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J. Cardiovasc. Dev. Dis. 2018, 5(2), 24; https://doi.org/10.3390/jcdd5020024

Some Isolated Cardiac Malformations Can Be Related to Laterality Defects

1
Department of Pediatrics, Sapienza University of Rome, 00161 Rome, Italy
2
Genetics and Rare Diseases Research Division, Bambino Gesù Children’s Hospital and Research Institute, 00165 Rome, Italy
3
Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital and Research Institute, 00165 Rome, Italy
*
Author to whom correspondence should be addressed.
Received: 22 March 2018 / Revised: 21 April 2018 / Accepted: 25 April 2018 / Published: 2 May 2018
(This article belongs to the Special Issue Left–Right Asymmetry and Cardiac Morphogenesis)
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Abstract

Human beings are characterized by a left–right asymmetric arrangement of their internal organs, and the heart is the first organ to break symmetry in the developing embryo. Aberrations in normal left–right axis determination during embryogenesis lead to a wide spectrum of abnormal internal laterality phenotypes, including situs inversus and heterotaxy. In more than 90% of instances, the latter condition is accompanied by complex and severe cardiovascular malformations. Atrioventricular canal defect and transposition of the great arteries—which are particularly frequent in the setting of heterotaxy—are commonly found in situs solitus with or without genetic syndromes. Here, we review current data on morphogenesis of the heart in human beings and animal models, familial recurrence, and upstream genetic pathways of left–right determination in order to highlight how some isolated congenital heart diseases, very common in heterotaxy, even in the setting of situs solitus, may actually be considered in the pathogenetic field of laterality defects. View Full-Text
Keywords: congenital heart disease; genetics; heterotaxy; atrioventricular canal defect; transposition of the great arteries congenital heart disease; genetics; heterotaxy; atrioventricular canal defect; transposition of the great arteries
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).
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Versacci, P.; Pugnaloni, F.; Digilio, M.C.; Putotto, C.; Unolt, M.; Calcagni, G.; Baban, A.; Marino, B. Some Isolated Cardiac Malformations Can Be Related to Laterality Defects. J. Cardiovasc. Dev. Dis. 2018, 5, 24.

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