title: The shape and function of the left ventricle in Ebstein's anomaly.
abstract: BACKGROUND
  Left ventricular (LV) failure is common in Ebstein's anomaly, though remains poorly understood. We investigated whether shape deformity impacts LV function.
  
  
  METHODS
  Three-dimensional models of the right ventricle (RV) and LV from 29 adult Ebstein's patients and nine normal subjects were generated from cardiac magnetic resonance image tracings. LV end diastolic (ED) shape, systolic function, septal motion and ventricular interaction were analyzed.
  
  
  RESULTS
  LV ED volume index was normal in Ebstein's (75 ± 19 vs. 78 ± 11 ml/m(2) in normals, p=0.50) but the LV was basally narrowed and modestly dilated apically. LV function was reduced globally (ejection fraction (EF) 41 ± 7 vs. 57 ± 5% in normals, p<0.0001) and regionally (decreased mean segment displacement at end systole (ES) in 12/16 segments, basal Z-scores -2.1 to -1.0). Septal dyskinesis was suggested by outward mean segment displacement in at least one basal septal segment in 25 patients (86%) but refuted by septal thickening in 14 (48%), normal septal curvature at ED and ES, and by visually evident basal LV anterior translation in 27 patients (93%). LV EF correlated better with normalized tricuspid annular plane systolic excursion (r=0.70) than with RV EF (r=0.42) or RVEDVI (r=0.18).
  
  
  CONCLUSIONS
  Although the Ebstein's LV has preserved volume, it exhibits basal narrowing, modest apical dilation and global hypokinesis. The apparent basal septal dyskinesis observed in most patients is likely attributable to anterior cardiac translation rather than true paradoxical motion. LV EF is unaffected by RV volume, correlating well instead with RV longitudinal shortening.
authors.affiliation: University of Washington, Seattle, WA, United States.
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: Goleski
authors.affiliation: University of Washington, Seattle, WA, United States. Electronic address: sheehan@uw.edu.
authors.forename: Florence H
authors.initials: FH
authors.lastname: Sheehan
authors.affiliation: Royal Brompton Hospital, London, United Kingdom.
authors.forename: Sylvia S M
authors.initials: SS
authors.lastname: Chen
authors.affiliation: Royal Brompton Hospital, London, United Kingdom.
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Kilner
authors.affiliation: Royal Brompton Hospital, London, United Kingdom.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
mesh_terms: D000328:Adult; D004437:Ebstein Anomaly / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D006352:Heart Ventricles / Q000033:anatomy & histology* / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D016277:Ventricular Function, Left / Q000502:physiology*; D055815:Young Adult
keywords: Ebstein's anomaly; Left ventricle; Magnetic resonance imaging