Quadriscupid Aortic Valve with Concurrent Aortic Stenosis and Insufficiency

Area of Science:

• Cardiology
• Medical Imaging
• Congenital Heart Disease

Background:

• Congenital heart defects, such as quadricuspid aortic valve (QAV), are rare but can lead to significant valvular dysfunction.
• Mixed aortic valve dysfunction, encompassing both stenosis and regurgitation, presents diagnostic challenges.

Purpose of the Study:

• To report a case of congenital quadricuspid aortic valve diagnosed via cardiac MRI.
• To illustrate the characteristic imaging findings of QAV.
• To highlight the management of QAV with associated mixed aortic valve dysfunction.

Main Methods:

• Cardiac Magnetic Resonance Imaging (MRI) was utilized for detailed assessment of aortic valve morphology and function.
• Clinical presentation and echocardiographic findings were considered in conjunction with MRI data.
• Surgical intervention (Bentall procedure) was performed for valve and aortic root replacement.

Main Results:

• Cardiac MRI revealed a quadricuspid aortic valve morphology, consistent with a four-leaf-clover appearance.
• The patient presented with mixed aortic valve dysfunction, including stenosis and regurgitation.
• Successful surgical correction was achieved through a Bentall procedure.

Conclusions:

• Cardiac MRI is a valuable tool for diagnosing congenital quadricuspid aortic valve and assessing associated dysfunction.
• Quadricuspid aortic valve is a rare congenital anomaly that can lead to severe mixed aortic valve disease.
• Surgical management, including aortic valve, root, and ascending aorta replacement, can be successful in selected cases.