Unicuspid aortic valve in Turner syndrome, detected by 3D transoesophageal echocardiography: The usefulness of 3D echo in challenge diagnosis.

Turner syndrome is a genetic disorder associated with a variable range of cardiac congenital diseases. Out of these, unicuspid aortic valve is a rare malformation, related to premature aortic stenosis and its diagnosis represents a challenge with transthoracic echocardiography. The application of 3D echocardiography could facilitate the diagnosis, especially with transoesophageal approach. Moreover, cardiac computed tomography and cardiac magnetic resonance have demonstrated their usefulness for detection of aortic morphology. We report a case of young patient affected by Turner syndrome, with unicuspid aortic valve, identified by 3D transoesophageal echocardiography and confirmed by cardiac computed tomography. The patient was submitted to aortic valve replacement.

Importance of anterior leaflet tethering in predicting recurrence of ischemic mitral regurgitation after restrictive annuloplasty.

OBJECTIVE: We investigated the relationship between anterior mitral leaflet (AML) tethering and recurrent ischemic mitral regurgitation (MR) after restrictive annuloplasty. We also explored whether the effect of AML tethering was secondary to modifications in left ventricular size and geometry. METHODS: The study population consisted of 435 consecutive patients with chronic ischemic MR who survived combined coronary artery bypass grafting and undersized mitral ring annuloplasty performed at 3 institutions (University Hospital, Maastricht, The Netherlands; Careggi Hospital, Florence, Italy; and Civic Hospital, Brescia, Italy) from 2001 to 2008. The median follow-up was 44.7 months (interquartile range 25.9-66.4). The patients were divided by the baseline measurements into quintiles of AML tethering angle α' as follows: group 1, normal/slight AML tethering; group 2, mild AML tethering; group 3, moderate AML tethering; group 4, moderate-to-severe AML tethering; and group 5, severe AML tethering. RESULTS: Recurrence of MR was significantly greater in patients with moderate-to-severe (28.3%) and severe (39.4%) AML tethering (P < .001). A strong correlation was found between α' (r = 0.83, P < .001) and recurrent MR but a weak correlation with the posterior mitral angle ß' (r = 0.12, P = .05). On logistic regression analysis corrected for other echocardiographic risk factors, moderate-severe AML tethering or worse (adjusted odds ratio, 3.6; 95% confidence interval, 3.0-4.1; P < .001) was a strong predictor of MR recurrence. Compared with patients with ß' of 45 or greater, those with severe and moderate-severe AML tethering had more than 3.7 and 1.7 times greater odds of MR recurrence, respectively. No significant interactions were found between α' and the indexes of left ventricular function and geometry. CONCLUSIONS: Preoperative moderate-severe AML tethering or worse was strongly associated with MR recurrence. Thus, assessment of leaflet tethering should be incorporated into clinical risk assessment and prediction models.