title: Systemic right ventricular fibrosis detected by cardiovascular magnetic resonance is associated with clinical outcome, mainly new-onset atrial arrhythmia, in patients after atrial redirection surgery for transposition of the great arteries.
abstract: BACKGROUND
  We hypothesized that fibrosis detected by late gadolinium enhancement (LGE) cardiovascular magnetic resonance predicts outcomes in patients with transposition of the great arteries post atrial redirection surgery. These patients have a systemic right ventricle (RV) and are at risk of arrhythmia, premature RV failure, and sudden death.
  
  
  RESULTS
  Fifty-five patients (aged 27±7 years) underwent LGE cardiovascular magnetic resonance and were followed for a median 7.8 (interquartile range, 3.8-9.6) years in a prospective single-center cohort study. RV LGE was present in 31 (56%) patients. The prespecified composite clinical end point comprised new-onset sustained tachyarrhythmia (atrial/ventricular) or decompensated heart failure admission/transplantation/death. Univariate predictors of the composite end point (n=22 patients; 19 atrial/2 ventricular tachyarrhythmia, 1 death) included RV LGE presence and extent, RV volumes/mass/ejection fraction, right atrial area, peak Vo(2), and age at repair. In bivariate analysis, RV LGE presence was independently associated with the composite end point (hazard ratio, 4.95 [95% confidence interval, 1.60-15.28]; P=0.005), and only percent predicted peak Vo(2) remained significantly associated with cardiac events after controlling for RV LGE (hazard ratio, 0.80 [95% confidence interval, 0.68-0.95]; P=0.009/5%). In 8 of 9 patients with >1 event, atrial tachyarrhythmia, itself a known risk factor for mortality, occurred first. There was agreement between location and extent of RV LGE at in vivo cardiovascular magnetic resonance and histologically documented focal RV fibrosis in an explanted heart. There was RV LGE progression in a different case restudied for clinical indications.
  
  
  CONCLUSIONS
  Systemic RV LGE is strongly associated with adverse clinical outcome especially arrhythmia in transposition of the great arteries, thus LGE cardiovascular magnetic resonance should be incorporated in risk stratification of these patients.
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Riikka
authors.initials: R
authors.lastname: Rydman
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Siew Yen
authors.initials: SY
authors.lastname: Ho
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Sabine
authors.initials: S
authors.lastname: Ernst
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Lorna
authors.initials: L
authors.lastname: Swan
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Wei
authors.initials: W
authors.lastname: Li
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Tom
authors.initials: T
authors.lastname: Wong
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Mary
authors.initials: M
authors.lastname: Sheppard
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Karen P
authors.initials: KP
authors.lastname: McCarthy
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Michael
authors.initials: M
authors.lastname: Roughton
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Kilner
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.).
authors.forename: Dudley J
authors.initials: DJ
authors.lastname: Pennell
authors.affiliation: From the NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital (R.R., M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., D.J.P., S.V.B.-N.), National Heart and Lung Institute (M.A.G., S.Y.H., S.E., L.S., W.L., T.W., M.S., K.P.M., P.J.K., M.R., D.J.P., S.V.B.-N.), Royal Brompton Hospital, Imperial College London, London, United Kingdom; and Section of Clinical Physiology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden (R.R.). dj.pennell@rbht.nhs.uk.
authors.forename: Sonya V
authors.initials: SV
authors.lastname: Babu-Narayan
mesh_terms: D000328:Adult; D003287:Contrast Media; D004562:Electrocardiography; D004719:Endomyocardial Fibrosis / Q000175:diagnosis* / Q000209:etiology*; D005080:Exercise Test; D005260:Female; D005682:Gadolinium; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D011446:Prospective Studies; D015996:Survival Rate; D014188:Transposition of Great Vessels / Q000150:complications* / Q000601:surgery; D016896:Treatment Outcome
chemical_list: D003287:Contrast Media; D005682:Gadolinium
keywords: arrhythmias, cardiac; fibrosis; magnetic resonance imaging; prognosis; transposition of great vessels