BCNatal Fetal Medicine Research Center, Hospital Clínic and Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain.
Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Fetal Diagn Ther. 2020;47(8):604-614. doi: 10.1159/000504979. Epub 2020 Feb 4.
Firstly, to describe the outcome of a series of fetuses with Ebstein's anomaly (EA) and, secondly, to study the utility of different second-trimester echocardiographic parameters to predict fetal and neonatal mortality.
39 fetuses with EA diagnosed between 18 and 28 weeks of gestation were included. Fetal echocardiography included the cardiothoracic ratio (CTR); right atrial (RA) area index; displacement of the tricuspid valve (TV); tricuspid regurgitation; pulmonary artery; and ductus arteriosus flow characteristics. Additionally, 2 novel parameters were obtained: the relative RA area ratio (RA area/cardiac area) and the TV displacement index (TVDI, TV displacement distance/longi-tudinal diameter of the left ventricle). Correlation between the echocardiographic variables and the primary outcome of perinatal mortality or survival at 1 year of life was evaluated.
From the initial cohort, 8 cases were excluded due to complex congenital heart defects. Termination of pregnancy (TOP) was performed in 15 cases, and fetal death was diagnosed in 3 cases. In the live-born cohort of 13 patients, 4 died in the neonatal period, yielding a perinatal survival rate of 29 and 56%, respectively, after excluding TOP cases. Compared with survivors, nonsurvivors showed a significantly higher CTR (56.7 ± 16.2 vs. 42.6 ± 8.6; p = 0.04), relative RA area ratio (0.39 ± 0.13 vs. 0.25 ± 0.05; p = 0.01), and TVDI (0.62 ± 0.17 vs. 0.44 ± 0.12; p = 0.03) at diagnosis. The best model to predict perinatal mortality was obtained by using a scoring system which included the relative RA area ratio and TVDI (AUC 0.905 [95% CI 0.732-1.000]).
Fetuses with a relative RA area ratio ≥0.29 and TVDI ≥0.65 at the second trimester have the highest risk of dying in the perinatal stage.
首先，描述一系列埃布斯坦畸形（EA）胎儿的结局；其次，研究不同的中期超声心动图参数在预测胎儿和新生儿死亡率方面的作用。
共纳入 39 例 18-28 周妊娠期间被诊断为 EA 的胎儿。胎儿超声心动图包括心胸比（CTR）；右心房（RA）面积指数；三尖瓣（TV）位移；三尖瓣反流；肺动脉；以及动脉导管血流特征。此外，还获得了 2 个新参数：相对 RA 面积比（RA 面积/心脏面积）和 TV 位移指数（TVDI，TV 位移距离/左心室长径）。评估超声心动图变量与围产期死亡率或 1 年生存率的主要结局之间的相关性。
从最初的队列中，因复杂先天性心脏病排除了 8 例。15 例病例行终止妊娠（TOP），3 例胎儿死亡。在 13 例存活出生的患儿中，4 例新生儿期死亡，排除 TOP 病例后，围产儿存活率分别为 29%和 56%。与存活者相比，非存活者的 CTR（56.7 ± 16.2 与 42.6 ± 8.6；p = 0.04）、相对 RA 面积比（0.39 ± 0.13 与 0.25 ± 0.05；p = 0.01）和 TVDI（0.62 ± 0.17 与 0.44 ± 0.12；p = 0.03）在诊断时显著更高。预测围产期死亡率的最佳模型是使用一种评分系统，该系统包括相对 RA 面积比和 TVDI（AUC 0.905 [95% CI 0.732-1.000]）。
妊娠中期相对 RA 面积比≥0.29 和 TVDI≥0.65 的胎儿在围产期死亡风险最高。
