Malalignment of the septum primum with abnormally positioned left atrial appendage: Unusual combination in a Shone's heart

Gregory H. Kurio, Rajiv Gulati, David P. Bichell, James C. Perry

Research output: Contribution to journalReview article


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.

Original languageEnglish (US)
Pages (from-to)628-632
Number of pages5
JournalPediatric Cardiology
Issue number5
StatePublished - Oct 1 2006



  • Appendage
  • Embryology
  • Juxtaposed
  • Septum primum
  • Shone's

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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