The quadricuspid aortic valve (QAV) is a rare congenital anomaly that typically is unrelated to other cardiac anomalies. It usually is discovered incidentally through imaging modalities such as echocardiogram or computed tomography angiogram, during surgery, or autopsy. Some patients with QAV develop aortic regurgitation and, rarely, other cardiovascular complications like aortic aneurysm. Due to its rarity, it is difficult to characterize these patients or standardize management. However, review of case reports can be very useful in rare medical conditions such as QAV. In this manuscript, in addition to presenting two cases of QAV, the authors reviewed 149 cases of QAV from 2010 to 2020 from published case studies and series in order to gain a better insight into the characteristics of the patient population with QAV and its management. (c) 2021 Elsevier Inc. All rights reserved.
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