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Early Aberrant Angiogenesis Due to Elastic Fiber Fragmentation in Aortic Valve Disease

1
Divisions of Cardiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USA
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Pediatric Cardiology, Wolfson Children’s Hospital, Jacksonville, FL 32207, USA
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Division of Pediatric Cardiology, Indiana University School of Medicine, Indianapolis, IN 46204, USA
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The Christ Hospital Heart Network, Cincinnati, OH 45219, USA
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Department of Cell Biology and Physiology, Washington University, St. Louis, MO 63130, USA
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Division of Pathology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USA
*
Author to whom correspondence should be addressed.
J. Cardiovasc. Dev. Dis. 2021, 8(7), 75; https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd8070075
Received: 6 May 2021 / Revised: 10 June 2021 / Accepted: 22 June 2021 / Published: 25 June 2021
(This article belongs to the Section Pediatric Cardiology and Congenital Heart Disease)
Elastic fiber fragmentation (EFF) is a hallmark of aortic valve disease (AVD), and neovascularization has been identified as a late finding related to inflammation. We sought to characterize the relationship between early EFF and aberrant angiogenesis. To examine disease progression, regional anatomy and pathology of aortic valve tissue were assessed using histochemistry, immunohistochemistry, and electron microscopy from early-onset (<40 yo) and late-onset (≥40 yo) non-syndromic AVD specimens. To assess the effects of EFF on early AVD processes, valve tissue from Williams and Marfan syndrome patients was also analyzed. Bicuspid aortic valve was more common in early-onset AVD, and cardiovascular comorbidities were more common in late-onset AVD. Early-onset AVD specimens demonstrated angiogenesis without inflammation or atherosclerosis. A distinct pattern of elastic fiber components surrounded early-onset AVD neovessels, including increased emilin-1 and decreased fibulin-5. Different types of EFF were present in Williams syndrome (WS) and Marfan syndrome (MFS) aortic valves; WS but not MFS aortic valves demonstrated angiogenesis. Aberrant angiogenesis occurs in early-onset AVD in the absence of inflammation, implicating EFF. Elucidation of underlying mechanisms may inform the development of new pharmacologic treatments. View Full-Text
Keywords: heart valves; aortic root; elastic fiber; angiogenesis; pediatrics heart valves; aortic root; elastic fiber; angiogenesis; pediatrics
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MDPI and ACS Style

Hinton, R.B.; Juraszek, A.L.; Opoka, A.M.; Landis, B.J.; Smith, J.M.; Mecham, R.P.; Bove, K.E. Early Aberrant Angiogenesis Due to Elastic Fiber Fragmentation in Aortic Valve Disease. J. Cardiovasc. Dev. Dis. 2021, 8, 75. https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd8070075

AMA Style

Hinton RB, Juraszek AL, Opoka AM, Landis BJ, Smith JM, Mecham RP, Bove KE. Early Aberrant Angiogenesis Due to Elastic Fiber Fragmentation in Aortic Valve Disease. Journal of Cardiovascular Development and Disease. 2021; 8(7):75. https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd8070075

Chicago/Turabian Style

Hinton, Robert B., Amy L. Juraszek, Amy M. Opoka, Benjamin J. Landis, J. M. Smith, Robert P. Mecham, and Kevin E. Bove 2021. "Early Aberrant Angiogenesis Due to Elastic Fiber Fragmentation in Aortic Valve Disease" Journal of Cardiovascular Development and Disease 8, no. 7: 75. https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd8070075

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