Assessment of left ventricular deformation in patients with Ebstein's anomaly by cardiac magnetic resonance tissue tracking.

PURPOSE: The aim of this study was to clarify the feasibility of myocardial strain using cardiovascular magnetic resonance (CMR) for the evaluation of left ventricular (LV) deformation in patients with Ebstein's anomaly (EA). MATERIALS AND METHODS: We recruited 32 patients with EA and 30 controls for CMR examination and measured LV function, dimension and tissue tracking parameters (the global and regional radial, circumferential and longitudinal peak strain), together with the right ventricle (RV) dimension. LV strain parameters were compared among the controls, patients with preserved LV ejection fraction (LVEF; ≥55%), and patients with reduced LVEF (<55%). Pearson's correlation was used to evaluate relationships between tissue tracking parameters with the RVEDD/LVEDD index and LVEF. An ROC analysis was also performed to determine whether the cut-off values for PS could be used to differentiate LV dysfunction between patients with EA and controls. The intraclass correlation coefficient (ICC) was used to assess the inter- and intra-observer variability. RESULTS: The global strain parameters all decreased significantly in the EA group compared with the control group (all P<0.05). Furthermore, the global radial and circumferential peak strain (PS) were obviously even lower in the reduced LVEF group than the strain measured in preserved LVEF groups (28.64% vs. 37.39%, p<0.05; and -8.20% vs. -17.89%; p<0.05; respectively). The regional strain abnormalities in EA patients were mainly involved in basal and middle segments. The results also demonstrated a significant correlation between the ratio of the RV end-diastolic dimension to the LV end-diastolic dimension (RVEDD/LVEDD index) with the global circumferential PS (r=0.508) and the longitudinal PS (r=0.474), as well as a good correlation between radial PS and LVEF (r=0.465). The ICCs for intra- and inter-observer variability were 0.797-0.904 and 0.701-0.896. CONCLUSIONS: LV strain serves an earlier and more comprehensive measurement of LV dysfunction than LVEF in EA, which could potentially be included as a supplementary diagnostic procedure in the evaluation of EA.

Echocardiographic assessment of neonates with pulmonary atresia and intact ventricular septum.

In this prospective study, 27 consecutive neonates suspected to be suffering from pulmonary atresia and intact ventricular septum underwent detailed two-dimensional echocardiographic examination before cardiac catheterization. Of the 27 neonates 25 had pulmonary atresia and intact ventricular septum and the remaining 2 had "functional pulmonary atresia" secondary to severe Ebstein's anomaly of the tricuspid valve. In all 25 neonates with pulmonary atresia and intact ventricular septum, the diagnosis and right ventricular morphology based on the tripartite approach were correctly established by echocardiography. The associated Ebstein's anomaly in two babies with pulmonary atresia and intact ventricular septum was also correctly identified by echocardiography. Among the five babies who had a sinusoidal-coronary artery communication, echocardiography demonstrated the fistula in one and provided clues for its diagnosis in two others. In the 25 neonates with pulmonary atresia and intact ventricular septum, the echocardiographic dimensions of their tricuspid anulus, right ventricular infundibulum and main pulmonary artery correlated well with the angiocardiographic measurements (r greater than 0.8). The results of this study suggest that, in the management of neonates with pulmonary atresia and intact ventricular septum, preoperative evaluation by echocardiography is usually sufficient and cardiac catheterization should be reserved for selected cases.

Two-dimensional echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment.

This report details the two-dimensional echocardiographic assessment of 41 patients with Ebstein's anomaly. The anatomic spectrum of Ebstein's anomaly is correlated with surgical and autopsy observations in 66% of these patients. Morphologic abnormalities of the tricuspid valve and right heart structures were uniquely assessed by noninvasive two-dimensional echocardiographic examination. Features of the tricuspid valve that best related to decreased functional capacity were: 1) absence of the septal leaflet, and 2) pronounced tethering, restriction of motion and displacement of the anterior leaflet of the tricuspid valve. This 4 1/2 year experience suggests that two-dimensional echocardiography can replace angiography as the procedure of choice for diagnostic and morphologic assessment of Ebstein's anomaly. Cardiac catheterization is necessary only when specific hemodynamic questions or clinical inconsistencies exist.