BACKGROUND Fetal Ebstein's anomaly and tricuspid valve dysplasia (EA/TVD) are associated with high perinatal mortality relative to pulmonary atresia with intact ventricular septum (PAIVS), despite both requiring redistribution of the cardiac output (CO) to the left ventricle (LV). LV dysfunction is suspected to contribute to adverse outcomes in EA/TVD. OBJECTIVE We sought to examine global and segmental LV function in fetal EA/TVD with comparison to normal controls and PAIVS. We hypothesized that LV dysfunction in EA/TVD is associated with abnormal LV remodeling and interventricular mechanics. METHODS We retrospectively identified 63 cases of fetal EA/TVD (40 with retrograde ductal flow) and 22 cases of PAIVS encountered from 2004 to 2015 and compared findings to 77 controls of comparable gestational age. We measured the combined CO and global LV function using two-dimensional, Doppler-derived, deformational (six-segmental vector velocity imaging) and dyssynchrony indices (DIs; SD of time to peak), and a novel global DI. RESULTS EA/TVD fetuses demonstrated abnormal LV global systolic function with reduced ejection fraction, fractional area change, and CO, while in PAIVS we observed a normal ejection fraction, fractional area change, and CO. PAIVS, but not EA/TVD, demonstrated increased LV sphericity, suggestive of remodeling, and associated enhanced radial function in the third trimester. In contrast, while EA/TVD fetuses had normal LV segmental longitudinal strain, there was abnormal radial segmental deformation and LV dyssynchrony with increased SD of time to peak and DI. CONCLUSIONS Fetal EA/TVD is associated with a lack of spherical remodeling and presence of mechanical dyssynchrony, which likely contribute to reduced CO and ejection fraction. Clinical monitoring of LV function is warranted in fetal EA/TVD. Further studies incorporating quantification of LV function into prediction models for adverse outcomes are required.

中文翻译：

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胎儿Ebstein异常和三尖瓣不典型增生中左心室功能障碍的定量评估。

背景相对于具有完整心室间隔的肺动脉闭锁（PAIVS），尽管胎儿Ebstein异常和三尖瓣不典型增生（EA / TVD）与高围产期死亡率相关，尽管两者都需要将心输出量（CO）重新分配至左心室（LV）。左室功能障碍被怀疑是导致EA / TVD不良结果的原因。目的我们试图检查胎儿EA / TVD中的整体和分段LV功能，并与正常对照和PAIVS进行比较。我们假设EA / TVD中的LV功能障碍与异常的LV重塑和心室力学有关。方法我们回顾性分析了2004年至2015年间63例胎儿EA / TVD（40例逆行导管血流）和22例PAIVS，并将调查结果与77个可比较胎龄的对照组进行了比较。我们使用二维多普勒衍生，变形（六段矢量速度成像）和不同步指数（DI；达到峰值的时间的标准差）以及新颖的全局DI来测量组合的CO和全局LV函数。结果EA / TVD胎儿表现出左室总体收缩功能异常，射血分数，分数面积变化和CO降低，而PAIVS则观察到正常的射血分数，分数面积变化和CO。左半球提示重塑，并在妊娠中期增强了径向功能。相比之下，虽然EA / TVD胎儿的LV节段纵向应变正常，但出现异常的径向节段变形和LV不同步，并且达到峰值的时间SD和DI增加。结论胎儿EA / TVD与缺乏球形重塑和机械不同步性有关，这可能有助于降低CO和射血分数。胎儿EA / TVD中需要对LV功能进行临床监测。需要进一步的研究，将LV功能的量化纳入不良结果的预测模型中。