Scimitar Syndrome

Ulf Gudjonsson, John W. Brown

Research output: Contribution to journalArticle

77 Scopus citations

Abstract

Scimitar syndrome is a rare congenital anomaly consisting in part of right pulmonary venous return to the inferior vena cava. There is a clear bimodal presentation of this syndrome with either an infantile manifestation or a pediatric/adult form. The infantile variant is marked by a higher incidence and severity of associated defects, heart failure, pulmonary hypertension, and significant mortality. The patient with the pediatric/adult form is less severely affected and may be asymptomatic on diagnosis. In this article, we review the historical aspects, presentation, and pathophysiology of Scimitar syndrome and discuss available treatment strategies. We emphasize the safe and effective approach developed at Indiana University that obviates both the need for an intra-atrial baffle or use of cardiopulmonary bypass. The results with our alternative approach to Scimitar syndrome are summarized and they compare favorably with other published reports.

Original languageEnglish (US)
Pages (from-to)56-62
Number of pages7
JournalPediatric Cardiac Surgery Annual
Volume9
Issue number1
DOIs
StatePublished - Apr 27 2006

Keywords

  • intra-atrial baffle
  • Scimitar syndrome

ASJC Scopus subject areas

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

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