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Unusually High Association of Hypertrophic Cardiomyopathy and Complex Heart Defects in Children with Fasciculoventricular Pathways.
MedLine Citation:
PMID:  22242628     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Introduction: Fasciculoventricular pathways (FVPs) are rare causes of preexcitation that do not mediate tachycardias. We report a two-center experience of pediatric patients with FVP and an unexpectedly high association of complex congenital heart defects (CHDs), chromosomal anomalies, and hypertrophic cardiomyopathy. Methods: A retrospective review of the electrophysiology database at two institutions was performed to identify patients with FVP from January 2000 to January 2011. Medical records of these patients were reviewed for clinical history and course, presence of comorbidities, and details of intracardiac electrophysiology (EP) study. Results: A total of 17 patients were identified. The primary indication for EP study was a preexcitation pattern on electrocardiogram. The majority of patients, 12/17 (71%), were found to have associated cardiac and genetic anomalies. Hypertrophic cardiomyopathy was found in 5/17 (29%) patients, with genetic testing in two patients demonstrating the lysosomal-associated membrane protein 2 mutation (Danon syndrome). Underlying complex CHDs were present in 3/17 (18%) patients. One patient (6%) was status post (s/p) cardiac transplant, one patient had hypertension, and another had Trisomy 21. Other electrophysiologic substrates mediating tachycardia were found in 3/17 (18%) patients. Only 5/17 patients (29%) were otherwise healthy with structurally normal hearts. Conclusions: In this largest reported series of FVP in children, there is an unusually high association of FVP with complex CHDs, chromosomal anomalies, and hypertrophic cardiomyopathy. Any patient with such disorders and manifest preexcitation should be evaluated with a high index of suspicion for a FVP. (PACE 2012;1-6).
Authors:
Sudhir Vashist; Jennifer N A Silva; George F VAN Hare; Andrew L Papez; Wilber W Su; Edward K Rhee
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-1-13
Journal Detail:
Title:  Pacing and clinical electrophysiology : PACE     Volume:  -     ISSN:  1540-8159     ISO Abbreviation:  -     Publication Date:  2012 Jan 
Date Detail:
Created Date:  2012-1-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7803944     Medline TA:  Pacing Clin Electrophysiol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
©2012, The Authors. Journal compilation ©2012 Wiley Periodicals, Inc.
Affiliation:
Department of Pediatric Cardiology, Washington University School of Medicine/St. Louis Children's Hospital, St. Louis, Missouri Phoenix Children's Hospital & Arizona Pediatric Cardiology Consultants/Pediatrix, Phoenix, Arizona St. Joseph's Hospital and Medical Center, Phoenix, Arizona Eller Congenital Heart Center, Heart Lung Institute, St. Joseph's Hospital and Medical Center, Phoenix, Arizona.
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