This review examines and discusses the morphology and embryology of two main groups of conotruncal cardiac malformations: (a) transposition of the great arteries (complete transposition and incomplete/partial transposition namely double outlet right ventricle), and (b) aortic dextroposition defects (tetralogy of Fallot and Eisenmenger malformation). In both groups, persistent truncus arteriosus was included because maldevelopment of the neural crest cell supply to the outflow tract, contributing to the production of the persistent truncus arteriosus, is shared by both groups of malformations. The potentially important role of the proximal conal cushions in the rotatory sequence of the conotruncus is emphasized. Most importantly, this study emphasizes the differentiation between the double-outlet right ventricle, which is a partial or incomplete transposition of the great arteries, and the Eisenmenger malformation, which is an aortic dextroposition. Special emphasis is also given to the leftward shift of the conoventricular junction, which covers an important morphogenetic role in both aortic dextropositions and transposition defects as well as in normal development, and whose molecular genetic regulation seems to remain unclear at present. Emphasis is placed on the distinct and overlapping roles of Tbx1 and Pitx2 transcription factors in modulating the development of the cardiac outflow tract.

Transpositions of the great arteries versus aortic dextropositions. A review of some embryogenetic and morphological aspects / Restivo, Angelo; DI GIOIA, Cira Rosaria Tiziana; MARINO TAUSSIG DE BODONIA, Bruno; Putotto, Carolina. - In: THE ANATOMICAL RECORD. - ISSN 1932-8486. - 3:306(2023), pp. 502-514. [10.1002/ar.25129]

Transpositions of the great arteries versus aortic dextropositions. A review of some embryogenetic and morphological aspects

Cira di Gioia
Secondo
;
Bruno Marino
Penultimo
;
Carolina Putotto
Ultimo
2023

Abstract

This review examines and discusses the morphology and embryology of two main groups of conotruncal cardiac malformations: (a) transposition of the great arteries (complete transposition and incomplete/partial transposition namely double outlet right ventricle), and (b) aortic dextroposition defects (tetralogy of Fallot and Eisenmenger malformation). In both groups, persistent truncus arteriosus was included because maldevelopment of the neural crest cell supply to the outflow tract, contributing to the production of the persistent truncus arteriosus, is shared by both groups of malformations. The potentially important role of the proximal conal cushions in the rotatory sequence of the conotruncus is emphasized. Most importantly, this study emphasizes the differentiation between the double-outlet right ventricle, which is a partial or incomplete transposition of the great arteries, and the Eisenmenger malformation, which is an aortic dextroposition. Special emphasis is also given to the leftward shift of the conoventricular junction, which covers an important morphogenetic role in both aortic dextropositions and transposition defects as well as in normal development, and whose molecular genetic regulation seems to remain unclear at present. Emphasis is placed on the distinct and overlapping roles of Tbx1 and Pitx2 transcription factors in modulating the development of the cardiac outflow tract.
2023
aortic dextroposition; conotruncal rotation; conotruncus; conoventricular junction; double outlet right ventricle
01 Pubblicazione su rivista::01g Articolo di rassegna (Review)
Transpositions of the great arteries versus aortic dextropositions. A review of some embryogenetic and morphological aspects / Restivo, Angelo; DI GIOIA, Cira Rosaria Tiziana; MARINO TAUSSIG DE BODONIA, Bruno; Putotto, Carolina. - In: THE ANATOMICAL RECORD. - ISSN 1932-8486. - 3:306(2023), pp. 502-514. [10.1002/ar.25129]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1677571
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