Prenatal and postnatal ultrasound assessment and clinical prognostic analysis for Ebstein anomaly

Abstract

Objective: To investigate the echocardiographic features of fetal Ebstein's anomaly (EA) and to analyze its clinical outcome and prognosis. Methods: A retrospective case-control study was conducted to analyze the echocardiographic features in fetus with EA. Thirty-five EA fetuses (EA group) and 35 normal fetuses matched for gestational age (control group) were enrolled. The main echocardiographic parameters of the two groups were collected and compared. According to the direction of blood flow in the ductus arteriosus (DA),fetuses in EA group were divided into DA reverse perfusion subgroup (n=11) and normal DA blood flow subgroup (n=24). The echocardiographic parameters and GOSE scores were compared between the two subgroups. The echocardiographic features of EA and the difference of fetal hemodynamics were summarized,and the clinical outcome of EA fetus was evaluated by GOSE score. Chi-square test Rank sum test or t test were used for comparison between groups. Results: Nineteen (54%) of the 35 patients terminated the pregnancy and 16 (46%) continued pregnancy until delivery during follow-up. Compared with the normal fetus group,the cardiothoracic ratio was significantly higher (0.47±0.11 vs. 0.34±0.01, t=6.640, P<0.01) and the transverse diameter ratio of right atrium to left atrium was significantly greater (1.42±0.38 vs. 1.08±0.11, t=5.030, P<0.01) in the EA group, and the ratio of pulmonary artery diameter to aortic diameter was significantly lower in the EA group (1.04±0.21 vs. 1.20±0.15, t=-3.770, P<0.01). Compared with the normal DA blood flow subgroup,the GOSE scores ≥ 1.5 was more frequently seen (7/11 vs. 3/24, P=0.004) and the ratio of pulmonary artery diameter to aortic diameter was significantly lower (0.91±0.18 vs. 1.24±0.20, t=-4.696, P=0.002) in the DA reverse perfusion subgroup. Six of the 16 delivery cases underwent EA corrective surgery after birth with 100% successful rate of operation. Among the 6 cases,two had preoperative GOSE score of ≥1.5 who were considered as severe cases,and four had low GOSE score of<1.5. The remaining ten cases who had not undergone the corrective surgery were followed up routinely. Conclusion: Prenatal hemodynamics of EA combined with GOSE score can more accurately evaluate the severity and prognosis of fetal EA, reduce unnecessary labor induction, and improve postpartum cure rate and clinical outcomes.