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Malalignment of the Septum Primum with Abnormally Positioned Left Atrial Appendage
Authors:Gregory H. Kurio  Rajiv Gulati  David P. Bichell  James C. Perry
Affiliation:(1) Division of Pediatric Cardiology, Children’s Hospital Los Angeles, 4650 Sunset Boulevard, No. 34, Los Angeles, CA 90027, USA;(2) Division of Cardiothoracic Surgery, Children’s Hospital & Health Center, San Diego 3020 Children’s Way, San Diego, CA 92123, USA;(3) Pediatric and Congenital Cardiac Surgery, University of Chicago, Comer Children’s Hospital, 5841 S. Maryland Ave., UC-5040, Chicago, IL, 60637;(4) Section of Pediatric Cardiology, Yale University Medical Center, 333 Cedar St., P.O. Box 208064, New Haven, CT 06520-8014, USA
Abstract:
This case discusses a Shone’s variant with small left ventricle and mitral valve, bicuspid aortic valve, coarctation of the aorta, an unusual arrangement of atrial appendages, and partially anomalous pulmonary venous drainage due to a deviated atrial septum. The left atrial appendage could be directly entered from the right atrium at catheterization, but it was positioned behind the great arteries. The right atrial appendage was normal in origin and orientation. The implications of this anatomy in fetal cardiac development are reviewed.
Keywords:Appendage  Juxtaposed  Shone’  s  Embryology  Septum primum
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