Olga Hösch1, Jan Martin Sohns1, Thuy-Trang Nguyen1, Peter Lauerer1, Christina Rosenberg1, Johannes Tammo Kowallick1, Shelby Kutty1, Christina Unterberg1, Andreas Schuster1, Martin Faßhauer1, Wieland Staab1, Thomas Paul1, Joachim Lotz1, Michael Steinmetz2. 1. 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.). 2. 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.
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. METHODS AND 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.
RCT Entities:
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. METHODS AND 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: Michael Steinmetz; Simon Usenbenz; Johannes Tammo Kowallick; Olga Hösch; Wieland Staab; Torben Lange; Shelby Kutty; Joachim Lotz; Gerd Hasenfuß; Thomas Paul; Andreas Schuster Journal: J Cardiovasc Magn Reson Date: 2017-12-14 Impact factor: 5.364
Authors: Michael Steinmetz; Marike Broder; Olga Hösch; Pablo Lamata; Shelby Kutty; Johannes T Kowallick; Wieland Staab; Christian Oliver Ritter; Gerd Hasenfuß; Thomas Paul; Joachim Lotz; Andreas Schuster Journal: Int J Cardiol Date: 2018-02-03 Impact factor: 4.164