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We report a rare subtype of quadricuspid aortic valve (QAV) associated with moderate aortic regurgitation in a 17- year old woman symptomatic for palpitations. The patient was admitted to our department for cardiac evaluation due to a previous diagnosis of bicuspid aortic valve; she underwent a new two-dimensional echocardiography revealing a rare type of quadricuspid aortic valve with a moderate regurgitation. For further investigating potentially associated abnormalities, patient was referred to Cardiac MRI; MRI showed no other abnormalities and confirmed echocardiographic findings. Quadricuspid aortic valve is a rare form of congenital valvular anomaly often occasionally diagnosed. In most cases this malformation causes a valve dysfunction, commonly aortic regurgitation, and can be associated with other cardiac abnormalities such as ventricular or atrial septal defect, anomalies of coronary arteries, patent ductus arteriosus, subaortic fibromuscolar stenosis and mitral valve malformation.
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