Aortic regurgitation as a cause of sudden cardiac death with aortic and left ventricular remodelling - the role of the bicuspid valve

AR is a rare cause of SCD, most commonly associated with bicuspid aortic valve. It can be recognised by prominent ridges on the AV cusps and/or thickening of the cusp free margin with aortic annular dilatation. It is only considered significant as a cause of death when found with increased heart weight, left ventricular dilatation, and/or ventricular fibrosis in the absence of other cardiac pathology. We demonstrate that there is a strong association between BAV, AR and SCD. As BAV is a congenital condition, clinical or surgical intervention could potentially prevent subsequent cardiac enlargement and fibrosis, thereby preventing SCD.