A double-orifice atrioventricular valve case: intraoperative transesophageal echocardiography in diagnosis and treatment.

We describe a 45-yr-old woman with an intermediate type atrioventricular septal defect associated with a double-orifice left atrioventricular valve (DOLAV). We diagnosed this exceptional anomaly by intraoperative transesophageal echocardiography (TEE) during surgery that was scheduled for only a primum type atrial septal defect (ASD) repair. Preoperative transthoracic echocardiography and angiography revealed the ASD but could not demonstrate the DOLAV. We were able to repair this rare and challenging abnormality successfully under the guidance of TEE imaging during the operation. TEE provides valuable information about both anatomy and functional aspect of the valvular structures. Besides its proven role in cardiac surgery, intraoperative use of TEE also serves as a useful tool for diagnosis of such unexpected and potentially missed abnormalities.