Document Detail


The pathogenesis of atrial and atrioventricular septal defects with special emphasis on the role of the dorsal mesenchymal protrusion.
MedLine Citation:
PMID:  22709652     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Partitioning of the four-chambered heart requires the proper formation, interaction and fusion of several mesenchymal tissues derived from different precursor populations that together form the atrioventricular mesenchymal complex. This includes the major endocardial cushions and the mesenchymal cap of the septum primum, which are of endocardial origin, and the dorsal mesenchymal protrusion (DMP), which is derived from the Second Heart Field. Failure of these structures to develop and/or fully mature results in atrial septal defects (ASDs) and atrioventricular septal defects (AVSD). AVSDs are congenital malformations in which the atria are permitted to communicate due to defective septation between the inferior margin of the septum primum and the atrial surface of the common atrioventricular valve. The clinical presentation of AVSDs is variable and depends on both the size and/or type of defect; less severe defects may be asymptomatic while the most severe defect, if untreated, results in infantile heart failure. For many years, maldevelopment of the endocardial cushions was thought to be the sole etiology of AVSDs. More recent work, however, has demonstrated that perturbation of DMP development also results in AVSD. Here, we discuss in detail the formation of the DMP, its contribution to cardiac septation and describe the morphological features as well as potential etiologies of ASDs and AVSDs.
Authors:
Laura E Briggs; Jayant Kakarla; Andy Wessels
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review     Date:  2012-06-17
Journal Detail:
Title:  Differentiation; research in biological diversity     Volume:  84     ISSN:  1432-0436     ISO Abbreviation:  Differentiation     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-07-03     Completed Date:  2012-11-12     Revised Date:  2013-07-12    
Medline Journal Info:
Nlm Unique ID:  0401650     Medline TA:  Differentiation     Country:  England    
Other Details:
Languages:  eng     Pagination:  117-30     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 International Society of Differentiation. Published by Elsevier B.V. All rights reserved.
Affiliation:
Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, 173 Ashley Avenue, Charleston, South Carolina 29425, USA.
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MeSH Terms
Descriptor/Qualifier:
Animals
Endocardial Cushion Defects / embryology,  etiology*
Endocardial Cushions / embryology*,  pathology
Heart Septal Defects, Atrial / embryology,  etiology*
Heart Septal Defects, Ventricular / embryology,  etiology*
Humans
Mesoderm / embryology*,  pathology
Mice
Grant Support
ID/Acronym/Agency:
3P20RR016434-10S1/RR/NCRR NIH HHS; 5T32-GM008716-12/GM/NIGMS NIH HHS; P01 HD039946/HD/NICHD NIH HHS; R01 HL084285/HL/NHLBI NIH HHS; R01HL084285/HL/NHLBI NIH HHS; //Wellcome Trust
Comments/Corrections

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