4.2 Article

Prediction of Perinatal Mortality in Ebstein's Anomaly Diagnosed in the Second Trimester of Pregnancy

期刊

FETAL DIAGNOSIS AND THERAPY
卷 47, 期 8, 页码 604-614

出版社

KARGER
DOI: 10.1159/000504979

关键词

Congenital heart defects; Ebstein's anomaly; Tricuspid valve anomaly; Fetal echocardiography; Prenatal diagnosis

资金

  1. Hospital Clinic de Barcelona (Ajut Josep Font, Barcelona, Spain)
  2. la Caixa Foundation [LCF/PR/GN14/10270005]
  3. Instituto de Salud Carlos III [PI14/00226, PI15/00263, PI17/00675]
  4. ISCIII-Subdireccion General de Evaluacion y el Fondo Europeo de Desarrollo Regional (FEDER) Una manera de hacer Europa
  5. Cerebra Foundation for the Brain Injured Child (Carmarthen, Wales, UK)
  6. Cellex Foundation
  7. AGAUR 2017 SGR [1531]

向作者/读者索取更多资源

Objectives: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.Methods: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.Results: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]).Conclusions: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.

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