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Topsy-Turvy Heart with Aortopulmonary Window and Severe Airway Malacia: Prenatal Diagnosis and Review of the Literature

Authors
  • Hejazi, Yahia1
  • Mann, Gurdeep1
  • Boudjemline, Younes1
  • Udassi, Jai P.1, 1
  • Van Leeuwen, Grace1, 1
  • Diab, Karim A.1
  • 1 Sidra Medicine,
Type
Published Article
Journal
Pediatric Cardiology
Publisher
Springer-Verlag
Publication Date
Aug 27, 2021
Pages
1–8
Identifiers
DOI: 10.1007/s00246-021-02710-1
PMID: 34448896
PMCID: PMC8391013
Source
PubMed Central
Keywords
Disciplines
  • Case Report
License
Unknown

Abstract

The topsy-turvy heart is a very rare cardiac malformation that involves a global 90° clockwise rotation of the heart along its long axis. This rotation results in the displacement of the great arteries and severe elongation and stretching of the brachiocephalic arteries and the bronchi. We present an unusual case of topsy-turvy heart diagnosed prenatally with a large aorto-pulmonary window and. This case gives an insight into the morphological details and clinical presentation of this rare malformation and its associated complications. We also present a review of the literature of this rare anomaly showing only 15 live cases that have been published with only three cases diagnosed prenatally.

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