Topsy-Turvy Heart: A Very Rare Congenital Rotational Heart Disease With Tracheobronchial Anomalies

The topsy-turvy heart is characterized by a global 90°clockwise rotation around the heart’s long axis. This rotation displaces all basal great arteries inferiorly and posteriorly, resulting in elongation and stretching of the brachiocephalic arteries and the bronchi. To date, reports of only four li...

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Bibliographic Details
Published in:World journal for pediatric & congenital heart surgery 2013-07, Vol.4 (3), p.308-311
Main Authors: Erek, Ersin, Guzeltas, Alper, Ozturk, Nilufer Yalindag, Kiyan, Gursu, Karakoc, Fazilet, Akalin, Figen, Odemis, Ender, Arsan, Sinan
Format: Article
Language:English
Subjects:
Aorta, Thoracic - abnormalities
Bronchi - abnormalities
Consanguinity
Female
Heart Ventricles - abnormalities
Humans
Infant
Male
Tomography, X-Ray Computed
Torsion Abnormality - surgery
Trachea - abnormalities
Transposition of Great Vessels - surgery
Treatment Outcome
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Summary:The topsy-turvy heart is characterized by a global 90°clockwise rotation around the heart’s long axis. This rotation displaces all basal great arteries inferiorly and posteriorly, resulting in elongation and stretching of the brachiocephalic arteries and the bronchi. To date, reports of only four living cases have been published in the literature. We report here three new cases, with additional aortopulmonary window defects, and present their morphological details, clinical presentations, and our management.
ISSN:2150-1351
2150-136X
DOI:10.1177/2150135113476718