Quadricuspid Aortic Valve: An Introduction for Clinicians

Area of Science:

• Cardiology
• Congenital Heart Disease
• Valvular Heart Disease

Background:

• Quadricuspid aortic valve (QAV) is a rare congenital anomaly where the aortic valve has four cusps.
• Historically diagnosed during surgery or autopsy, QAV is now increasingly detected via echocardiography.
• Associated congenital cardiovascular defects, particularly coronary artery anomalies, are noted in some cases.

Purpose of the Study:

• To summarize the current understanding of quadricuspid aortic valve.
• To highlight diagnostic advancements and clinical implications of QAV.
• To review associated defects and management strategies for QAV.

Main Methods:

• Literature review of published cases of QAV.
• Analysis of diagnostic modalities, focusing on echocardiography.
• Review of clinical outcomes, including associated defects and surgical interventions.

Main Results:

• QAV incidence is estimated between 0.013% and 0.043%, with increased detection due to improved diagnostics.
• Aortic regurgitation (AR) develops in over half of QAV cases, typically without aortic stenosis.
• Surgical intervention is required in approximately 20% of all QAV cases and two-thirds of those with AR, with tricuspidization often preferred.

Conclusions:

• Quadricuspid aortic valve is an uncommon congenital heart anomaly with significant potential for developing aortic regurgitation.
• Early and accurate diagnosis via echocardiography is crucial for managing QAV and its complications.
• Surgical management, including valve repair, is often necessary for symptomatic QAV, particularly in cases of aortic regurgitation.