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Hot Topics In Tetralogy Of Fallot.
MedLine Citation:
PMID:  24076489     Owner:  NLM     Status:  Publisher    
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore "Hot Topics" to highlight areas of emerging science for clinicians and scientists in moving towards a better understanding of long-term management in patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac Magnetic Resonance (CMR) is the gold-standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta and AP collaterals, and its ability to quantify biventricular size and function, pulmonary regurgitation (PR) and myocardial viability. Atrial reentrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2 % per year. Risk stratification even with electrophysiologic (EP) testing and CMR remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite high complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, pulmonary regurgitation (PR) may be treated by transcatheter valve insertion. Ongoing surveillance of RV function is a critical component of clinical assessment. Once RV dysfunction occurs, there are no medical therapies that have been shown to be effective, except for resynchronization with biventricular pacing. For patients with significant PR with RV dilatation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.
Juan Villafañe; Jeffrey A Feinstein; Kathy J Jenkins; Robert N Vincent; Edward P Walsh; Anne M Dubin; Tal Geva; Jeffrey A Towbin; Meryl S Cohen; Charles Fraser; Joseph Dearani; David Rosenthal; Beth Kaufman; Thomas P Graham
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-9-26
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  -     ISSN:  1558-3597     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  2013 Sep 
Date Detail:
Created Date:  2013-9-30     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
Department of Pediatrics (Cardiology), University of Kentucky, Lexington, KY. Electronic address:
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