title: Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein's anomaly: comparison with CMR-derived ejection fraction in 49 patients.
abstract: OBJECTIVE
  Ebstein's anomaly (EA) is often associated with right ventricular (RV) dysfunction. Data on echocardiographic quantification of RV function are, however, rare. The aim of this study was to determine how non-volumetric echocardiographic indices and qualitative assessment of global systolic RV function correlate with cardiovascular magnetic resonance (CMR)-derived RV ejection fraction (EF).
  
  
  RESULTS
  We compared six echocardiographic indices and qualitative assessment of RV function with the gold standard CMR. A total of 49 unoperated patients with EA and a mean age of 32 ± 18 years were examined. Tricuspid annular plane systolic excursion, tissue Doppler myocardial velocities (peak S and IVA) and 2D strain and strain rate measures for the RV were compared with CMR-derived EF. Only 2D global longitudinal strain (2D-GLS), out of the six parameters investigated, showed a weak, although statistically significant correlation with CMR-derived RVEF (R = -0.4, P = 0.01). Using a cut-off value of -20.15, 2D-GLS sensitivity (77%) and specificity (46%) in detecting patients with a CMR-derived EF of <50% were comparable with qualitative assessment (sensitivity 77%, specificity 45%).
  
  
  CONCLUSIONS
  Overall echocardiographic parameters of RV function correlate poorly with CMR-derived EF in patients with EA. Only 2D global longitudinal RV strain correlated weakly with CMR-derived RVEF. However, the sensitivity and specificity for detecting RV dysfunction using 2D strain imaging were comparable with qualitative RV functional assessment.
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, Munich 80636, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Kühn
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, Munich 80636, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Meierhofer
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, Munich 80636, Germany.
authors.forename: Tobias
authors.initials: T
authors.lastname: Rutz
authors.affiliation: Institute of Medical Statistics and Epidemiology, Klinikum Rechts der Isar, Technische Universität München, Munich, Germany.
authors.forename: Ina-Christine
authors.initials: IC
authors.lastname: Rondak
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, Munich 80636, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Röhlig
authors.affiliation: Department of Cardiovascular Surgery, Deutsches Herzzentrum München, Technische Universität München, Munich, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Schreiber
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, Munich 80636, Germany.
authors.forename: Sohrab
authors.initials: S
authors.lastname: Fratz
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, Munich 80636, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Ewert
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, Munich 80636, Germany drvogt@dhm.mhn.de.
authors.forename: Manfred
authors.initials: M
authors.lastname: Vogt
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D004437:Ebstein Anomaly / Q000150:complications / Q000000981:diagnostic imaging*; D004452:Echocardiography; D018618:Echocardiography, Doppler, Color / Q000379:methods; D005260:Female; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D036301:Qualitative Research; D013318:Stroke Volume / Q000502:physiology*; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging* / Q000209:etiology / Q000503:physiopathology; D055815:Young Adult
keywords: Ebstein's anomaly; cardiovascular magnetic resonance; echocardiography; right ventricular function