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(Circulation Research. 2002;91:87.)
© 2002 American Heart Association, Inc.

Editorials

Embryogenesis of Transposition of the Great Arteries

A Lesson From the Heart

Margaret L. Kirby

From the Neonatal-Perinatal Research Institute, Division of Neonatology, Department of Pediatrics, Duke University Medical Center, Durham, NC.

Correspondence to Margaret L. Kirby, PhD, Box 3179, Neonatology, 307B Nanaline Duke Bldg, Research Drive, Dept of Pediatrics, DUMC, Durham, NC 20012. E-mail mlkirby@duke.edu

Key Words: perlecan • transposition of the great arteries • heart • outflow tract

An extract of the first 250 words of the full text is provided, because this article has no abstract.

Defective development of the cardiac outflow tract results in congenital cardiac anomalies that are known clinically as conotruncal defects. Development of the outflow tract is complicated, and the large number of conotruncal malformations that are seen in children reflects this complexity. Historically, knowledge of the embryogenesis of any heart defects has been difficult to attain because of the lack of models in which the pathogenesis of the defect could be studied prospectively. It is not difficult to induce cardiac malformations experimentally in animals but such malformations are not consistently of the same type, even using the same experimental paradigm. It is difficult to study a particular outflow malformation in a model with only 20% or even 50% incidence of the malformation. There is another problem with outflow development that further confounds our understanding: many but not all of the outflow malformations are related to each other in that they represent more or less severe manifestations of the same developmental pathology.¹

The retinoic acid model was one of the earliest teratogenic models of heart defects in the 1940s and 50s.² A strain of keeshound dog developed by Patterson became the first genetic model to be studied extensively.³ Both of these models provided information about the morphology of some defects at various stages of development, but these models said little about the pathogenesis of the defects because of the problems mentioned above, ie, variability and low penetrance of any particular phenotype.

The first and (possibly) most informative model of heart defects was . . . [Full Text of this Article]

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