title: Subjective evaluation of right ventricular systolic function in hypoplastic left heart syndrome: how accurate is it?
abstract: BACKGROUND
  The geometry and heterogeneity of the right ventricle in hypoplastic left heart syndrome makes objective echocardiographic assessment of systolic function challenging. Consequently, subjective echocardiographic assessment of right ventricular (RV) function is still routinely undertaken. The aims of this study were to compare this with magnetic resonance imaging (MRI), investigate the impact of experience and training on the accuracy of subjective assessment, and critically analyze the role of echocardiography to detect impaired systolic function.
  
  
  METHODS
  A retrospective analysis of prospectively acquired data was performed. Children with hypoplastic left heart syndrome underwent routine preoperative cardiac MRI and echocardiography under the same general anesthetic. Echocardiograms were reviewed, and members of the congenital heart disease team with differing echocardiography experience subjectively graded RV systolic function (good, moderate, or poor). This was compared with MRI-derived ejection fraction.
  
  
  RESULTS
  Twenty-eight patients at different palliative stages were included. Twenty-eight observers were divided into five experience categories (congenital heart disease junior trainees to attending cardiologists). Median agreement was 47.6% (range, 31.4%-58.2%), with the lowest agreement among junior trainees and the highest among attending cardiologists. When used as a screening test for poor RV systolic function, the median sensitivity of echocardiography was 0.89 (range, 0.86-0.96), and median specificity was 0.45 (range, 0.26-0.55). The highest sensitivity was observed among junior trainees but with the lowest specificity. The highest specificity was observed among attending cardiologists (0.55).
  
  
  CONCLUSIONS
  Agreement between echocardiographic and MRI RV ejection fraction improves with experience but remains suboptimal. When used as a screening test for poor RV function, echocardiography is sensitive, but specificity is heavily influenced by operator experience.
authors.affiliation: Department of Paediatric Cardiology, Level 6 Evelina Children's Hospital, London, United Kingdom. hannah.bellsham-revell@kcl.ac.uk
authors.forename: Hannah R
authors.initials: HR
authors.lastname: Bellsham-Revell
authors.forename: John M
authors.initials: JM
authors.lastname: Simpson
authors.forename: Owen I
authors.initials: OI
authors.lastname: Miller
authors.forename: Aaron J
authors.initials: AJ
authors.lastname: Bell
mesh_terms: D002675:Child, Preschool; D015150:Echocardiography, Doppler / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery; D007223:Infant; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D009200:Myocardial Contraction; D011379:Prognosis; D012372:ROC Curve; D015203:Reproducibility of Results; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013599:Systole; D016278:Ventricular Function, Right / Q000502:physiology*