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Ann Pediatr Cardiol. 2015 Jan-Apr;8(1):56-8. doi: 10.4103/0974-2069.149523.

Uncommon variants of the scimitar syndrome in two siblings.

Author information

1
Division of Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom.
2
Division of Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom ; Reader in Paediatric Cardiology at Imperial College, London, United Kingdom.

Abstract

The Scimitar syndrome is a complex association of cardiovascular and bronchopulmonary abnormalities, with the main feature a partial or total anomalous right pulmonary venous drainage to the inferior vena cava. A number of cases that lack of all the features of the typical syndrome have been described as Scimitar variant, but the incidence is rare. Familial occurrence is exceptional and limited to few cases in literature. We report two sibling diagnosed with an uncommon variant of the Scimitar syndrome.

KEYWORDS:

Anomalous pulmonary venous drainage; Scimitar; Scimitar syndrome; Scimitar variant; familial Scimitar syndrome

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