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Quadricuspid aortic valve (QAV) is a rare congenital valvular abnormality with less than 200 cases reported to date. The first QAV was reported in 1862 by Balington. Clinical manifestations of patients with a QAV depend on the functional status of the QAV and the associated cardiac disorders. Most QAV are asymptomatic and are incidentally found. Severe aortic regurgitation and/or stenosis can develop overtime, requiring aortic valve replacement or repair. Transesophageal echocardiography is the preferred modality to diagnose QAV. We pre