Quadricuspid aortic valve (QAV) is a rare congenital cardiac anomaly that commonly becomes symptomatic in the fifth or sixth decade of life and can present as an isolated finding or in association with other congenital cardiovascular abnormalities. Previously, QAV was mainly a postmortem or intraoperative diagnosis and data was very limited on its natural history, associated complications, and long-term outcomes. In recent decades, however, there has been an increase in the reported cases of QAV, considering the advances in the diagnostic modalities such as transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), and cardiac magnetic resonance (CMR).
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