Quadricuspid aortic valve (QAV) is a rare congenital heart disease. The functional status of QAV is predominantly a pure aortic regurgitation. Clinical manifestations of patients with a QAV depend on the functional status of the QAV and the associated disorders. Significant valvular regurgitation and (or) stenosis is often present with subsequent operation performed at the fifth to sixth decade...

BACKGROUND Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. This study sought to determine QAV frequency in a large echocardiography database, to characterize associated cardiovascular abnormalities, and to describe long-term outcomes. METHODS AND RESULTS Fifty patients (mean ± SD age, 43.5 ± 21.8 years at the time of the index diagnosis; female sex, 52%) received a diagno...

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In tetralogy of Fallot (TOF), the most common form of cyanotic congenital heart disease, only a few patients reach adulthood without surgical correction. We present the case of a man with TOF who survived until the age of 75 years without surgical intervention and had a very unusual combination of TOF and quadricuspid aortic valve (QAV). QAV, complicated by aortic regurgitation, is an uncommon ...