Right ventricular performance in Ebstein’s disease by transesophageal strain Doppler echocardiography: A morpho-functional study in adult patients

Background: Ebstein’s anomaly of the tricuspid valve is a complex and fascinating congenital heart defect characterized by a spectrum of anatomical abnormalities that also involve the right atrium and right ventricle. The purpose of the present study was to assess right ventricular function using tissue Doppler imaging (TDI) and strain echocardiography in Ebstein’s patients (pts) to better understand the relationship between functional severity and anatomic derangement. Methods: Transesophageal echocardiography with TDI and strain capabilities (Aplio, Toshiba corp, Tokyo, Japan) was performed in 9 pts aged 12-39 years. 10 age- and sex-matched subjects with no signs of heart disease were selected as normal controls (CTR). Right ventricular ejection fraction (EF), fractional shortening (FS), and tricuspid flow filling parameters (E/A ratio, DT) were determined. Offline analysis of the myocardial velocity data sets was performed using dedicated software. Velocity and strain traces from right ventricular free wall were processed in the esophageal 4-chamber view. Systolic and diastolic TDI values (Sw, Ew, Aw), peak systolic strain and systolic and diastolic strain rate values were determined. Results: Measurements of TDI/SR parameters were rapidly obtained with a low inter- and intra-observer variability. Ew/Aw ratio was significantly reduced in pts compared to CTR (p<0.005). Peak systolic strain and systolic and diastolic strain rate at apical and mid level were also lower in pts than in CTR (p<0.001). Right ventricular EF, FS, E/A ratio, and DT were not significantly different among the two groups. No correlation was found between EF, FS, E/A ratio, DT, and anatomic derangement. A significant relationship was shown between peak systolic strain and apical displacement of septal/posterior leaflet (r=-0.67, p<0.001) and between peak systolic strain and the ratio of proximal to distal right ventricle (r=-0.72, p