title: The functional right ventricle and tricuspid regurgitation in Ebstein's anomaly.
abstract: BACKGROUND
  The size of the functional right ventricle of patients with Ebstein's anomaly is, according to contemporary opinion, small. However, the in vivo size of the functional right ventricle in patients with Ebstein's anomaly is unknown. Furthermore, it is unknown how the apical displacement and regurgitant fraction of the tricuspid valve in Ebstein's anomaly affects the size of the functional right ventricle. Therefore the primary aim of this study was to determine the volume of the functional right ventricle in patients with Ebstein's anomaly. The secondary aim of this study was to assess correlation of the volume of the functional right ventricle to the apical displacement and regurgitant fraction of the tricuspid valve.
  
  
  METHODS
  Thirty-two consecutive patients with Ebstein's anomaly without previous cardiac surgery or shunts were studied prospectively by cardiovascular magnetic resonance. Functional right ventricle, left ventricle, and atrialized right ventricle volumes and tricuspid valve regurgitation were measured.
  
  
  RESULTS
  Functional right ventricle end diastolic volumes were median 127 ml/m² (range: 76-339 ml/m²) and were median 2.5 (range: 1.3-8.8) times larger than the left ventricle volumes. Furthermore, functional right ventricle volumes correlated in a strong positive fashion with tricuspid valve regurgitation (p<0.001, R(2)=0.65) and modestly with the atrialized right ventricle volumes (p=0.027, R(2)=0.16).
  
  
  CONCLUSIONS
  Patients with untreated Ebstein's anomaly have large functional right ventricles. The size of the enlarged functional right ventricle seems to depend on the degree of tricuspid valve regurgitation and not on the size of the atrialized right ventricle or the age of the patient.
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Klinikum an der Technischen Universität München, Munich, Germany. fratz@dhm.mhn.de
authors.forename: Sohrab
authors.initials: S
authors.lastname: Fratz
authors.forename: Christine
authors.initials: C
authors.lastname: Janello
authors.forename: Dorothea
authors.initials: D
authors.lastname: Müller
authors.forename: Manuel
authors.initials: M
authors.lastname: Seligmann
authors.forename: Christian
authors.initials: C
authors.lastname: Meierhofer
authors.forename: Tibor
authors.initials: T
authors.lastname: Schuster
authors.forename: Christian
authors.initials: C
authors.lastname: Schreiber
authors.forename: Stefan
authors.initials: S
authors.lastname: Martinoff
authors.forename: John
authors.initials: J
authors.lastname: Hess
authors.forename: Andreas
authors.initials: A
authors.lastname: Kühn
authors.forename: Manfred
authors.initials: M
authors.lastname: Vogt
authors.forename: Heiko
authors.initials: H
authors.lastname: Stern
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D014262:Tricuspid Valve Insufficiency / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology*; D016278:Ventricular Function, Right / Q000502:physiology*; D055815:Young Adult