Evolution of Precision Medicine and Surgical Strategies for Bicuspid Aortic Valve-Associated Aortopathy

Abstract

Bicuspid aortic valve (BAV) is a common congenital cardiac malformation affecting 1–2% of people. BAV results from fusion of two adjacent aortic valve cusps, and is associated with dilatation of the aorta, known as bicuspid valve associated aortopathy. Bicuspid valve aortopathy is progressive and associated with catastrophic clinical events, such as aortic dissection and rupture. Therefore, frequent monitoring and early intervention with prophylactic surgical resection of the proximal aorta is often recommended. However, the specific pattern of aortopathy is highly variable among patients, with different segments of the ascending aorta being affected. Individual patient risks are sometimes difficult to predict. Resection strategies are informed by current surgical guidelines which are primarily based on aortic size and growth criteria. These criteria may not optimally reflect the risk of important aortic events. To address these issues in the care of patients with bicuspid valve aortopathy, our translational research group has focused on validating use of novel imaging techniques to establish non-invasive hemodynamic biomarkers for risk-stratifying BAV patients. In this article, we review recent efforts, successes, and ongoing challenges in the development of more precise and individualized surgical approaches for patients with bicuspid aortic valves and associated aortic disease.

Evolving Surgical Approaches to Bicuspid Aortic Valve Associated Aortopathy

“…There is a growing body of evidence supporting valve-related hemodynamics as an underlying cause of bicuspid aortopathy (13). Using four dimensional (4-D) flow MRI to map blood flow through the valve and aorta, normally-functioning bicuspid aortic valve patients were observed to have disturbed ascending aortic flow and increased regional hemodynamic stresses (14).…”

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

“…Studies currently focusing on the identification of new criteria for risk stratification in BAV aortopathy are based on 3 research lines: clinical significance of aorta "phenotypes" [1], novel imaging techniques capable to describe and quantify flow-related mechanical stimuli, 7 and circulating molecules representing potential biomarkers of the presence or severity of the aortic wall disease. 8 The present review addresses these 3 lines of research underscoring both interesting applicative perspectives and current limitations.…”

Assessment of aortic and cerebral haemodynamics and vascular brain injury with 3 and 7 T magnetic resonance imaging in patients with aortic coarctation

“…This finding is likely attributable to the eccentric blood flow and resulting energy loss within the ascending aorta in patients with BAV. 29 , 30 …”