Ebstein’s anomaly: a multicenter analysis of 56 prenatal diagnosis cases
Authors: M.V. Medvedev , I.V. Komarova , E.V. Potolova et all  
Published: April 2019

Retrospectively, 56 cases of prenatal diagnosis of Ebstein’s anomaly (EA) were analysed. The mean gestational age at diagnosis was 24.3 weeks (range: 11+6–39 weeks). 14.8% cases were diagnosed until 14 weeks,
46.3% — before 22 weeks. The successfulness of prenatal ultrasound diagnosis of EA at 11–14 weeks of gestation is equal to 26.7%. 24 (42.8%) pregnant women opted for termination of pregnancy, intrauterine fetal death
occurred in 4 cases (7.1%), 23 (41.1%) were born alive, in 5 cases the outcomes were not available. 8 liveborn
infants were died at the neonatal age (34.8%), 2 — until 1 year (8.7%). 13 children were alive during the period
of monitoring 3 months — 9 years old (56.5%), from them 5 children were get operated (38.5%). In all cases
EA successful diagnostics was based on the detection of tricuspid valve displacement and tricuspid regurgitation. The cardiac arrhythmia was not detected. 26 cases (46.4%) were isolated without accompanying cardiac
pathology, and that was 1.5 times more frequently for alive infants, than for died. Among the accompanying
congenital heart diseases pulmonary artery obstructions were dominated — 20 cases (66.7%) and ventricular
septal defects — 14 cases (46.7%). Accompanying congenital disorders were detected at 6 cases (10.7%), among
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56 случаев пренатальной диагностики
2018 Т 17 № 4; 310-317
them — 3 cases of cystic hygroma, 2 — corpus callosum agenesis, 1 — trisomy 21. At 7 cases were detected
nonimmune hydrops (12.5%). The characteristics of cardiomegaly – cardiothoracic diameter ratio (CDR) and
cardiothoracic square ratio (CSR) — trend upward with increasing gestational age and these factors were worse
in the group of fetus died than alive. CDR and CSR as an isolated prognostic markers were effective in prediction of infant mortality with sensitivity about 100% upon  0.75 for CDR and  0.4 for CSR.