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Clinical and pathophysiological aspects of bicuspid aortic valve disease

Published online by Cambridge University Press:  30 October 2018

Maria Weinkouff Pedersen ,
Kristian Ambjørn Groth ,
Kristian Havmand Mortensen ,
John Brodersen ,
Claus Højbjerg Gravholt  and
Niels Holmark Andersen Open the ORCID record for Niels Holmark Andersen [Opens in a new window]
Maria Weinkouff Pedersen
Affiliation:
Department of Cardiology, Aalborg University Hospital, Aalborg, Denmark
Kristian Ambjørn Groth
Affiliation:
Department of Cardiology, Aarhus University Hospital, Aarhus, Denmark
Kristian Havmand Mortensen
Affiliation:
Centre for Cardiovascular MR, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
John Brodersen
Affiliation:
Center of Research & Education in General Practice, Department of Public Health, University of Copenhagen, Faculty of Medical Health Sciences, Copenhagen, Denmark Region Zealand, Primary Health Care Research Unit, Copenhagen, Denmark
Claus Højbjerg Gravholt
Affiliation:
Department of Endocrinology and Internal Medicine (MEA), Aarhus University Hospital, Aarhus, Denmark Department of Molecular Medicine, Aarhus University Hospital, Aarhus, Denmark
Niels Holmark Andersen*
Affiliation:
Department of Cardiology, Aalborg University Hospital, Aalborg, Denmark
*
Author for correspondence: Dr N. H. Andersen, DMSc, Department of Cardiology, Aalborg University Hospital, Aalborg, Denmark. Tel: +4522558552; Fax: +9748905674; E-mail: [email protected]
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Abstract

A bicuspid aortic valve is not only a common congenital heart defect but also an enigmatic condition that can cause a large spectrum of diseases, such as aortic valve stenosis and severe heart failure in newborns whereas aortic dissection in adults. On the contrary, a bicuspid aortic valve can also occur with normal function throughout life and never need treatment. Numerous genetic mechanisms are involved in the abnormal cellular functions that may cause abnormal development of the aortic valve during early foetal life. As several chromosomal disorders are also associated with a bicuspid valve, there does not appear to be an apparent common trigger to the abnormal development of the aortic valve. The clinical care of the bicuspid aortic valve patient has been changed by a significant body of evidence that has improved the understanding of the natural history of the disease, including when to best intervene with valve replacement and when to provide prophylactic aortic root surgery. Moreover, as bicuspid valve disease is also part of various syndromes, we can identify high-risk patients in whom a bicuspid valve is much more unfavourable than in the normal population. This review provides an overview of all aspects of the bicuspid aortic valve condition and gives an updated perspective on issues from pathophysiology to clinical care of bicuspid aortic valve disease and associated aortic disease in asymptomatic, symptomatic, and pregnant patients, as well as our viewpoint on population screening.

Keywords

Embryologypregnancyaortic dissectionaortascreening
Type
Review Article
Information
Cardiology in the Young , Volume 29 , Issue 1 , January 2019 , pp. 1 - 10
Copyright
© Cambridge University Press 2018 

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Footnotes

Cite this article: Pedersen MW, Groth KA, Mortensen KH, Brodersen J, Gravholt CH, Andersen NH. (2018) Clinical and pathophysiological aspects of bicuspid aortic valve disease. Cardiology in the Young29: 1–10. doi: 10.1017/S1047951118001658

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