The aim of this study is to evaluate the role of contrast transesophageal echocardiography (cTEE) in the diagnostic characterization of acute aortic syndromes (AAS) [aortic dissection, intramural hematoma, penetrating ulcer]. We enrolled 66 non-consecutive patients with clinical suspicion of AAS. Standard transesophageal echocardiography and cTEE were performed prior to gated-CT angiography, which has been assumed as reference standard. cTEE was obtained with a single bolus of contrast agent injection. The definitive diagnosis of AAS was made in 48 patients by gated-CT angiography: 22 aortic dissections, 15 intramural hematomas and 11 penetrating aortic ulcers. Standard TEE and cTEE correctly diagnosed AAS in 87 and 100% (P = 0.03) cases respectively. Standard TEE correctly diagnosed aortic dissection in 20/22 (91%) and cTEE in 22/22 (100 %) (P = 0.5) cases. cTEE was superior than standard TEE in the visualization of false lumen entry tear (22/22 vs. 16/22, P = 0.03). Standard TEE correctly diagnosed intramural hematoma in 11/15 and cTEE 15/15 (P = 0.12) cases. Microtears were identified in 3 patients by cTEE an in 1 patient by standard TEE (P = 0.4). The presence of focal contrast enhancement was identified in 4 and 0 patients by cTEE and standard TEE respectively (P = 0.06). Both standard and cTEE correctly diagnosed penetrating aortic ulcer in 11/11 (100%) (P = 1.0) cases. cTEE provides additional value over standard TEE in the diagnosis and in the anatomic and functional characterization of AAS.