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Published on: February 8, 2022
The Dorsal Mesenchymal Protrusion and the Pathogenesis of Atrioventricular Septal Defects
Tara Burns1, Yanping Yang2, Emilye Hiriart1
1Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, 173 Ashley Avenue, Charleston, SC 29425, USA.
Insights
Congenital heart defects, particularly Atrioventricular Septal Defects (AVSD), are common birth abnormalities. Research highlights abnormal Dorsal Mesenchymal Protrusion (DMP) development as a key factor in AVSD pathogenesis.
Area of Science:
- Developmental biology
- Cardiovascular research
- Pediatric medicine
Background:
- Congenital heart malformations are the most common birth defects, affecting ~1% of infants annually.
- Congenital Heart Disease (CHD) is a leading cause of infant mortality, surpassing childhood cancers.
- Atrioventricular Septal Defects (AVSD) occur in ~1 in 2000 live births, often necessitating early surgery and leading to lifelong complications like mitral valve regurgitation.
Purpose of the Study:
- To review recent advancements in understanding the molecular and morphological mechanisms of AVSD.
- To elucidate the role of the Dorsal Mesenchymal Protrusion (DMP) in normal atrioventricular septal complex formation.
- To explore the DMP's contribution to the pathogenesis of Atrioventricular Septal Defects.
Main Methods:
- Literature review focusing on recent studies.
- Analysis of molecular and morphological data related to heart development.
- Synthesis of findings on the Dorsal Mesenchymal Protrusion's role in AVSD.
Main Results:
- Abnormal development of the Dorsal Mesenchymal Protrusion (DMP) is increasingly linked to AVSD.
- The DMP, a structure derived from the Second Heart Field, is crucial for normal septal complex formation.
- Understanding DMP development offers new insights into AVSD pathogenesis.
Conclusions:
- The Dorsal Mesenchymal Protrusion plays a critical role in the development of Atrioventricular Septal Defects.
- Further research into the DMP's molecular etiology and morphological mechanisms is essential for understanding AVSD.
- Targeting DMP development may offer future therapeutic strategies for congenital heart defects.
Abstract:
Congenital heart malformations are the most common type of defects found at birth. About 1% of infants are born with one or more heart defect on a yearly basis. Congenital Heart Disease (CHD) causes more deaths in the first year of life than any other congenital abnormality, and each year, nearly twice as many children die in the United States from CHD as from all forms of childhood cancers combined. Atrioventricular septal defects (AVSD) are congenital heart malformations affecting approximately 1 in 2000 live births. Babies born with an AVSD often require surgical intervention shortly after birth. However, even after successful surgery, these individuals typically have to deal with lifelong complications with the most common being a leaky mitral valve. In recent years the understanding of the molecular etiology and morphological mechanisms associated with the pathogenesis of AVSDs has significantly changed. Specifically, these studies have linked abnormal development of the Dorsal Mesenchymal Protrusion (DMP), a Second Heart Field-derived structure, to the development of this congenital defect. In this review we will be discuss some of the latest insights into the role of the DMP in the normal formation of the atrioventricular septal complex and in the pathogenesis of AVSDs.
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