospective implantable cardioverter-defibrillator registry of pediatric and congenital heart disease patients
    Charles I Berul
    Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Am Coll Cardiol 51:1685-91. 2008
  5. ncbi Pacemaker lead prolapse through the pulmonary valve in children
    Charles I Berul
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pacing Clin Electrophysiol 30:1183-9. 2007
  6. ncbi Electrophysiological phenotyping in genetically engineered mice
    Charles I Berul
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Physiol Genomics 13:207-16. 2003
  7. pmc Prevention of ventricular arrhythmia and calcium dysregulation in a catecholaminergic polymorphic ventricular tachycardia mouse model carrying calsequestrin-2 mutation
    Ronny Alcalai
    Department of Genetics, Harvard Medical School, Boston, Massachusetts, USA
    J Cardiovasc Electrophysiol 22:316-24. 2011
  8. ncbi Circadian and seasonal variation of malignant arrhythmias in a pediatric and congenital heart disease population
    Elizabeth A Stephenson
    Departmentof Cardilogy, Children s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Cardiovasc Electrophysiol 13:1009-14. 2002
  9. doi System survival of nontransvenous implantable cardioverter-defibrillators compared to transvenous implantable cardioverter-defibrillators in pediatric and congenital heart disease patients
    Andrew E Radbill
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA
    Heart Rhythm 7:193-8. 2010
  10. pmc Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease
    Cordula M Wolf
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, MA 02115, USA
    J Mol Cell Cardiol 44:293-303. 2008

Detail Information

Publications86

  1. pmc Molecular mechanisms of inherited arrhythmias
    Cordula M Wolf
    Department of Cardiology, Children s Hospital Boston, Department of Pediatrics, Harvard Medical School, Boston, MA, USA
    Curr Genomics 9:160-8. 2008
    ....
  2. doi Implantable cardioverter defibrillator criteria for primary and secondary prevention of pediatric sudden cardiac death
    Charles I Berul
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pacing Clin Electrophysiol 32:S67-70. 2009
    ....
  3. ncbi Indications and techniques of pediatric cardiac pacing
    Charles I Berul
    Department of Cardiology, Children s Hospital Boston, MA 02115, USA
    Expert Rev Cardiovasc Ther 1:165-76. 2003
    ..This article will first review the indications for permanent pacing in children and will then describe the unique challenges associated with such use...
  4. doi Results of a multicenter retrospective implantable cardioverter-defibrillator registry of pediatric and congenital heart disease patients
    Charles I Berul
    Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Am Coll Cardiol 51:1685-91. 2008
    ..We sought to determine the implications of implantable cardioverter-defibrillator (ICD) placement in children and patients with congenital heart disease (CHD)...
  5. ncbi Pacemaker lead prolapse through the pulmonary valve in children
    Charles I Berul
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pacing Clin Electrophysiol 30:1183-9. 2007
    ..This excess lead may rarely prolapse into the pulmonary artery and potentially interfere with valve function. We sought to determine the response to lead repositioning on pulmonary valve insufficiency...
  6. ncbi Electrophysiological phenotyping in genetically engineered mice
    Charles I Berul
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Physiol Genomics 13:207-16. 2003
    ..This paper reviews current in vivo murine electrophysiology study techniques and genetic mouse models pertinent to human arrhythmia disorders...
  7. pmc Prevention of ventricular arrhythmia and calcium dysregulation in a catecholaminergic polymorphic ventricular tachycardia mouse model carrying calsequestrin-2 mutation
    Ronny Alcalai
    Department of Genetics, Harvard Medical School, Boston, Massachusetts, USA
    J Cardiovasc Electrophysiol 22:316-24. 2011
    ..Mechanistic studies indicate that CPVT is mediated by diastolic Ca(2+) overload and increased Ca(2+) leak through the RyR2 channel, implying that treatment targeting these defects might be efficacious in CPVT...
  8. ncbi Circadian and seasonal variation of malignant arrhythmias in a pediatric and congenital heart disease population
    Elizabeth A Stephenson
    Departmentof Cardilogy, Children s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Cardiovasc Electrophysiol 13:1009-14. 2002
    ..We sought to establish whether pediatric and young adult congenital heart disease implantable cardioverter defibrillator (ICD) recipients have circadian or seasonal variability in shock frequency, similar to adult populations...
  9. doi System survival of nontransvenous implantable cardioverter-defibrillators compared to transvenous implantable cardioverter-defibrillators in pediatric and congenital heart disease patients
    Andrew E Radbill
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA
    Heart Rhythm 7:193-8. 2010
    ..Outcomes with these hybrid configurations are not well understood...
  10. pmc Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease
    Cordula M Wolf
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, MA 02115, USA
    J Mol Cell Cardiol 44:293-303. 2008
    ....
  11. doi Cardiac resynchronization therapy (and multisite pacing) in pediatrics and congenital heart disease: five years experience in a single institution
    Frank Cecchin
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Cardiovasc Electrophysiol 20:58-65. 2009
    ..We sought to determine the functional assessment and clinical outcomes in pediatric and CHD CRT patients followed uniformly at one institution...
  12. ncbi Impact of transvenous ventricular pacing leads on tricuspid regurgitation in pediatric and congenital heart disease patients
    Gregory Webster
    Department of Cardiology, Children s Hospital, Boston, 300 Longwood Ave, Boston, MA 02115, USA
    J Interv Card Electrophysiol 21:65-8. 2008
    ..The literature in adults is inconclusive and no studies have investigated the association between pacing leads and TR in children or congenital heart disease patients...
  13. pmc Reversibility of PRKAG2 glycogen-storage cardiomyopathy and electrophysiological manifestations
    Cordula M Wolf
    Department of Cardiology, Children s Hospital Boston, Boston, MA 02115, USA
    Circulation 117:144-54. 2008
    ..A genetic approach that utilizes a binary inducible transgenic system was used to investigate the disease mechanism and to assess preventability and reversibility of disease features in a mouse model of glycogen-storage cardiomyopathy...
  14. ncbi Heart block, ventricular tachycardia, and sudden death in ACE2 transgenic mice with downregulated connexins
    Mary Donoghue
    Millennium Pharmaceuticals, Inc, Cambridge, MA, USA
    J Mol Cell Cardiol 35:1043-53. 2003
    ..Spontaneous downregulation of the ACE2 transgene in surviving older animals correlated with restoration of nearly normal conduction, rhythm, and connexin expression...
  15. ncbi Permanent atrial pacing lead implant route after Fontan operation
    Kazuhiro Takahashi
    Department of Cardiology, Children s Hospital Boston, Boston, MA 02115, USA
    Pacing Clin Electrophysiol 32:779-85. 2009
    ..Atrial pacing is indicated for sinus node dysfunction (SND) after Fontan surgery; preferred lead implantation technique is debated. We compare outcomes of transvenous (TV) and epicardial (Epi) atrial lead implants in this population...
  16. ncbi Absence of Msx2 does not affect cardiac conduction or rescue conduction defects associated with Nkx2-5 mutation
    Patrick Y Jay
    Department of Cardiology, Children s Hospital, Boston, Massachusetts 02139, USA
    J Cardiovasc Electrophysiol 16:82-5. 2005
    ..Several observations support a model in which Msx2 negatively regulates formation of the conduction system and inappropriate Msx2 up-regulation causes the conduction defects associated with Nkx2-5 mutation...
  17. ncbi Relation of right ventricular pacing in tetralogy of Fallot to electrical resynchronization
    Elizabeth A Stephenson
    Department of Cardiology, Children s Hospital, 300 Longwood Avenue, Boston, MA 02115, USA
    Am J Cardiol 93:1449-52, A12. 2004
    ..Effective pacing therapy may require site-specific placement of pacing leads and precise pacemaker programming...
  18. doi Digital music players cause interference with interrogation telemetry for pacemakers and implantable cardioverter-defibrillators without affecting device function
    Gregory Webster
    Department of Cardiology, Children s Hospital, and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    Heart Rhythm 5:545-50. 2008
    ..A preliminary study reported interference in 50% of patients whose devices were interrogated near Apple iPods...
  19. ncbi Comparison of modern steroid-eluting epicardial and thin transvenous pacemaker leads in pediatric and congenital heart disease patients
    Elizabeth B Fortescue
    Department of Cardiology, Children s Hospital Boston, Boston, MA 02115, USA
    J Interv Card Electrophysiol 14:27-36. 2005
    ..We compared performances of modern thin transvenous (TTV) and steroid-eluting epicardial (SEE) leads in patients followed at one pediatric center...
  20. ncbi Cardiac electrophysiological phenotypes in postnatal expression of Nkx2.5 transgenic mice
    Hiroko Wakimoto
    Department of Cardiology, Children s Hospital, Boston, Massachusetts 02115, USA
    Genesis 37:144-50. 2003
    ..5 also causes conduction abnormalities, although the onset is after the neonatal stage. Bradycardia and AV conduction failure may contribute to the lethal heart failure and early mortality...
  21. doi Lead extraction in pediatric and congenital heart disease patients
    Frank Cecchin
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA
    Circ Arrhythm Electrophysiol 3:437-44. 2010
    ..Data on the safety and efficacy of different lead extraction techniques in this specific patient population are limited...
  22. ncbi Age, size, and lead factors alone do not predict venous obstruction in children and young adults with transvenous lead systems
    Yaniv Bar-Cohen
    From the Department of Cardiology, Children s Hospital Boston, Boston, Massachusetts, USA
    J Cardiovasc Electrophysiol 17:754-9. 2006
    ..Venous occlusion is a recognized complication of transvenous pacing, and lead cross-sectional area indexed to body surface area (BSA) has been used to predict venous obstruction in children...
  23. pmc Multiple accessory pathways in the young: the impact of structural heart disease
    Justin P Zachariah
    Department of Cardiology, Boston Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA, USA
    Am Heart J 165:87-92. 2013
    ..In a large cohort of pediatric patients with and without SHD undergoing invasive electrophysiology study, we examined the prevalence of MultAP and the effect of both MultAP and SHD on ablation outcomes...
  24. ncbi Function follows form: cardiac conduction system defects in Nkx2-5 mutation
    Patrick Y Jay
    Department of Cardiology, Children s Hospital, Boston, Massachusetts, USA
    Anat Rec A Discov Mol Cell Evol Biol 280:966-72. 2004
    ..Hypoplasia of the atrioventricular node, His bundle, and Purkinje system can explain in whole or in part specific conduction and electrophysiologic defects present in Nkx2-5 haploinsufficiency...
  25. ncbi Cryoablation for accessory pathways located near normal conduction tissues or within the coronary venous system in children and young adults
    Yaniv Bar-Cohen
    Electrophysiology Division, Department of Cardiology, Children s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA
    Heart Rhythm 3:253-8. 2006
    ..Cryoablation may offer advantages over radiofrequency (RF) ablation for certain arrhythmia substrates, such as septal accessory pathways (APs). Data for young patients, especially regarding recurrence risk, require expansion...
  26. ncbi Patient, procedural, and hardware factors associated with pacemaker lead failures in pediatrics and congenital heart disease
    Elizabeth B Fortescue
    Arrhythmia Service, Department of Cardiology, Children s Hospital Boston and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
    Heart Rhythm 1:150-9. 2004
    ..To examine outcomes of children with pacemakers over a 22-year period and identify risk factors for lead failure...
  27. doi Cardiac tumors and associated arrhythmias in pediatric patients, with observations on surgical therapy for ventricular tachycardia
    Christina Y Miyake
    Department of Cardiology, Children s Hospital Boston, Massachusetts 02115, USA
    J Am Coll Cardiol 58:1903-9. 2011
    ..The aim of this study was to describe a large experience with primary cardiac tumors in pediatric patients, characterize associated arrhythmias, and expand knowledge of natural history and treatment options...
  28. doi Nonfluoroscopic imaging systems reduce radiation exposure in children undergoing ablation of supraventricular tachycardia
    Christina Y Miyake
    Department of Cardiology, Children s Hospital Boston, Boston, Massachusetts 02115, USA
    Heart Rhythm 8:519-25. 2011
    ..The current standard of care for imaging during supraventricular tachycardia (SVT) ablation uses fluoroscopy, which exposes otherwise healthy children to the potential harmful effects of radiation...
  29. ncbi Increased alpha2 subunit-associated AMPK activity and PRKAG2 cardiomyopathy
    Ferhaan Ahmad
    Department of Genetics, Harvard Medical School, Howard Hughes Medical Institute, Boston, MA, USA
    Circulation 112:3140-8. 2005
    ..Mice overexpressing a dominant-negative alpha2 subunit of AMPK (TGalpha2DN) provide a tool for selectively inhibiting alpha2, but not alpha1, subunit-associated AMPK activity...
  30. pmc Circulating matrix metalloproteinases in adolescents with hypertrophic cardiomyopathy and ventricular arrhythmia
    Justin P Zachariah
    Department of Cardiology, Children s Hospital Boston and Department of Pediatrics, Harvard Medical School, 300 Longwood Ave, Boston, MA 02115, USA
    Circ Heart Fail 5:462-6. 2012
    ..We explored differences in circulating markers of extracellular matrix turnover between young HCM patients with versus without history of serious arrhythmia...
  31. ncbi Ascending aortic dilation in patients with congenital complete heart block
    Andrew E Radbill
    Department of Cardiology, Children s Hospital Boston, and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
    Heart Rhythm 5:1704-8. 2008
    ..Aortic dilation has been anecdotally observed in some children with CCHB, but detailed reports are lacking...
  32. doi Atrioventricular nodal reentrant tachycardia with 2:1 block in pediatric patients
    Tarun Mahajan
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston Massachusetts 02115, USA
    Heart Rhythm 5:1391-5. 2008
    ..Episodic 2:1 block occurs in 9% of adults with atrioventricular nodal reentry tachycardia (AVNRT), but developmental differences in conduction physiology among children could influence this phenomenon...
  33. pmc Ablation of LKB1 in the heart leads to energy deprivation and impaired cardiac function
    Niels Jessen
    Joslin Diabetes Center, Boston, MA 02215, USA
    Biochim Biophys Acta 1802:593-600. 2010
    ..Ablation of LKB1 leads to a decreased cardiac efficiency despite normal mitochondrial oxidative metabolism...
  34. doi Mitral valve replacement in infants and children 5 years of age or younger: evolution in practice and outcome over three decades with a focus on supra-annular prosthesis implantation
    Elif Seda Selamet Tierney
    Department of Cardiology, Children s Hospital, Boston, Massachusetts 02115, USA
    J Thorac Cardiovasc Surg 136:954-61, 961.e1-3. 2008
    ..Supra-annular prosthesis implantation can facilitate mitral valve replacement with a larger prosthesis in children with a small annulus, but little is known about its effect on the outcomes of mitral valve replacement in young children...
  35. ncbi Radiofrequency catheter ablation of septal accessory pathways in the pediatric age group
    Ravi Mandapati
    Department of Cardiology, Boston Children s Hospital, Boston, Massachusetts 02115, USA
    Am J Cardiol 92:947-50. 2003
    ..The risks of AV block and accessory pathway recurrence are most relevant to anteroseptal and midseptal pathways. These data may be factored into patient selection and the decision whether to ablate...
  36. doi Prospective evaluation of defibrillation threshold and postshock rhythm in young ICD recipients
    Andrew E Radbill
    Department of Pediatrics, Vanderbilt University School of Medicine, and the Monroe Carell Jr Children s Hospital at Vanderbilt, Nashville, Tennessee 37232 9119, USA
    Pacing Clin Electrophysiol 35:1487-93. 2012
    ..We describe the first prospective evaluation of defibrillation threshold (DFT) and postshock rhythm in this population...
  37. ncbi Pediatric nonpost-operative junctional ectopic tachycardia medical management and interventional therapies
    Kathryn K Collins
    Department of Pediatrics, Division of Cardiology, The Children s Hospital, 13123 E 16th Avenue, Aurora, CO 80045, USA
    J Am Coll Cardiol 53:690-7. 2009
    ..To determine the outcomes of medical management, pacing, and catheter ablation for the treatment of nonpost-operative junctional ectopic tachycardia (JET) in a pediatric population...
  38. pmc Somatic events modify hypertrophic cardiomyopathy pathology and link hypertrophy to arrhythmia
    Cordula M Wolf
    Department of Cardiology, Children s Hospital, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 102:18123-8. 2005
    ..We suggest that a shared pathway triggered by sarcomere gene mutations links cardiac hypertrophy and arrhythmias in HCM...
  39. ncbi The T-Box transcription factor Tbx5 is required for the patterning and maturation of the murine cardiac conduction system
    Ivan P G Moskowitz
    Department of Genetics, Harvard Medical School and Howard Hughes Medical Institute, Boston, MA 02115, USA
    Development 131:4107-16. 2004
    ..We conclude that Tbx5 is required for Cx40-independent patterning of the cardiac conduction system, and suggest that the electrophysiologic defects in Holt-Oram syndrome reflect a developmental abnormality of the conduction system...
  40. ncbi Functional role of AMP-activated protein kinase in the heart during exercise
    Nicolas Musi
    Research Division, Joslin Diabetes Center, One Joslin Place, Boston, MA 02215, USA
    FEBS Lett 579:2045-50. 2005
    ..We found that inactivation of AMPK did not result in abnormal ATP and glycogen consumption during exercise, cardiac function assessed by heart rhythm telemetry and stress echocardiography, or in maximal exercise capacity...
  41. ncbi Cardiac electrophysiological characteristics of the mdx ( 5cv ) mouse model of Duchenne muscular dystrophy
    Dorothy M Branco
    Department of Cardiology, Children s Hospital Boston, Boston, MA 02115, USA
    J Interv Card Electrophysiol 20:1-7. 2007
    ..Cardiomyopathy, conduction abnormalities, and ventricular arrhythmias are significant complications of this disease. The mdx ( 5cv ) mouse carries a dystrophin mutation and demonstrates a more severe phenotype than the classic mdx mouse...
  42. ncbi Radiofrequency catheter ablation of ventricular tachycardia in children and young adults with congenital heart disease
    James G Morwood
    Department of Cardiology, Boston Children s Hospital, Harvard Medical School, Boston, Massachusetts, USA
    Heart Rhythm 1:301-8. 2004
    ..The aim of this study was to expand data on outcomes for radiofrequency catheter ablation (RFCA) of ventricular tachycardia (VT) in young patients and to identify obstacles to success...
  43. ncbi Utility of routine follow-up defibrillator threshold testing in congenital heart disease and pediatric populations
    Elizabeth A Stephenson
    Department of Cardiology, Children s Hospital, Boston, Boston, Massachusetts 02115, USA
    J Cardiovasc Electrophysiol 16:69-73. 2005
    ..The congenital heart disease and pediatric populations are a unique group of ICD recipients having a higher incidence of lead failure. We investigated the utility of follow-up DFT testing in this population...
  44. ncbi Implications of implantable cardioverter defibrillator therapy in congenital heart disease and pediatrics
    Mark E Alexander
    Arrhythmia Service, Department of Cardiology, Children s Hospital, Boston, Massachusetts 02115, USA
    J Cardiovasc Electrophysiol 15:72-6. 2004
    ..The incidence of appropriate and inappropriate discharges, indicators of system failure, and clinical implications of implantable cardioverter defibrillator (ICD) therapy in children and young adults with heart disease is poorly defined...
  45. pmc Nkx2-5 mutation causes anatomic hypoplasia of the cardiac conduction system
    Patrick Y Jay
    Department of Cardiology, Children s Hospital, Boston, Massachusetts 02115, USA
    J Clin Invest 113:1130-7. 2004
    ..Postnatal conduction defects in Nkx2-5 mutation may result at least in part from a defect in the genetic program that governs the recruitment or retention of embryonic cardiac myocytes in the conduction system...
  46. ncbi Nkx2.5 homeoprotein regulates expression of gap junction protein connexin 43 and sarcomere organization in postnatal cardiomyocytes
    Hideko Kasahara
    Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, SL201, Boston, MA 02215, USA
    J Mol Cell Cardiol 35:243-56. 2003
    ..5 overexpression but not due to heart failure phenotype in vivo. These studies indicate that overexpression of Nkx2.5 in terminally differentiated cardiomyocytes dramatically alters cardiac cell structure and function...
  47. pmc Calsequestrin 2 (CASQ2) mutations increase expression of calreticulin and ryanodine receptors, causing catecholaminergic polymorphic ventricular tachycardia
    Lei Song
    Department of Genetics, Harvard Medical School, Boston, MA 02115, USA
    J Clin Invest 117:1814-23. 2007
    ..The central role of RyR2 dysfunction in CASQ2 deficiency unifies the pathophysiologic mechanism underlying CPVT due to RyR2 or CASQ2 mutations and suggests a therapeutic approach for these inherited cardiac arrhythmias...
  48. ncbi Implications of ventricular arrhythmia vulnerability during murine electrophysiology studies
    Colin T Maguire
    Department of Cardiology, Children s Hospital, Boston, Massachusetts 02115, USA
    Physiol Genomics 15:84-91. 2003
    ..These factors should be carefully considered when analyzing PES and burst pacing data in murine models to minimize false positives and optimize accuracy...
  49. doi Ectopic atrial rhythm is a preablation predictor of atrioventricular nodal reentrant tachycardia in children
    Christina Y Miyake
    Department of Cardiology, Children s Hospital Boston, MA 02115, USA
    Pediatr Cardiol 29:1066-70. 2008
    ..Clinical experience suggested that ectopic atrial rhythm (EAR) is more frequently observed on preablation ECGs of pediatric patients with AVNRT. This study aimed to determine whether EAR is predictive of AVNRT...
  50. doi Impact of manufacturer advisories and FDA recalls of implantable cardioverter defibrillator generators in pediatric and congenital heart disease patients
    Tarun Mahajan
    Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Cardiovasc Electrophysiol 19:1270-4. 2008
    ..This study was undertaken to determine implications of ICD manufacturers' advisories and recalls on children and CHD patients...
  51. ncbi Cardiac conduction and arrhythmia: insights from Nkx2.5 mutations in mouse and humans
    Patrick Y Jay
    Department of Cardiology, Children s Hospital, Boston, MA 02115, USA
    Novartis Found Symp 250:227-38; discussion 238-41, 276-9. 2003
    ..We propose that consideration of the two alternative hypotheses, in addition to the traditional ICC-centric model, should lead to a richer understanding of cardiac conduction defects and arrhythmogenesis...
  52. doi Congenital long-QT syndromes: a clinical and genetic update from infancy through adulthood
    Gregory Webster
    Department of Cardiology, Children s Hospital Boston, Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    Trends Cardiovasc Med 18:216-24. 2008
    ..Future research will determine the etiology of the remaining cases, further risk-stratify the known genetic defects, improve current treatment options for these syndromes, and uncover novel therapies...
  53. doi How revealing are insertable loop recorders in pediatrics?
    Patricia A Frangini
    Department of Cardiology, Children s Hospital Boston, Boston, Massachusetts 02115, USA
    Pacing Clin Electrophysiol 31:338-43. 2008
    ..We sought to determine the diagnostic utility of the Reveal ILR (Medtronic, Inc., Minneapolis, MN, USA) in pediatric patients...
  54. doi Pulmonary valve replacement in tetralogy of Fallot: impact on survival and ventricular tachycardia
    David M Harrild
    Department of Cardiology, Children s Hospital Boston, Boston, MA 02115, USA
    Circulation 119:445-51. 2009
    ..This study aimed to determine the incidence of death and VT in TOF after PVR and to test the hypothesis that PVR leads to improvement in these outcomes...
  55. ncbi Inaccuracy of Wolff-Parkinson-white accessory pathway localization algorithms in children and patients with congenital heart defects
    Yaniv Bar-Cohen
    Electrophysiology Division, Department of Cardiology, Children s Hospital Boston, Boston, Massachusetts 02115, USA
    J Cardiovasc Electrophysiol 17:712-6. 2006
    ..We hypothesize that these algorithms have low diagnostic accuracy in children and even lower in those with CHD...
  56. ncbi Microvolt t-wave alternans with exercise in pediatrics and congenital heart disease: limitations and predictive value
    Mark E Alexander
    Arrhythmia Service, Department of Cardiology, Children s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pacing Clin Electrophysiol 29:733-41. 2006
    ..The incidence and potential significance of sustained TWA in pediatric and congenital heart disease (CHD) populations has not been well defined...
  57. ncbi Characterization of cardiac conduction system abnormalities in mice with targeted disruption of Six5 gene
    Hiroko Wakimoto
    Department of Cardiology, Children s Hospital, Boston, Massachusetts 02115, USA
    J Interv Card Electrophysiol 7:127-35. 2002
    ..Six5 loss may partly contribute to conduction abnormalities in myotonic dystrophy, particularly infraHisian conduction delay, one of the initial phenotypes of adult-onset cardiac conduction abnormalities in DM patients...
  58. ncbi Efficacy of atrial antitachycardia pacing using the Medtronic AT500 pacemaker in patients with congenital heart disease
    Elizabeth A Stephenson
    Department of Cardiology, Children s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Am J Cardiol 92:871-6. 2003
    ..Atrial tachycardias in congenital heart disease are amenable to ATP algorithms in the AT500 pacemaker...
  59. ncbi Prospective trial of electroanatomically guided, irrigated catheter ablation of atrial tachycardia in patients with congenital heart disease
    John K Triedman
    Children s Hospital Boston, Massachusetts 02115, USA
    Heart Rhythm 2:700-5. 2005
    ..Retrospective studies suggest these rates might be increased by the use of irrigated radiofrequency (RF) ablation...
  60. ncbi Molecular biological and genetic approaches to the evaluation of inherited electrophysiologic disorders
    Charles I Berul
    Department of Cardiology, Children s Hospital, Harvard Medical School, Boston, Massachusetts 02215, USA
    Drugs Today (Barc) 38:351-64. 2002
    ..Recent discoveries of genetic etiologies have shed new light on the mechanisms underlying arrhythmogenesis and molecular genetics of cardiac electrophysiology...
  61. ncbi Advances in pediatric electrophysiology
    Laura M Bevilacqua
    Department of Cardiology, Children s Hospital, Boston, Massachusetts 02115, USA
    Curr Opin Pediatr 16:494-9. 2004
    ..This paper reviews selected studies germane to physicians and health care personnel who treat pediatric and adult congenital heart patients with arrhythmias and electrophysiologic disorders...
  62. pmc Atrial remodeling after the Fontan operation
    Cordula M Wolf
    Department of Pediatrics, Harvard Medical School, Boston, MA, USA
    Am J Cardiol 104:1737-42. 2009
    ..The degree of wall thickening observed in the Fontan atria was not so excessive as to preclude transmural lesions during catheter or surgical ablation of reentrant arrhythmias...
  63. doi Outcomes of heart transplantation using donor hearts from infants with sudden infant death syndrome
    Jennifer N A Silva
    Departments of Cardiology, Children s Hospital Boston, 300 Longwood Ave, Boston, MA 02115, USA
    J Heart Lung Transplant 29:1226-30. 2010
    ..We analyzed post-transplant outcomes in infants who received a heart from a donor where SIDS was the primary cause of brain death...
  64. doi Electrical dyssynchrony and endocardial fibroelastosis resection in the rehabilitation of hypoplastic left cardiac syndrome
    Richard J Czosek
    Department of Cardiology and Cardiac Surgery, Children s Hospital Boston, Harvard Medical School, Massachusetts, United States of America
    Cardiol Young 20:516-21. 2010
    ..We hypothesised that endocardial fibroelastosis removal may significantly affect ventricular conduction and myocardial electrical characteristics...
  65. ncbi Developmentally modulated cardiac conduction failure in transgenic mice with fetal or postnatal overexpression of DNA nonbinding mutant Nkx2.5
    Hiroko Wakimoto
    Department of Cardiology, Children s Hospital, Boston, Massachusetts 02115, USA
    J Cardiovasc Electrophysiol 13:682-8. 2002
    ....
  66. ncbi Influence of patient factors and ablative technologies on outcomes of radiofrequency ablation of intra-atrial re-entrant tachycardia in patients with congenital heart disease
    John K Triedman
    Department of Cardiology, Boston Children s Hospital, 300 Longwood Avenue, Boston, MA 02115, USA
    J Am Coll Cardiol 39:1827-35. 2002
    ..The goal of this study was to identify factors associated with radiofrequency catheter ablation (RFCA) outcomes of intra-atrial re-entrant tachycardia (IART)...
  67. ncbi A multicenter experience with novel implantable cardioverter defibrillator configurations in the pediatric and congenital heart disease population
    Elizabeth A Stephenson
    Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA
    J Cardiovasc Electrophysiol 17:41-6. 2006
    ..Epicardial patches require a thoracotomy, and may lead to a restrictive pericardial process. Because of these issues, we have explored novel ICD configurations...
  68. ncbi Transgenic mice overexpressing mutant PRKAG2 define the cause of Wolff-Parkinson-White syndrome in glycogen storage cardiomyopathy
    Michael Arad
    Department of Genetics, Harvard Medical School and Howard Hughes Medical Institute, 200 Longwood Ave, Boston, Mass 02115, USA
    Circulation 107:2850-6. 2003
    ..Pathological examinations of affected human hearts reveal vacuoles containing amylopectin, a glycogen-related substance...
  69. ncbi Implantable cardioverter defibrillator lead complications and laser extraction in children and young adults with congenital heart disease: implications for implantation and management
    Joshua M Cooper
    Cardiovascular Division, Brigham and Women s Hospital, Boston, Massachusetts 02115, USA
    J Cardiovasc Electrophysiol 14:344-9. 2003
    ..Over time, ICD leads adhere to venous endothelium and endocardium. Lead removal, when necessary, often requires disruption of this fibrous tissue...
  70. ncbi Impaired parasympathetic heart rate control in mice with a reduction of functional G protein betagamma-subunits
    Josef Gehrmann
    Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115, USA
    Am J Physiol Heart Circ Physiol 282:H445-56. 2002
    ..Our data demonstrate in vivo that Gbetagamma plays a crucial role for parasympathetic heart rate control, sinus node automaticity, and atrial arrhythmia vulnerability...
  71. ncbi Psychosocial factors and quality of life in children and adolescents with implantable cardioverter-defibrillators
    David Ray DeMaso
    Department of Psychiatry, Children s Hospital Boston, Boston, Massachusetts 02115, USA
    Am J Cardiol 93:582-7. 2004
    ..Their quality of life was more strongly correlated with their feelings of anxiety and depression as well as their family functioning than to the severity of their cardiac illness...
  72. ncbi Prenatal features of Costello syndrome: ultrasonographic findings and atrial tachycardia
    Angela E Lin
    Genetics Unit, Department of Pediatrics, MassGeneral Hospital for Children, 185 Cambridge Street, Simches 2222, Boston, MA, USA
    Prenat Diagn 29:682-90. 2009
    ..Delineate prenatal features of Costello syndrome (caused by HRAS mutations), which consists of mental retardation, facial, cardiovascular, skin, and musculoskeletal anomalies, and tumor predisposition...
  73. ncbi Inherited conduction system abnormalities--one group of diseases, many genes
    Cordula M Wolf
    Department of Cardiology, Children s Hospital, Boston Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA
    J Cardiovasc Electrophysiol 17:446-55. 2006
    ..Understanding of the molecular and ionic mechanisms underlying cardiac conduction is essential for the appreciation of the pathogenesis of conduction abnormalities in structurally normal and altered hearts...
  74. doi Rhythm management in pediatric heart failure
    Charles I Berul
    Children s Hospital, Harvard University, Boston, MA, USA
    Congenit Heart Dis 1:140-7. 2006
    ..These therapies and the available supporting data relevant to pediatrics will be the focus of this review...
  75. doi Heart Rhythm Society/Pediatric and Congenital Electrophysiology Society Clinical Competency Statement: training pathways for implantation of cardioverter-defibrillators and cardiac resynchronization therapy devices in pediatric and congenital heart patien
    J Philip Saul
    Medical University of South Carolina, Charleston, South Carolina, USA
    Heart Rhythm 5:926-33. 2008
  76. ncbi Electrophysiologic characterization and postnatal development of ventricular pre-excitation in a mouse model of cardiac hypertrophy and Wolff-Parkinson-White syndrome
    Vickas V Patel
    Molecular Cardiology Research Center and Section of Cardiac Electrophysiology, University of Pennsylvania, Philadelphia, PA, USA
    J Am Coll Cardiol 42:942-51. 2003
    ..We sought to characterize an animal model of the Wolff-Parkinson-White (WPW) syndrome to help elucidate the mechanisms of accessory pathway formation...
  77. ncbi Electrophysiological interventions for treatment of congestive heart failure in pediatrics and congenital heart disease
    Anne M Dubin
    Stanford University, 750 Welch Rd, Suite 305, Palo Alto, CA 94304, USA
    Expert Rev Cardiovasc Ther 5:111-8. 2007
    ..This review will address issues of rhythm management and resynchronization therapy in pediatric and congenital heart disease patients with heart failure...
  78. ncbi Electrophysiological interventions for inherited arrhythmia syndromes
    Elizabeth A Stephenson
    Hospital for Sick Children, Toronto, Ontario, Canada
    Circulation 116:1062-80. 2007
  79. ncbi Ablation of nonautomatic focal atrial tachycardia in children and adults with congenital heart disease
    Stephen P Seslar
    Electrophysiology and Pacing, Children s Specialists of San Diego, Division of Cardiology, Children s Hospital San Diego, MC 5004, San Diego, CA 92123, USA
    J Cardiovasc Electrophysiol 17:359-65. 2006
    ..Nonautomatic focal atrial tachycardia (NAFAT) has been characterized in adults with structurally normal hearts. This article characterizes NAFAT in a population of patients with complex congenital heart disease...
  80. ncbi Late recovery of surgically-induced atrioventricular block in patients with congenital heart disease
    Elchanan Bruckheimer
    Section of Pediatric Cardiology, Yale University School of Medicine, New Haven, CT, USA
    J Interv Card Electrophysiol 6:191-5. 2002
    ..To assess the incidence and establish possible predictors of late recovery of post-surgical heart block, treated with pacemaker implantation, in patients with congenital heart defects...
  81. ncbi The Jervell and Lange-Nielsen syndrome: natural history, molecular basis, and clinical outcome
    Peter J Schwartz
    Department of Cardiology, University of Pavia, IRCCS Policlinico S Matteo, Pavia, Italy
    Circulation 113:783-90. 2006
    ....
  82. ncbi A beat is born: embryonic development of arrhythmogenesis
    Charles I Berul
    J Cardiovasc Electrophysiol 17:1360-1. 2006
  83. ncbi The use of implantable cardioverter-defibrillators in pediatric patients awaiting heart transplantation
    Anne M Dubin
    Division of Pediatric Cardiology, Stanford University, 750 Welch Road, Suite 305, Palo Alto, CA 94303, USA
    J Card Fail 9:375-9. 2003
    ..This multicenter study evaluated experience with implantable cardioverter defibrillators (ICD) as a bridge to orthotopic heart transplantation (OHT) in children...
  84. doi Supraventricular tachycardia in an adolescent with attention-deficit/hyperactivity disorder (ADHD)
    Paul G Hammerness
    J Am Acad Child Adolesc Psychiatry 47:219-20. 2008
  85. ncbi Modification to the Fontan procedure for the prophylaxis of intra-atrial reentrant tachycardia: short-term results of a prospective randomized blinded trial
    Kathryn K Collins
    University of California San Francisco, San Francisco, CA, USA
    J Thorac Cardiovasc Surg 127:721-9. 2004
    ..We evaluated the feasibility, safety, and short-term efficacy of an interventional atrial incision placed at the time of the Fontan operation to reduce the development of intra-atrial reentrant tachycardia...
  86. ncbi Atrial tachyarrhythmias and permanent pacing after pediatric heart transplantation
    Kathryn K Collins
    Pediatric Arrhythmia Center, University of California San Francisco, San Francisco, CA, USA
    J Heart Lung Transplant 22:1126-33. 2003
    ..Limited information is available on arrhythmias in pediatric transplant patients. Our objective was to determine the prevalence and significance of atrial tachyarrhythmias and permanent pacing following pediatric heart transplantation...