Abstract
Due to the increased number of prenatal screenings, the number of cases diagnosed as Ebstein anomaly has been increasing. However, Ebstein anomaly remains a condition associated with a poor prognosis. The purpose of this study is to clarify prognostic factors of fetal Ebstein anomaly diagnosed by prenatal screening, which may provide information for improved counseling. Fifty-six fetuses with an antenatal diagnosis of Ebstein anomaly were included in the study. At screening, the gestational age was 20 to 39 weeks (median: 34). Outcomes of the fetuses were abortion in 14, fetal death in 14, neonatal death in 12, and live birth in 20. The following complications were observed: hydrops fetalis (n = 13), chromosome aberration (n = 2), and myocardial anomaly (n = 10). We assigned 42 cases excluding 14 abortions to fetal or neonatal death (D group; n = 22) and live birth (S group; n = 20) groups to compare fetal echocardiography findings and outcomes. We compared non-cardiac complications, as well as the parameters of fetal echocardiography findings including the morphology of the pulmonary valve (presence/absence of anterograde flow), cardio-thoracic area ratio (CTAR), maximal TR flow (TR), and left-ventricular (LV) Tei index between the two groups. The cut-off values for survival were calculated from receiver operating characteristic (ROC) curve analysis. The following results were obtained (D vs. S): complication rate, 65 vs. 19% (p = 0.002); morphology of pulmonary valve (presence of anterograde flow), 17 vs. 82% (p < 0.0001); CTAR, 63 ± 11 vs. 50 ± 9% (p = 0.002); TR, 2.0 ± 0.4 vs. 3.3 ± 0.5 m/s (p < 0.0001); LV Tei, 1.0 ± 0.3 vs. 0.6 ± 0.2 (p < 0.0001). Cut-off values were CTAR, 56% (sensitivity, 62%; specificity, 80%), TR, 2.7 m/s (sensitivity, 94%; specificity, 99%), and LV Tei, 0.73 (sensitivity, 82%; specificity, 90%). The right ventricular function evaluated by TR and morphology of the pulmonary valve can be used to predict the outcome of fetal Ebstein anomaly.
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