Quadricuspid aortic valve is one of the rare forms of congenital cardiac valvular disease. Its diagnosis is often missed, even with the transthoracic echocardiogram. Many of these patients progress to aortic incompetence later in life requiring surgical intervention. In addition, quadricuspid aortic valve can be associated with other congenital cardiac deformities. Hence early recognition and follow-up is critical in these patients. We report a patient with quadricuspid aortic valve identified on intraoperative transesophageal 3-D echocardiography. This 66-year-old male presented with the features of congestive heart failure. The preoperative transthoracic echocardiogram (TTE) disclosed, moderately severe aortic valve insufficiency along with severe mitral and tricuspid regurgitation, but failed to reveal the quadricuspid anomaly of the aortic valve. Interestingly, this patient had undergone transthoracic echocardiography on two previous occasions during the past seven years for the evaluation of his valvular heart disease, which all failed to document this anomaly. Intraoperatively, transesophageal echocardiography (TEE) displayed an aortic valve composed of three medium and one small cusps. Our patient's case demonstrates the usefulness of transesophageal echocardiography in detection of this uncommon congenital malformation.
