Risk Stratification in Bicuspid Aortic Valve Aortopathy: Emerging Evidence and Future Perspectives

The current management of aortic dilatation associated with congenital bicuspid aortic valve (bicuspid aortic valve aortopathy) is based on dimensional parameters (diameter of the aneurysm, growth of the diameter over time) and few other criteria. The disease is however heterogeneous in terms of natural and clinical history and risk of acute complications, ie aortic dissection. Dimensional criteria are now admitted to have limited value as predictors of such complications. Thus, novel principles for risk stratification have been recently investigated, including phenotypic criteria, flow-related metrics, and circulating biomarkers. A systematization of the typical anatomoclinical forms that the aortopathy can assume has led to the identification of the more severe root phenotype, associated with higher risk of progression of the aneurysm and possible higher aortic dissection risk. Four-dimensional-flow magnetic resonance imaging studies are searching for potentially clinically significant metrics of flow derangement, based on the recognized association of local abnormal shear stress with wall pathology. Other research initiatives are addressing the question whether circulating molecules could predict the presence or, more importantly, the future development of aortopathy. The present review summarizes the latest progresses in the knowledge on risk stratification of bicuspid aortic valve aortopathy, focusing on critical aspects and debated points.