Division of Cardiology, Department of Pediatrics, Fetal and Neonatal Cardiology Program, Women's and Children Health Research Institute, University of Alberta, Edmonton, Alberta, Canada.
Division of Cardiology, Department of Pediatrics, University of Colorado, Denver, Colorado.
J Am Soc Echocardiogr. 2019 Dec;32(12):1598-1607. doi: 10.1016/j.echo.2019.07.008. Epub 2019 Sep 21.
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.
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.
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.
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.
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.
胎儿埃布斯坦畸形和三尖瓣发育不良（EA/TVD）与肺动脉瓣闭锁伴完整室间隔（PAIVS）相比，围产期死亡率较高，尽管两者都需要将心输出量（CO）重新分配到左心室（LV）。LV 功能障碍被怀疑是导致 EA/TVD 不良结局的原因。
我们试图检查胎儿 EA/TVD 的整体和节段性 LV 功能，并与正常对照组和 PAIVS 进行比较。我们假设 EA/TVD 中的 LV 功能障碍与 LV 重构和室间力学异常有关。
我们回顾性地确定了 2004 年至 2015 年期间遇到的 63 例胎儿 EA/TVD（40 例伴有逆行导管血流）和 22 例 PAIVS，并将这些发现与 77 例具有相似胎龄的正常对照组进行比较。我们使用二维、多普勒衍生的变形（六节段向量速度成像）和不同步指数（峰值时间的标准差，SD）以及新的整体不同步指数（DI）来测量 CO 和整体 LV 功能。
EA/TVD 胎儿的 LV 整体收缩功能异常，表现为射血分数、面积分数和 CO 降低，而在 PAIVS 中，我们观察到射血分数、面积分数和 CO 正常。PAIVS 但不是 EA/TVD 表现出 LV 球形度增加，提示存在重构，并且在第三个三个月时伴随径向功能增强。相比之下，虽然 EA/TVD 胎儿的 LV 节段性纵向应变正常，但存在异常的径向节段变形和 LV 不同步，峰值时间 SD 和 DI 增加。
胎儿 EA/TVD 与缺乏球形重构和存在机械不同步有关，这可能导致 CO 和射血分数降低。需要对胎儿 EA/TVD 的 LV 功能进行临床监测。需要进一步的研究将 LV 功能定量纳入不良结局预测模型中。
