title: Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow aneurysms or akinesia and adverse right-to-left ventricular interaction.
abstract: OBJECTIVE
  We examined the relationship among biventricular hemodynamics, pulmonary regurgitant fraction (PRF), right ventricular outflow tract (RVOT) aneurysm or akinesia, and baseline and surgical characteristics in adults with repaired tetralogy of Fallot (rTOF).
  
  
  BACKGROUND
  The precise relationship of pulmonary regurgitation with biventricular hemodynamics has been hampered by limitations of right ventricular (RV) imaging.
  
  
  METHODS
  We assessed 85 consecutive adults with rTOF and 26 matched healthy controls using cardiovascular magnetic resonance imaging.
  
  
  RESULTS
  Patients had higher right ventricular end-diastolic volume index (RVEDVi) (p < 0.001), right ventricular end-systolic volume index (RVESVi) (p < 0.001), right ventricular mass index (RVMi) (p < 0.001), and lower right ventricular ejection fraction (RVEF) (p < 0.001) and left ventricular ejection fraction (LVEF) (p = 0.002) compared to controls. The PRF (range 0% to 55%) independently predicted RVEDVi (p < 0.01) and the latter predicted RVESVi (p < 0.01) and RVMi (p < 0.01). The RVOT aneurysm/akinesia was present in 48/85 (56.9%) of patients and predicted RV volumes (RVEDVi, p = 0.01, and RVESVi, p = 0.03). There was a negative effect of RVOT aneurysm/akinesia and RVMi on RVEF (p < 0.01 and p = 0.02, respectively). There was only a tendency among patients with transannular or RVOT patching toward RVOT aneurysm/akinesia (p = 0.09). The LVEF correlated with RVEF (r = 0.67, p < 0.001).
  
  
  CONCLUSIONS
  Pulmonary regurgitation and RVOT aneurysm/akinesia were independently associated with RV dilation and the latter with RV hypertrophy late after rTOF. The RVOT aneurysm/akinesia was common but related only in part to RVOT or transannular patching. Both RV hypertrophy and RVOT aneurysm/akinesia were associated with lower RVEF. Left ventricular systolic dysfunction correlated with RV dysfunction, suggesting an unfavorable ventricular-ventricular interaction. Measures to maintain or restore pulmonary valve function and avoid RVOT aneurysm/akinesia are mandatory for preserving biventricular function late after rTOF.
authors.affiliation: Department of Cardiology, Royal Brompton Hospital, London, United Kingdom.
authors.forename: Periklis A
authors.initials: PA
authors.lastname: Davlouros
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Kilner
authors.forename: Tim S
authors.initials: TS
authors.lastname: Hornung
authors.forename: Wei
authors.initials: W
authors.lastname: Li
authors.forename: Jane M
authors.initials: JM
authors.lastname: Francis
authors.forename: James C C
authors.initials: JC
authors.lastname: Moon
authors.forename: Gillian C
authors.initials: GC
authors.lastname: Smith
authors.forename: Tri
authors.initials: T
authors.lastname: Tat
authors.forename: Dudley J
authors.initials: DJ
authors.lastname: Pennell
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D013504:Cardiovascular Surgical Procedures; D003935:Diagnostic Techniques, Cardiovascular; D020820:Dyskinesias / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D005260:Female; D006322:Heart Aneurysm / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D008131:London; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011446:Prospective Studies; D011665:Pulmonary Valve Insufficiency / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D011666:Pulmonary Valve Stenosis / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D012720:Severity of Illness Index; D013318:Stroke Volume / Q000502:physiology; D013771:Tetralogy of Fallot / Q000175:diagnosis* / Q000503:physiopathology* / Q000601:surgery; D016896:Treatment Outcome; D014463:Ultrasonography; D016278:Ventricular Function, Right / Q000502:physiology*; D014694:Ventricular Outflow Obstruction / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery