Abstract
Background
Quadricuspid aortic valve (QAV) is a rare congenital heart disease with an estimated incidence of 0.008% to 1.46%. The functional status of QAV is predominantly a pure aortic regurgitation. The extensive use of echocardiography has allowed an early and accurate diagnosis of this malformation. In many cases, the transthoracic echocardiography (TTE) is suitable for the diagnosis but, transesophageal echocardiography (TEE), especially 3-dimensional (3D) TEE, is a tool for the accurate definition of the valve anatomy.
Clinical case
A 60-year-old female underwent a head CT for intermittent headaches for one month ago. She was admitted to neurosurgery in our hospital diagnosed of subarachnoid hemorrhage. Four mm aneurysm was found on the periphery of the right middle cerebral artery on CT examination, and a cerebral aneurysm coil embolization was performed with a catheter in emergency. The possibility of infectious cerebral aneurysm was pointed out, and we examined in cardiovascular internal medicine. TTE revealed moderate aortic regurgitation. The ventricular septum exhibits sigmoid septum. A mobile mass was found near the left ventricular outflow tract in the sigmoid septum. TEE revealed a hypoplasia cusp (accessory cusp) is found between non coronary cusp and right coronary cusp. Aortic valve leaflets have become thickened and regurgitation from the central part of the cusps. 3DTEE was able to more accurately visualize that only the accessory cusp was hypoplastic, and the size of the other three leaflets appears almost the same. Similarly, vegetation on the left ventricular outflow tract were clearly revealed by 3DTEE.
Based on the above, it was diagnosed that quadricuspid aortic valve complicated with infective endocarditis (IE) with aortic valve regurgitation. After treatment with antibiotics according to IE, surgical treatment was scheduled.
Discussion
QAV is a rare congenital heart disease, and TTE is an important imaging tool for accurate diagnosis. Furthermore, TEE, especially 3DTEE, was a more appropriate diagnostic method in QAV and IE.
Abstract P1713 Figure. 3DTEE
Van Praagh's operation for ductus-dependent congenital heart diseases with left ventricular outflow tract obstruction.