Chapitre D'ouvrage Année : 2024

Molecular Pathways and Animal Models of Tetralogy of Fallot and Double Outlet Right Ventricle

Résumé

Tetralogy of Fallot and Double outlet right ventricle are outflow tract (OFT) alignment defects situated on a continuous disease spectrum. A myriad of upstream causes can impact on ventriculoarterial alignment that can be summarized as defects in either i) OFT elongation during looping morphogenesis or ii) OFT remodeling during cardiac septation. Embryological processes underlying these two developmental steps include deployment of second heart field cardiac progenitor cells, establishment and transmission of embryonic left/right information driving OFT rotation and OFT cushion and valve morphogenesis. The formation and remodeling of pulmonary trunk infundibular myocardium is a critical component of both steps.

Defects in myocardial, endocardial or neural crest cell lineages can result in alignment defects, reflecting the complex intercellular signaling events that coordinate arterial pole development.

Importantly, however, OFT alignment is mechanistically distinct from neural crest driven OFT septation, although neural crest cells impact indirectly on alignment through their role in modulating signaling during SHF development. As yet poorly understood non-genetic causes of alignment defects that impact the above processes include hemodynamic changes, maternal exposure to environmental teratogens and stochastic events. The heterogeneity of causes converging on alignment defects characterizes the OFT as a hotspot of congenital heart defects.

Fichier sous embargo
Fichier sous embargo
0 5 0
Année Mois Jours
Avant la publication
mercredi 18 juin 2025
Fichier sous embargo
mercredi 18 juin 2025
Connectez-vous pour demander l'accès au fichier

Dates et versions

hal-04893043 , version 1 (17-01-2025)

Identifiants

Citer

Robert G Kelly. Molecular Pathways and Animal Models of Tetralogy of Fallot and Double Outlet Right Ventricle. Congenital Heart Diseases: The Broken Heart, 1441, Springer International Publishing, pp.645-659, 2024, Advances in Experimental Medicine and Biology, 978-3-031-44086-1. ⟨10.1007/978-3-031-44087-8_37⟩. ⟨hal-04893043⟩

Collections

UNIV-AMU
0 Consultations
0 Téléchargements

Altmetric

Partager

More