Focus on a rare clinical entity: unicuspid aortic valve disease

ABSTRACT

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.

KEYWORDS:

• Acommisural aortic valve
• aortopathy
• bicuspid aortic valve
• unicommissural aortic valve
• unicuspid aortic valve

Article highlights

• The natural history and pathophysiology of unicuspid aortic valve disease remains unclear.

• For the accurate diagnosis of unicuspid aortic valve, diagnostic scoring system combinated echocardiography and other imaging modality including MRT or CT might be useful.

Treatment recommendation in guidelines should be warranted

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.