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Review

Role of the Epicardium in the Development of the Atrioventricular Valves and Its Relevance to the Pathogenesis of Myxomatous Valve Disease

1
Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, 173 Ashley Avenue, Charleston, SC 29425, USA
2
Department of Medical Biology, Amsterdam UMC, Academic Medical Center, Meibergdreef 15, 1105 AZ Amsterdam, The Netherlands
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
J. Cardiovasc. Dev. Dis. 2021, 8(5), 54; https://doi.org/10.3390/jcdd8050054
Received: 26 April 2021 / Revised: 7 May 2021 / Accepted: 8 May 2021 / Published: 12 May 2021
This paper is dedicated to the memory of Dr. Adriana “Adri” Gittenberger-de Groot and in appreciation of her work in the field of developmental cardiovascular biology and the legacy that she has left behind. During her impressive career, Dr. Gittenberger-de Groot studied many aspects of heart development, including aspects of cardiac valve formation and disease and the role of the epicardium in the formation of the heart. In this contribution, we review some of the work on the role of epicardially-derived cells (EPDCs) in the development of the atrioventricular valves and their potential involvement in the pathogenesis of myxomatous valve disease (MVD). We provide an overview of critical events in the development of the atrioventricular junction, discuss the role of the epicardium in these events, and illustrate how interfering with molecular mechanisms that are involved in the epicardial-dependent formation of the atrioventricular junction leads to a number of abnormalities. These abnormalities include defects of the AV valves that resemble those observed in humans that suffer from MVD. The studies demonstrate the importance of the epicardium for the proper formation and maturation of the AV valves and show that the possibility of epicardial-associated developmental defects should be taken into consideration when determining the genetic origin and pathogenesis of MVD. View Full-Text
Keywords: atrioventricular valve; epicardium; lateral cushion; major cushion; myxomatous degeneration atrioventricular valve; epicardium; lateral cushion; major cushion; myxomatous degeneration
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MDPI and ACS Style

Wolters, R.; Deepe, R.; Drummond, J.; Harvey, A.B.; Hiriart, E.; Lockhart, M.M.; van den Hoff, M.J.B.; Norris, R.A.; Wessels, A. Role of the Epicardium in the Development of the Atrioventricular Valves and Its Relevance to the Pathogenesis of Myxomatous Valve Disease. J. Cardiovasc. Dev. Dis. 2021, 8, 54. https://doi.org/10.3390/jcdd8050054

AMA Style

Wolters R, Deepe R, Drummond J, Harvey AB, Hiriart E, Lockhart MM, van den Hoff MJB, Norris RA, Wessels A. Role of the Epicardium in the Development of the Atrioventricular Valves and Its Relevance to the Pathogenesis of Myxomatous Valve Disease. Journal of Cardiovascular Development and Disease. 2021; 8(5):54. https://doi.org/10.3390/jcdd8050054

Chicago/Turabian Style

Wolters, Renélyn, Ray Deepe, Jenna Drummond, Andrew B. Harvey, Emilye Hiriart, Marie M. Lockhart, Maurice J.B. van den Hoff, Russell A. Norris, and Andy Wessels. 2021. "Role of the Epicardium in the Development of the Atrioventricular Valves and Its Relevance to the Pathogenesis of Myxomatous Valve Disease" Journal of Cardiovascular Development and Disease 8, no. 5: 54. https://doi.org/10.3390/jcdd8050054

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