Abstract：
This study aimed to analyze prenatal cardiac ultrasound markers of outcome in fetuses with Ebstein\'s anomaly (EA). From a retrospective database, 35 fetuses diagnosed with EA at fetal medicine centers in Brazil, Italy, and Poland were retrieved. The primary outcome was perinatal mortality. We analyzed prenatal cardiac ultrasound markers of outcomes and perinatal follow-up. Gestational age at diagnosis, extracardiac fetal anomalies, spontaneous fetal demise, and gestational age at each event were recorded. In postnatal survivors, data on cardiac surgery and short-term postoperative outcomes were collected. Our study included a cohort of 35 fetuses with EA (mean gestational age of 29.4 weeks), in which 6 fetuses were excluded due to termination of pregnancy (3), pregnancy still ongoing (2), and missed follow-up (1). Of the remaining 29 cases, severe tricuspid regurgitation and absence of anterograde pulmonary flow (pulmonary atresia) were observed in 88%. Significant cardiomegaly accounts for 58% of these data with a mean cardiothoracic ratio of 0.59. The cardiovascular profile (CVS) score ≤ 6 in six patients with one survival (4 fetal deaths, one stillbirth, and one survival). All fetuses with CVS score of 5 had intrauterine demise. Seventeen fetuses were born alive (53.1% of 29 cases). Of the remaining fetuses, one (1%) fetal was a stillbirth, six (20%) fetuses were neonatal deaths, and five (17%) fetuses were fetal deaths. Of the nineteen patients who underwent surgery to correct the cardiac defect, 17 survived after surgery. Among the survivors, biventricular cardiac repair was performed using the cone technique (da Silva\'s approach) in the majority of cases. We observed 2 abnormal karyotypes among in the remaining 29 fetuses. One of the patients with abnormal karyotype was a fetus with ascites and large for gestational age. The other patient with abnormal karyotype underwent cardiac surgery and progressed to neonatal death. Nine patients (25%) had extracardiac anomalies (genitourinary anomalies and single umbilical artery), being that 2 of them are alive and 4 died (2 had fetal and 2 neonatal death). Fetal EA is associated with high mortality. The most common prenatal marker associated with non-survival was CVP score ≤ 6. Fetuses that survived and underwent postnatal corrective surgery are significantly favorable outcomes.
摘要：
本研究旨在分析Ebstein异常（EA）胎儿的产前心脏超声指标。从回顾性数据库中，在巴西的胎儿医学中心诊断为EA的35个胎儿，意大利,波兰被找回。主要结局是围产期死亡率。我们分析了产前心脏超声指标的结局和围产期随访。诊断时的妊娠年龄，胎儿心外畸形,胎儿自发死亡,并记录每个事件的胎龄.在产后幸存者中，收集有关心脏手术和术后短期结局的数据.我们的研究包括35例EA胎儿（平均胎龄29.4周），其中6个胎儿因终止妊娠而被排除在外（3），怀孕仍在进行中（2），错过随访（1）。其余29例,88%的患者观察到严重的三尖瓣反流和不存在顺行性肺血流(肺动脉闭锁).显著的心脏肥大占这些数据的58％，平均心胸比率为0.59。6例存活1例(4例胎儿死亡,一次死产,和一个生存）。所有CVS评分为5的胎儿宫内死亡。17例胎儿存活（29例，占53.1%）。剩下的胎儿中,一个（1%）胎儿是死胎，六个（20％）胎儿是新生儿死亡，5例(17%)胎儿为胎儿死亡。在19名接受手术纠正心脏缺陷的患者中，手术后17人存活。在幸存者中,在大多数情况下，使用锥形技术（达席尔瓦入路）进行双心室心脏修复。我们在其余29个胎儿中观察到2个异常核型。核型异常的患者之一是腹水胎儿，胎龄较大。另一名核型异常的患者接受了心脏手术，并进展为新生儿死亡。9例患者（25%）心外异常（泌尿生殖系统异常和单脐动脉），其中2人还活着，4人死亡（2人胎儿死亡，2人新生儿死亡）。胎儿EA与高死亡率相关。与非生存相关的最常见的产前标志物是CVP评分≤6。存活并接受产后矫正手术的胎儿是显着有利的结果。
