Bicuspid aortic valve aortopathy: mechanistic and clinical insights from recent studies.

Abstract

This focused review summarizes key insights from the past 12 months of basic science and clinical research on bicuspid aortic valve (BAV)-associated aortopathy. Recent studies in BAV-associated aortopathy support a heterogeneous spectrum of disease with distinct phenotypes. Basic science studies provide further support for the concept of regional differences in the severity of aortopathy within the aorta of BAV patients. Clinical studies compared outcomes of BAV patients after isolated aortic valve replacement and showed that those with primarily valvular insufficiency as compared with stenosis may be at greater risk for important aortic events over time. These novel insights will be important to optimize future aortic resection strategies and clinical practice guidelines. As the most common congenital heart defect, BAV disease is a considerable health burden. Recent studies show differences in the clinical manifestation of disease patterns that may have important implications for future research and the evolution toward more patient-specific surgical practice guidelines.