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Identification and Morphogenesis of Vestibular Atrial Septal Defects

Background: The vestibular atrial septal defect is an interatrial communication located in the antero-inferior portion of the atrial septum. Reflecting either inadequate muscularization of the vestibular spine and mesenchymal cap during development, or excessive apoptosis within the developing anter...

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Autores principales: Loomba, Rohit S., Tretter, Justin T., Mohun, Timothy J., Anderson, Robert H., Kramer, Scott, Spicer, Diane E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7570000/
https://www.ncbi.nlm.nih.gov/pubmed/32927616
http://dx.doi.org/10.3390/jcdd7030035
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author Loomba, Rohit S.
Tretter, Justin T.
Mohun, Timothy J.
Anderson, Robert H.
Kramer, Scott
Spicer, Diane E.
author_facet Loomba, Rohit S.
Tretter, Justin T.
Mohun, Timothy J.
Anderson, Robert H.
Kramer, Scott
Spicer, Diane E.
author_sort Loomba, Rohit S.
collection PubMed
description Background: The vestibular atrial septal defect is an interatrial communication located in the antero-inferior portion of the atrial septum. Reflecting either inadequate muscularization of the vestibular spine and mesenchymal cap during development, or excessive apoptosis within the developing antero-inferior septal component, the vestibular defect represents an infrequently recognized true deficiency of the atrial septum. We reviewed necropsy specimens from three separate archives to establish the frequency of such vestibular defects and their associated cardiac findings, providing additional analysis from developing mouse hearts to illustrate their potential morphogenesis. Materials and methods: We analyzed the hearts in the Farouk S. Idriss Cardiac Registry at Ann and Robert H. Lurie Children’s Hospital in Chicago, IL, the Van Mierop Archive at the University of Florida in Gainesville, Florida, and the archive at Johns Hopkins All Children’s Heart Institute in St. Petersburg, Florida, identifying all those exhibiting a vestibular atrial septal defect, along with the associated intracardiac malformations. We then assessed potential mechanisms for the existence of such defects, based on the assessment of 450 datasets of developing mouse hearts prepared using the technique of episcopic microscopy. Results: We analyzed a total of 2100 specimens. Of these, 68 (3%) were found to have a vestibular atrial septal defect. Comparable defects were identified in 10 developing mouse embryos sacrificed at embryonic data 15.5, by which stage the antero-inferior component of the atrial septum is usually normally formed. Conclusion: The vestibular defect is a true septal defect located in the muscular antero-inferior rim of the oval fossa. Our retrospective review of autopsied hearts suggests that the defect may be more common than previously thought. Increased awareness of the location of the defect should optimize its future clinical identification. We suggest that the defect exists because of failure, during embryonic development, of union of the components that bind the leading edge of the primary atrial septum to the atrioventricular junctions, either because of inadequate muscularisation or excessive apoptosis.
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spelling pubmed-75700002020-10-29 Identification and Morphogenesis of Vestibular Atrial Septal Defects Loomba, Rohit S. Tretter, Justin T. Mohun, Timothy J. Anderson, Robert H. Kramer, Scott Spicer, Diane E. J Cardiovasc Dev Dis Article Background: The vestibular atrial septal defect is an interatrial communication located in the antero-inferior portion of the atrial septum. Reflecting either inadequate muscularization of the vestibular spine and mesenchymal cap during development, or excessive apoptosis within the developing antero-inferior septal component, the vestibular defect represents an infrequently recognized true deficiency of the atrial septum. We reviewed necropsy specimens from three separate archives to establish the frequency of such vestibular defects and their associated cardiac findings, providing additional analysis from developing mouse hearts to illustrate their potential morphogenesis. Materials and methods: We analyzed the hearts in the Farouk S. Idriss Cardiac Registry at Ann and Robert H. Lurie Children’s Hospital in Chicago, IL, the Van Mierop Archive at the University of Florida in Gainesville, Florida, and the archive at Johns Hopkins All Children’s Heart Institute in St. Petersburg, Florida, identifying all those exhibiting a vestibular atrial septal defect, along with the associated intracardiac malformations. We then assessed potential mechanisms for the existence of such defects, based on the assessment of 450 datasets of developing mouse hearts prepared using the technique of episcopic microscopy. Results: We analyzed a total of 2100 specimens. Of these, 68 (3%) were found to have a vestibular atrial septal defect. Comparable defects were identified in 10 developing mouse embryos sacrificed at embryonic data 15.5, by which stage the antero-inferior component of the atrial septum is usually normally formed. Conclusion: The vestibular defect is a true septal defect located in the muscular antero-inferior rim of the oval fossa. Our retrospective review of autopsied hearts suggests that the defect may be more common than previously thought. Increased awareness of the location of the defect should optimize its future clinical identification. We suggest that the defect exists because of failure, during embryonic development, of union of the components that bind the leading edge of the primary atrial septum to the atrioventricular junctions, either because of inadequate muscularisation or excessive apoptosis. MDPI 2020-09-10 /pmc/articles/PMC7570000/ /pubmed/32927616 http://dx.doi.org/10.3390/jcdd7030035 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Loomba, Rohit S.
Tretter, Justin T.
Mohun, Timothy J.
Anderson, Robert H.
Kramer, Scott
Spicer, Diane E.
Identification and Morphogenesis of Vestibular Atrial Septal Defects
title Identification and Morphogenesis of Vestibular Atrial Septal Defects
title_full Identification and Morphogenesis of Vestibular Atrial Septal Defects
title_fullStr Identification and Morphogenesis of Vestibular Atrial Septal Defects
title_full_unstemmed Identification and Morphogenesis of Vestibular Atrial Septal Defects
title_short Identification and Morphogenesis of Vestibular Atrial Septal Defects
title_sort identification and morphogenesis of vestibular atrial septal defects
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7570000/
https://www.ncbi.nlm.nih.gov/pubmed/32927616
http://dx.doi.org/10.3390/jcdd7030035
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