[Echo and Doppler cardiographic findings of isolated quadricuspid aortic valve: a case report and a review of the literature].

The two-dimensional and pulsed Doppler echocardiographic features of a case of isolated quadricuspid aortic valve with aortic regurgitation are described. A 62-year-old woman was hospitalized for exertional palpitation and dyspnea. Her physical examination showed the typical findings of aortic regurgitation. Two-dimensional echocardiograms revealed the aortic valve to have four cusps of nearly equal size. The accessory cusp was situated between the right and left coronary cusps. By pulsed Doppler echocardiography, holodiastolic turbulent flow signals were observed in the left ventricle, and the aortic flow pattern showed holodiastolic reverse flow, indicating severe aortic regurgitation. These findings were confirmed by aortography and by surgery. The coronary arteries and pulmonary valve were normal. We attempted a classification of the anatomical variations of the previously reported 34 cases of isolated quadricuspid aortic valves, including our own. They were classified as one smaller (67%), four equal (18%), three smaller (6%), four unequal (6%) and two smaller (3%) types. Twenty of the 34 patients had aortic regurgitation. Bacterial endocarditis and congestive heart failure were the main causes of death.