Quadricuspid aortic valve (QAV) is a rare congenital anomaly with <200 cases reported to date. Most patients are asymptomatic. Symptoms depend on the functionality of the valve and associated heart defects, most often presenting as aortic regurgitation followed by aortic stenosis. Management guidelines are ill-defined. We present two cases of QAV with rare associations (ventricular septal defect) and variable clinical presentation.
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Mondal et al. (2024) studied this question.
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