Riikka Rydman1, Michael A Gatzoulis1, Siew Yen Ho1, Sabine Ernst1, Lorna Swan1, Wei Li1, Tom Wong1, Mary Sheppard1, Karen P McCarthy1, Michael Roughton1, Philip J Kilner1, Dudley J Pennell1, Sonya V Babu-Narayan2. 1. 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.). 2. 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.
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. METHODS AND 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.
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. METHODS AND 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.
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