Des Moines University, Des Moines, IA, United States.
Int J Cardiol. 2013 Aug 10;167(3):704-10. doi: 10.1016/j.ijcard.2012.03.062. Epub 2012 Mar 31.
Ebstein's anomaly involves both the right ventricle (RV) and tricuspid valve.
The functional RV and tricuspid orifice were traced from magnetic resonance images in 29 adult Ebstein patients and 9 normal subjects and reconstructed for visualization and measurement of regional RV size, function, and shape at 20 cross sections, and inlet and outflow tract ejection fractions (EFs).
The RV in Ebstein's had RV dilation (end diastolic volume index 179 ± 69 vs. 84 ± 22 ml/m(2) in normals, p<0.001) and global dysfunction (EF 45 ± 8 vs. 55 ± 5% in normals, p<0.001). Longitudinal contraction was preserved (26 ± 13 vs. 26 ± 4 mm in normals) and correlated more weakly with EF than short axis fractional shortening (r=0.44 vs. r=0.71, p<0.05 for both). The apical region in Ebstein's RV was enlarged, rounded and contributed more than normal to the global stroke volume. However this contribution correlated inversely with global EF. In contrast slices in the basal region had normal cross sectional area and their function correlated directly with global EF. Inlet EF was depressed (46 ± 8% vs. 55 ± 6 in normals, p=.002); outflow tract EF was even more depressed (39 ± 14, p=0.019).
The three dimensional shape of the RV in adult Ebstein patients was demonstrated. The Ebstein's RV remodels in diverse regional patterns rather than following a shape continuum. Changes at the apex and base had opposing effects on function. Global EF was supported more by short axis than longitudinal contraction.
Ebstein 畸形同时累及右心室（RV）和三尖瓣。
从 29 例成人 Ebstein 患者和 9 例正常对照的磁共振图像中追踪功能性 RV 和三尖瓣瓣口，重建 20 个横截面对 RV 局部大小、功能和形状的可视化和测量，以及流入道和流出道射血分数（EF）。
Ebstein 患者的 RV 扩张（舒张末期容积指数 179±69 比正常 84±22ml/m²，p<0.001）和整体功能障碍（EF 45±8 比正常 55±5%，p<0.001）。纵向收缩得以保留（26±13 比正常 26±4mm），与 EF 的相关性比短轴缩短率弱（r=0.44 比 r=0.71，两者均 p<0.05）。Ebstein RV 的心尖区域增大，变圆，对整体搏出量的贡献大于正常。然而，这种贡献与整体 EF 呈负相关。相比之下，基底区域的切片具有正常的横截面积，其功能与整体 EF 直接相关。流入道 EF 降低（46±8%比正常 55±6%，p=0.002）；流出道 EF 降低更明显（39±14，p=0.019）。
本研究展示了成人 Ebstein 患者 RV 的三维形态。Ebstein RV 的重塑呈现出多种区域性模式，而不是遵循连续的形状。心尖部和基底部的变化对功能有相反的影响。整体 EF 更多地依赖于短轴而非纵向收缩来维持。
