Division of Cardiology, Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network and University of Toronto, Toronto, Ontario, Canada.
Int J Cardiol. 2013 Apr 30;165(1):146-50. doi: 10.1016/j.ijcard.2011.08.004. Epub 2011 Aug 27.
The utility of cardiac magnetic resonance imaging (CMR) for assessment of adults with Ebstein anomaly is not well-defined. We sought to evaluate CMR characteristics in this population and to relate these to exercise parameters.
We analyzed CMR studies in adults with unrepaired Ebstein anomaly for measures of severity of Ebstein disease, including atrialized, functional and total right ventricular (RV) volumes, ejection fraction (EF) and severity index (area of atrialized RV+right atrium/functional RV+left ventricle+left atrium). We related these CMR values to cardiopulmonary exercise test measurements.
Twenty-seven adults (mean age 41 ± 14 years, 70% female) were included. Functional RV end-diastolic volume (EDV) was 150 ± 68 mL/m(2) and atrialized RVEDV was 25 ± 24 mL/m(2). In 17 patients (63%), the functional RVEDV was enlarged (>114 mL/m(2)). Percent predicted peak VO2 for the population was 65 ± 20%. On univariable analysis, peak VO2 was inversely related to atrialized RVEDV (p = 0.011), total RVEDV (p = 0.041), functional RVEDV/left ventricular EDV ratio (p = 0.015) and magnitude of tricuspid valve displacement (p = 0.031). In the multivariate model, the only CMR factor to relate to peak VO2 was atrialized RVEDV (p = 0.011, β = -0.48). No significant correlations were found between CMR measures and heart rate response or ventilatory response to exercise.
In adults with unrepaired Ebstein anomaly, atrialized RV volume was independently related to aerobic capacity. The volume of the atrialized RV is a novel CMR measure which may express severity of disease. Further research is needed to evaluate the prognostic relevance of this exploratory work.
心脏磁共振成像（CMR）在评估成人埃布斯坦畸形中的应用尚未明确。我们旨在评估该人群的 CMR 特征，并将其与运动参数相关联。
我们分析了未经修复的埃布斯坦畸形成人的 CMR 研究，以评估埃布斯坦病的严重程度，包括心房化、功能性和总右心室（RV）容积、射血分数（EF）和严重指数（心房化 RV+右心房/功能性 RV+左心室+左心房面积）。我们将这些 CMR 值与心肺运动试验测量值相关联。
共纳入 27 例成人（平均年龄 41 ± 14 岁，70%为女性）。功能性 RV 舒张末期容积（EDV）为 150 ± 68 mL/m²，心房化 RVEDV 为 25 ± 24 mL/m²。在 17 例患者（63%）中，功能性 RVEDV 增大（>114 mL/m²）。该人群的预测峰值 VO2 百分比为 65 ± 20%。单变量分析显示，峰值 VO2 与心房化 RVEDV（p = 0.011）、总 RVEDV（p = 0.041）、功能性 RVEDV/左心室 EDV 比值（p = 0.015）和三尖瓣瓣叶位移程度（p = 0.031）呈负相关。多变量模型中，与峰值 VO2 相关的唯一 CMR 因素是心房化 RVEDV（p = 0.011，β = -0.48）。CMR 指标与心率或运动时的通气反应之间无显著相关性。
在未经修复的埃布斯坦畸形成人中，心房化 RV 容积与有氧运动能力独立相关。心房化 RV 容积是一种新的 CMR 测量方法，可能反映疾病的严重程度。需要进一步研究来评估这项探索性工作的预后相关性。
