Focus on a rare clinical entity: unicuspid aortic valve disease.

INTRODUCTION: While the understanding of the pathophysiology and clinical implication of bicuspid aortic valves evolves rapidly, the natural history of unicuspid aortic valves is still poorly understood. Hence, a universally accepted diagnostic work-up process and therapy recommendations for UAV still have to be established. AREAS COVERED: This article aims to give an overview on the most recent literature addressing the pathophysiology, the diagnostic tools and appropriate surgical therapy options of unicuspid aortic valve. Due to the rare prevalence, the understanding of pathophysiology is still missing. Further, symptomatic aortic valve disease are seen much earlier life stage in this cohorts. Thus, it highlights the several surgical treatment options with pro and contra especially for the young adult cohorts. EXPERT OPINION: Large scale prospective observational studies using standardized diagnostic criteria are needed to reveal the clinical course. Further appropriate treatment strategies of unicuspid aortic valve patients is demanded.