三尖瓣下移畸形产前及生后超声评估与临床预后分析

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

目的探讨胎儿三尖瓣下移畸形(EA)的超声图像特征,分析其临床转归及预后。方法病例对照研究。以经胎儿超声心动图诊断为EA的35例胎儿为EA组,以孕周相匹配的正常胎儿35例为对照组,比较两组患儿的主要超声参数差异。根据动脉导管血流方向分为逆灌组(11例)和正向组(24例),比较两亚组主要超声参数和GOSE评分值的差异。总结EA的超声心动图特征和胎儿期血流动力学差异,并以GOSE评分评价EA胎儿的临床结局。组间比较采用t检验、秩和检验或χ^2检验。结果随访35例中,19例(54%)选择终止妊娠,16例(46%)继续妊娠至分娩。EA组胎儿心胸面积比值(0.47±0.11比0.34±0.01,t=6.640,P<0.01),右心房与左心房内径比值(1.42±0.38比1.08±0.11,t=5.030,P<0.01)明显高于对照组,差异均有统计学意义。EA组肺动脉内径与主动脉内径之比明显低于对照组,差异有统计学意义(1.04±0.21比1.20±0.15,t=-3.770,P<0.01)。11例动脉导管血流逆行灌注的EA胎儿有7例GOSE≥1.5,明显高于动脉导管血流正向组重症比例(3/24),差异有统计学意义(P=0.004)。逆灌组肺动脉内径与主动脉内径比值明显低于正向组(0.91±0.18比1.24±0.20,t=-4.696,P=0.002)。16例出生患儿中,6例在生后行EA矫治术,手术均成功,其中2例经术前再评估GOSE评分≥1.5(重症),4例GOSE评分<1.5,余10例未手术者定期随访中。结论EA的产前血流动力学特征结合GOSE评分,能更准确地评价胎儿EA的严重程度和预后,减少盲目引产,提高产后治愈率,并改善患者临床结局。

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.