Journal
BIRTH DEFECTS RESEARCH PART A-CLINICAL AND MOLECULAR TERATOLOGY
Volume 91, Issue 6, Pages 565-577Publisher
WILEY
DOI: 10.1002/bdra.20826
Keywords
atrioventricular conduction system; atrioventricular canal; preexcitation; lineage analysis; WPW; T-box transcription factors
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Defects originating from the atrioventricular canal region are part of a wide spectrum of congenital cardiovascular malformations that frequently affect newborns. These defects include partial or complete atrioventricular septal defects, atrioventricular valve defects, and arrhythmias, such as atrioventricular re-entry tachycardia, atrioventricular nodal block, and ventricular preexcitation. Insight into the cellular origin of the atrioventricular canal myocardium and the molecular mechanisms that control its development will aid in the understanding of the etiology of the atrioventricular defects. This review discusses current knowledge concerning the origin and fate of the atrioventricular canal myocardium, the molecular mechanisms that determine its specification and differentiation, and its role in the development of certain malformations such as those that underlie ventricular preexcitation. Birth Defects Research (Part A) 91:565-577, 2011. (C) 2011 Wiley-Liss, Inc.
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