Sinus of Valsalva aneurysm is a rare congenital anomaly which, if overlooked, may be associated with increased mortality and morbidity. Multiplane transesophageal echocardiography proved useful in identifying a variety of associated structural heart disease. This study sought to assess the accuracy of the surgical result on the basis of the multiplane transesophageal echocardiography findings and to describe patient demographics and clinical outcome in an Oriental patient cohort. From July 1984 to December 1995, clinical, catheterization, echocardiographic, and surgical results were retrospectively studied in 23 patients with documented sinus of Valsalva aneurysm. Compared with previous reports of Oriental patients, our patient cohort was older (p < 0.025), had more associated aortic, mitral, and tricuspid regurgitation (p < 0.01), but had fewer coexistent ventricular septal defects (p < 0.01), and had more associated coronary artery disease (9%). Multiplane transesophageal echocardiography precisely showed three undiagnosed and/or ambiguous transthoracic echocardiographic studies, and the preoperative transesophageal echocardiography TEE findings were confirmed intraoperatively in the last eight consecutive patients. We concluded that multiplane transesophageal echocardiography provides conclusive information and is the current technique of choice for diagnosis and clinical management of patients with sinus of Valsalva aneurysm; although the natural history of sinus of Valsalva aneurysm remains uncertain, it is likely that the incidence of unruptured sinus of Valsalva aneurysm is considerably higher in the elderly than has been previously reported.