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Open AccessEditorial

Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach

1
Department of Cardiovascular Surgery, University Heart and Vascular Center Hamburg, Martinistraße 52, 20246 Hamburg, Germany
2
Partner site Hamburg/Lübeck/Kiel, German Center of Cardiovascular Research (DZHK), 20246 Hamburg, Germany
3
Department of Congenital Heart Disease and Pediatric Cardiology German Heart Center Munich, Technical University Munich, 80333 Munich, Germany
4
Department of Cardiology, University Heart and Vascular Center Hamburg, 20246 Hamburg, Germany
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2020, 9(1), 204; https://doi.org/10.3390/jcm9010204
Received: 6 January 2020 / Accepted: 9 January 2020 / Published: 11 January 2020
Congenital heart disease (CHD)-associated aortopathy is a very heterogeneous entity with a wide spectrum of clinical presentations. The pathogenesis of aortopathy is still incompletely understood, and, therefore, the best prevention and management strategy is currently unknown. The most common entity of CHD-associated aortopathies is bicuspid aortic valve (BAV)-associated aortic disease (so called bicuspid aortopathy) that is found in 50%–60% of BAV individuals. BAV aortopathy has been reported in association with an increased risk of aortic events, especially aortic dissection and sudden cardiac death. Risk stratification of adverse aortic events is still very rudimentary and considers only the maximal aortic diameter, which makes it unsuitable for an individual risk prediction. This introductory Editorial highlights the unmet clinical need for more integrative and translational research to unravel pathogenetic pathways in the development of CHD-associated aortopathies, integrating recently identified genetic lesions and knowledge on circulating biomarkers and microstructural changes in the diseased aorta. View Full-Text
Keywords: aortopathy; bicuspid aortic valve; congenital heart disease aortopathy; bicuspid aortic valve; congenital heart disease
MDPI and ACS Style

Girdauskas, E.; Kaemmerer, H.; von Kodolitsch, Y. Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach. J. Clin. Med. 2020, 9, 204.

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