Congenital Anomalies of the Aortic Valve

  • Jan VojacekEmail author
  • Pavel ZacekEmail author
  • Jan Dominik
  • Tomas Holubec


The aortic valve may exist in congenitally anomalous variants that differ in the number of valve leaflets. Most important is the bicuspid aortic valve which is relatively frequent congenital disorder. Unicuspid aortic valve is less frequent and quadricuspid aortic valve is rare. All congenitally anomalous aortic valves display aberrant haemodynamic flow pattern and, despite a considerably long asymptomatic interval, are prone to develop into a significant aortic valve disease, either a stenosis or regurgitation (or both). Interrelation of aberrant aortic valve types and aortic dilation aortopathies is well documented though yet not fully understood. A thorough knowledge of congenital aortic anomalies is essential both for proper surgical strategy regarding the valve disease and decision for preventive surgery on a dilated ascending aorta.


Bicuspid aortic valve Unicuspid aortic valve Quadricuspid aortic valve Aortic dilation Surgery 


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Cardiac SurgeryCharles University, Faculty of Medicine in Hradec Králové, University Hospital Hradec KrálovéHradec KrálovéCzech Republic
  2. 2.Department of Cardiac SurgeryKerckhoff Heart and Lung CentreBad NauheimGermany

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