Topsy-Turvy Heart: A Very Rare Congenital Rotational Heart Disease With Tracheobronchial Anomalies. (July 2013)

• Record Type: Journal Article
• Authors: Erek, Ersin; Guzeltas, Alper; Ozturk, Nilufer Yalindag; Kiyan, Gursu; Karakoc, Fazilet; Akalin, Figen; Odemis, Ender; Arsan, Sinan
• Is Part Of: World journal for pediatric & congenital heart surgery. Volume 4:Number 3(2013)
• Page Start: 308
• Page End: 311
• Publication Date: 2013-07
• Subjects: congenital heart disease -- aortic arch -- airway -- bronchus -- cardiac anatomy; Pediatric cardiology -- Periodicals; Congenital heart disease in children -- Periodicals; Heart -- Abnormalities -- Surgery -- Periodicals; Heart -- Surgery -- Periodicals; Heart Defects, Congenital -- surgery -- Periodicals; Cardiac Surgical Procedures -- Periodicals; Child -- Periodicals; Adult -- Periodicals; 618.9212
• DOI: 10.1177/2150135113476718
• Languages: English
• ISSNs: 2150-1351

Abstract

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.