title: The total right/left-volume index: a new and simplified cardiac magnetic resonance measure to evaluate the severity of Ebstein anomaly of the tricuspid valve: a comparison with heart failure markers from various modalities.
abstract: BACKGROUND
  The classification of clinical severity of Ebstein anomaly still remains a challenge. The aim of this study was to focus on the interaction of the pathologically altered right heart with the anatomically-supposedly-normal left heart and to derive from cardiac magnetic resonance (CMR) a simple imaging measure for the clinical severity of Ebstein anomaly.
  
  
  RESULTS
  Twenty-five patients at a mean age of 26±14 years with unrepaired Ebstein anomaly were examined in a prospective study. Disease severity was classified using CMR volumes and functional measurements in comparison with heart failure markers from clinical data, ECG, laboratory and cardiopulmonary exercise testing, and echocardiography. All examinations were completed within 24 hours. A total right/left-volume index was defined from end-diastolic volume measurements in CMR: total right/left-volume index=(RA+aRV+fRV)/(LA+LV). Mean total right/left-volume index was 2.6±1.7 (normal values: 1.1±0.1). This new total right/left-volume index correlated with almost all clinically used biomarkers of heart failure: brain natriuretic peptide (r=0.691; P=0.0003), QRS (r=0.432; P=0.039), peak oxygen consumption/kg (r=-0.479; P=0.024), ventilatory response to carbon dioxide production at anaerobic threshold (r=0.426; P=0.048), the severity of tricuspid regurgitation (r=0.692; P=0.009), tricuspid valve offset (r=0.583; P=0.004), and tricuspid annular plane systolic excursion (r=0.554; P=0.006). Previously described severity indices ([RA+aRV]/[fRV+LA+LV]) and fRV/LV end-diastolic volume corresponded only to some parameters.
  
  
  CONCLUSIONS
  In patients with Ebstein anomaly, the easily acquired index of right-sided to left-sided heart volumes from CMR correlated well with established heart failure markers. Our data suggest that the total right/left-volume index should be used as a new and simplified CMR measure, allowing more accurate assessment of disease severity than previously described scoring systems.
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Olga
authors.initials: O
authors.lastname: Hösch
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Jan Martin
authors.initials: JM
authors.lastname: Sohns
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Thuy-Trang
authors.initials: TT
authors.lastname: Nguyen
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Peter
authors.initials: P
authors.lastname: Lauerer
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Christina
authors.initials: C
authors.lastname: Rosenberg
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Johannes Tammo
authors.initials: JT
authors.lastname: Kowallick
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Shelby
authors.initials: S
authors.lastname: Kutty
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Christina
authors.initials: C
authors.lastname: Unterberg
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Andreas
authors.initials: A
authors.lastname: Schuster
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Martin
authors.initials: M
authors.lastname: Faßhauer
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Wieland
authors.initials: W
authors.lastname: Staab
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Thomas
authors.initials: T
authors.lastname: Paul
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.).
authors.forename: Joachim
authors.initials: J
authors.lastname: Lotz
authors.affiliation: From the Department of Pediatric Cardiology and Intensive Care Medicine (O.H., T.-T.N., P.L., T.P., M.S.), Institute for Diagnostic and Interventional Radiology (J.M.S., C.R., J.T.W., M.F., W.S., J.L.), and Department of Cardiology and Pneumology (C.U., A.S.), Georg-August University, Göttingen, Germany; German Centre for Cardiovascular Research, Germany (J.M.S., C.U., A.S., M.F., W.S., J.L., M.S.); and University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha (S.K.). Michael.steinmetz@med.uni-goettingen.de.
authors.forename: Michael
authors.initials: M
authors.lastname: Steinmetz
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood*; D002306:Cardiac Volume; D002648:Child; D004437:Ebstein Anomaly / Q000175:diagnosis* / Q000503:physiopathology; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000097:blood* / Q000175:diagnosis / Q000503:physiopathology; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010101:Oxygen Consumption; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014261:Tricuspid Valve / Q000002:abnormalities*; D016278:Ventricular Function, Right / Q000502:physiology; D055815:Young Adult
chemical_list: D015415:Biomarkers; D020097:Natriuretic Peptide, Brain
keywords: congenital; heart failure; magnetic resonance imaging