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.).
Circ Cardiovasc Imaging. 2015 May;8(5). doi: 10.1161/CIRCIMAGING.114.002628.
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.
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.
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.
我们假设，晚期钆增强（LGE）心血管磁共振检测到的纤维化可预测大动脉转位（TGA）经房间隔转向术后患者的结局。这些患者有一个系统性右心室（RV），并存在心律失常、RV 过早衰竭和猝死的风险。
55 例患者（年龄 27±7 岁）接受了 LGE 心血管磁共振检查，并在一项前瞻性单中心队列研究中进行了中位数为 7.8（四分位间距 3.8-9.6）年的随访。31 例（56%）患者存在 RV LGE。复合临床终点包括新发持续性心动过速（房性/室性）或失代偿性心力衰竭入院/移植/死亡。复合终点的单变量预测因素（n=22 例患者；19 例房性/2 例室性心动过速，1 例死亡）包括 RV LGE 存在和程度、RV 容积/质量/射血分数、右心房面积、峰值 Vo（2）和修复时的年龄。在二元分析中，RV LGE 存在与复合终点独立相关（风险比，4.95[95%置信区间，1.60-15.28]；P=0.005），而仅 RV LGE 后预测峰值 Vo（2）的百分比与心脏事件显著相关（风险比，0.80[95%置信区间，0.68-0.95]；P=0.009/5%）。在 9 例有>1 次事件的患者中，8 例患者首先发生已知与死亡率相关的房性心动过速。在活体心血管磁共振和离体心脏组织学检查中记录的 RV 局灶性纤维化中，RV LGE 的位置和程度存在一致性。在因临床指征重新研究的不同病例中，出现了 RV LGE 的进展。
系统性 RV LGE 与不良临床结局（尤其是 TGA 中的心律失常）密切相关，因此 LGE 心血管磁共振应纳入这些患者的风险分层。
