Is. Kathiriya et D. Srivastava, Left-right asymmetry and cardiac looping: Implications for cardiac development and congenital heart disease, AM J MED G, 97(4), 2000, pp. 271-279
Proper morphogenesis and positioning of internal organs requires delivery a
nd interpretation of precise signals along the anterior-posterior, dorsal-v
entral, and left-right axes. An elegant signaling cascade determines left-
versus right-sided identity in visceral organs in a concordant fashion, res
ulting in a predictable left-right (LR) organ asymmetry in all vertebrates.
The complex morphogenesis of the heart and its connections to the vasculat
ure are particularly dependent upon coordinated LR signaling pathways. Diso
rganization of LR signals can result in myriad congenital heart defects tha
t are a consequence of abnormal looping and remodeling of the primitive hea
rt tube into a multi-chambered organ. A framework for understanding how LR
asymmetric signals contribute to normal organogenesis has emerged and begin
s to explain the basis of many human diseases of LR asymmetry. Here we revi
ew the impact of LR signaling pathways on cardiac development and congenita
l heart disease. Am. J. Med. Genet. (Semin. Med. Genet.) 97:271-279, 2000.
(C) 2001 Wiley-Liss, Inc.