Journal ArticleAnn Thorac Surg · April 2014
BACKGROUND: This case series describes 3 patients with the unusual location of focal atrial tachycardia in the left atrial appendage who failed catheter ablation but were successfully treated by left atrial appendage resection by a totally thoracoscopic su ...
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Journal ArticleJ Am Soc Echocardiogr · April 2014
BACKGROUND: Although quantitative right ventricular (RV) strain analysis may be useful in congenital and acquired heart disease populations with RV failure, a comprehensive, standardized approach is lacking. An 18-segment RV strain analysis obtained from t ...
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Journal ArticleCongenit Heart Dis · 2014
The relationship between accessory pathway-mediated ventricular preexcitation and left ventricular dyssynchrony-induced dysfunction has been described in patients with Wolff-Parkinson-White (WPW) syndrome in the absence of sustained supraventricular tachyc ...
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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 ArticleCongenital Heart Disease · 2013
Multicenter clinical registries are increasingly recognized as valuable tools for establishing benchmarks, facilitating patient-centered quality improvement and research. In 2010, the Pediatric and Congenital Electrophysiology Society convened a taskforce ...
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Journal ArticleCirc Arrhythm Electrophysiol · October 2012
BACKGROUND: Supraventricular tachycardia (SVT) is one of the most common conditions requiring emergent cardiac care in children, yet its management has never been subjected to a randomized controlled clinical trial. The purpose of this study was to compare ...
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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 ArticleCan J Physiol Pharmacol · October 2011
BACKGROUND: Mutations in the SCN5A gene have been linked to Brugada syndrome (BrS), conduction disease, Long QT syndrome (LQT3), atrial fibrillation (AF), and to pre- and neonatal ventricular arrhythmias. OBJECTIVE: The objective of this study is to charac ...
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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 ArticleAm J Cardiol · April 1, 2011
Psychosocial and quality-of-life (QOL) outcomes in adult patients with implantable cardioverter-defibrillators (ICDs) are well studied. Minimal research exists regarding pediatric adjustment, despite a potentially more challenging adjustment process. The p ...
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Journal ArticleAm Heart J · January 2011
BACKGROUND: Mortality between stage I and II palliation for hypoplastic left heart syndrome (HLHS) has been associated with arrhythmias. The stage-related proportion, associations, and clinical impact of arrhythmias in patients with HLHS have not been eval ...
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Journal ArticlePediatric Cardiology · 2011
Supraventricular tachycardia (SVT) presenting in the neonatal period may resolve by 1 year of age. Predicting which patients require therapy beyond 1 year of age is desirable. Pediatric electrophysiology databases from two institutions were reviewed for pa ...
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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 ArticleCirc Cardiovasc Genet · April 2010
BACKGROUND: Danon disease is an X-linked dominant disorder characterized by the clinical triad of hypertrophic cardiomyopathy, skeletal myopathy, and variable mental retardation. Pathologically, autophagic vacuoles are noted in both skeletal and cardiac mu ...
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Journal ArticleJ Cardiovasc Electrophysiol · February 2010
Tachyarrhythmias occur in patients having congenital heart disease most commonly related to surgically created or naturally occurring conduction obstacles, and to postoperative hemodynamic effects on cardiac muscle. Less frequently, the underlying disease ...
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Journal ArticleCongenit Heart Dis · 2010
Aortic valve atresia with interruption of the aortic arch is an extremely rare anomaly; only eleven cases of this anomaly have been reported to date. In the absence of additional sources of blood flow to the ascending aorta, aortic valve atresia with inter ...
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Journal ArticleHeart Rhythm · 2010
Background L-type calcium channel (LTCC) mutations have been associated with Brugada syndrome (BrS), short QT (SQT) syndrome, and Timothy syndrome (LQT8). Little is known about the extent to which LTCC mutations contribute to the J-wave syndromes associate ...
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Journal ArticleJ Cardiovasc Electrophysiol · February 2009
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 · October 2008
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 ...
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Journal ArticleJ Thorac Cardiovasc Surg · July 2008
OBJECTIVES: Our objective was to determine the relationship between functional outcome and abnormalities of heart rate and rhythm after the Fontan operation. METHODS: The National Heart, Lung, and Blood Institute Pediatric Heart Network conducted a cross-s ...
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Journal ArticlePacing Clin Electrophysiol · November 2007
A 16-year-old boy with Ebstein's anomaly of the tricuspid valve developed exercise-induced rapid ventricular tachycardia. He had previously undergone tricuspid valvuloplasty and bidirectional cavopulmonary anastamosis due to progressive symptoms. A single- ...
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Journal ArticlePacing Clin Electrophysiol · October 2007
BACKGROUND: Transvenous pacemaker leads in children are often placed with redundant lead length to allow for anticipated patient growth. This excess lead may rarely prolapse into the pulmonary artery and potentially interfere with valve function. We sought ...
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Journal ArticleJournal for Vascular Ultrasound · January 1, 2007
Childhood syncope is common, and there are three categories of causes of syncope: noncardiovascular pseudosyncope, cardiovascular syncope, and neurally mediated syncope. Tilt table testing has become a valuable tool in the evaluation for the cause of synco ...
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Journal ArticleJ Pediatr · November 2006
Patients with neurofibromatosis type 1 (NF1) can manifest a characteristic vasculopathy that in adults is rarely associated with fatal coronary artery occlusion. We describe the clinical and pathological findings from 2 unrelated young children with NF1, a ...
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Journal ArticleInt J Eat Disord · November 2006
OBJECTIVE: This is the first case report of a second-degree heart block (Mobitz Type I) described in a patient with anorexia nervosa (AN). METHOD: We present the case of a 20-year-old woman with AN and second-degree heart block. Pertinent reports in the li ...
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Journal ArticleBr J Anaesth · August 2006
Postural orthostatic tachycardia syndrome encompasses a group of disorders characterized by orthostatic intolerance. We describe the anaesthetic management of analgesia for labour and of Caesarean section in a parturient suffering from this disorder. Worse ...
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Journal ArticleGenet Med · May 2006
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 ...
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Journal ArticleGenet Med · May 2006
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 ArticlePediatr Cardiol · 2006
Endomyocardial biopsy is the gold standard survey for cardiac graft rejection. Signal-averaged electrocardiography (SAECG) identifies slowly conducting, diseased myocardium. We sought to determine whether SAECG is a sensitive, noninvasive transplant survei ...
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Journal ArticleJ Am Coll Cardiol · December 20, 2005
OBJECTIVES: Our objective was to evaluate the short-term safety and efficacy of cardiac resynchronization therapy (CRT) in children. BACKGROUND: Cardiac resynchronization therapy has been beneficial for adult patients with poor left ventricular function an ...
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Journal ArticleN Engl J Med · January 27, 2005
BACKGROUND: Unexplained left ventricular hypertrophy often prompts the diagnosis of hypertrophic cardiomyopathy, a sarcomere-protein gene disorder. Because mutations in the gene for AMP-activated protein kinase gamma2 (PRKAG2) cause an accumulation of card ...
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Journal ArticleJournal of Interventional Cardiac Electrophysiology · 2005
An 11-year old female presented with paroxysmal tachycardia and was diagnosed with a Mahaim fiber during electrophysiologic study. A preexcited tachycardia and the typical variety of AV nodal reentry tachycardia were induced at different times. During pree ...
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Journal ArticleJ Am Coll Cardiol · October 19, 2004
Heart Rhythm 2004, the 25th Annual Scientific Sessions of the Heart Rhythm Society (formerly the North American Society of Pacing and Electrophysiology), met in San Francisco in May 2004. The meeting is the world's premier forum for the presentation of res ...
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Journal ArticleJ Cardiovasc Electrophysiol · July 2004
INTRODUCTION: A multicenter prospective study was designed and implemented to assess the short- and longer-term results and risks associated with radiofrequency (RF) ablation in children. METHODS AND RESULTS: Patients recruited for the study were aged 0 to ...
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Journal ArticleHeart Rhythm · July 2004
OBJECTIVES: A multicenter prospective study was performed to assess the results and risks associated with radiofrequency ablation in children. This report focuses on recurrences following initially successful ablation. METHODS: Patients recruited for the s ...
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Journal ArticleClin 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 ...
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Journal ArticleAnesth Analg · December 2002
UNLABELLED: In children, radiofrequency catheter ablation (RFCA) is typically performed under general anesthesia. With the use of volatile anesthetics, postoperative nausea and vomiting (PONV) are common, with an incidence of emesis as frequent as 60%. We ...
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Journal ArticleAnesthesiology · June 2002
BACKGROUND: Radiofrequency catheter ablation (RFCA), which is typically performed with general anesthesia in children, is an interventional therapy for tachyarrhythmia. Although the electrophysiologic (EP) effects of isoflurane- and propofol-based anesthet ...
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Journal ArticleJ Cardiovasc Electrophysiol · June 2002
INTRODUCTION: One of the characteristics of the Mahaim fiber is that it possesses a decremental property related to the slow rate of recovery of its excitability similar to that of AV node. The aim of this study was to evaluate the recovery property of the ...
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Journal ArticleJ Cardiovasc Electrophysiol · March 2002
INTRODUCTION: Prior studies in adults have shown significant shortening of the fast pathway effective refractory period after successful slow pathway ablation. As differences between adults and children exist in other characteristics of AV nodal reentrant ...
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Journal ArticleJournal of Clinical Investigation · January 1, 2002
Mutations in PRKAG2, the gene for the γ2 regulatory subunit of AMP-activated protein kinase, cause cardiac hypertrophy and electrophysiologic abnormalities, particularly preexcitation (Wolff-Parkinson-White syndrome) and atrioventricular conduction block. ...
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Journal ArticleJ Am Coll Cardiol · February 2000
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 ArticleJ Cardiovasc Electrophysiol · May 1999
An infant presented with neonatal syncope and seizures. An ECG showed a preexcitation pattern, most compatible with Wolff-Parkinson-White (WPW) syndrome. Rhythm monitoring during an event demonstrated prolonged periods of complete AV block with no ventricu ...
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Journal ArticleCritical Care Medicine · January 1, 1999
Introduction: Cisapride therapy has been associated with life threatening arrhythmias (Torsades de pointes) and prolongation of the QTc interval in adults and children. Cisapride exhibits a dose dependent class III anti-arrhythmic effect. Infants have a de ...
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Journal ArticleCardiol Young · July 1998
A patient with repaired tetralogy of Fallot presented with recurrent syncope and had multiple haemodynamically unstable ventricular tachycardias unresponsive to antiarrhythmic medications. Ventricular tachycardias became haemodynamically tolerated with ami ...
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Journal ArticleJ Cardiovasc Electrophysiol · November 1997
In the next decade, "better" management will be defined by cost effectiveness including morbidity, mortality, and cost. We used a cost-effectiveness model for children with Wolff-Parkinson-White syndrome (WPW) and supraventricular tachycardia (SVT) compari ...
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Journal ArticleAnn 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 ...
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Journal ArticleJ Cardiovasc Electrophysiol · February 1997
The cardiac conduction system from infants suddenly dying with hypertrophic cardiomyopathy has not been described. We studied by serial section examination the conduction system from a 13-month old infant also known to have had supraventricular tachycardia ...
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Journal ArticlePacing Clin Electrophysiol · February 1997
After surgery for complex congenital heart disease, clinically important atrial tachyarrhythmias have a higher than normal incidence if sufficiently large regions of conduction block occur within the atria, especially in the presence of hemodynamic alterat ...
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Journal ArticleInt J Cardiol · November 15, 1996
Eight children, age between 4.5 and 19 years were treated with moricizine for supraventricular tachycardia during the last 3 years. The tachycardia was documented by surface electrocardiogram (ECG), and/or by ambulatory ECG in all the children and the mech ...
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Journal ArticlePacing Clin Electrophysiol · May 1996
A 26-year-old man underwent an electrophysiological study for evaluation of a history of congenital heart disease, presyncope, and wide complex tachycardia. During the study the patient developed sustained atrial fibrillation with a rapid ventricular respo ...
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Journal ArticleJ Am Vet Med Assoc · January 1, 1996
Rapid, clinical supraventricular tachycardia (SVT), without apparent underlying heart disease, was identified in 3 young Labrador Retrievers and 1 Labrador Retriever-type dog. The electrocardiographic characteristics, identification of ventricular preexcit ...
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Journal ArticleAnn 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 ...
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Journal ArticleAm J Med Genet · October 23, 1995
Most congenital cutaneous hemangiomas are a sporadic occurrence. Hemangiomas have been found in association with coarctation of the aorta and a right aortic arch. A separate association has been noted of midline ventral defects with hemangiomas. We report ...
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Journal ArticleJ Vet Intern Med · 1995
A 4-month-old male Labrador Retriever was presented for recurrent bouts of pulmonary edema associated with tachycardia. Initial physical examination and echocardiography were unremarkable, and the electrocardiogram revealed only an intraventricular conduct ...
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Journal ArticleJ Cardiovasc Electrophysiol · September 1994
Ventricular bigeminy in children is regarded as a benign arrhythmia in the absence of coexisting heart disease. We present the case of a patient with an atriofascicular fiber that electrocardiographically presented as wide complex bigeminy and wide complex ...
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Journal ArticlePediatr Cardiol · March 1993
A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography ...
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Journal ArticleCurr Opin Cardiol · January 1993
Our understanding of pediatric arrhythmias continues to evolve through natural history studies of paroxysmal supraventricular tachycardia, the congenital long QT syndrome, and postoperative atrial and ventricular tachyarrhythmias. The influence of the auto ...
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Journal ArticleAnn Thorac Surg · October 1992
Our group has previously reported a large prospective experience with the use of intraoperative echocardiography with Doppler color-flow imaging (IE-DCFI) during the repair of congenital heart defects. We have now performed IE-DCFI in 621 patients and have ...
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Journal ArticleAnn Thorac Surg · July 1990
Development of a reversible pulmonary artery band might obviate the need for a second cardiac surgical procedure in children with some forms of congenital heart disease. We evaluated a segmented Silastic-coated Dacron mesh band 2 to 4 mm wide sewn together ...
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Journal ArticleJ Am Coll Cardiol · March 15, 1990
Intravascular ultrasound images were employed to evaluate aortic coarctation before and after balloon angioplasty. Measurements obtained with use of an ultrasound imaging catheter correlated well with measurements made with digital aortography, both in the ...
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