Selamet Tierney Elif Seda, McElhinney Doff B, Freud Lindsay R, Tworetzky Wayne, Cuneo Bettina F, Escobar-Diaz Maria C, Ikemba Catherine, Kalish Brian T, Komarlu Rukmini, Levasseur Stéphanie M, Puchalski Michael D, Satou Gary M, Silverman Norman H, Moon-Grady Anita J
Division of Cardiology, Department of Pediatrics, Stanford University, Palo Alto, California.
Division of Cardiology, Department of Pediatrics, Stanford University, Palo Alto, California.
Am J Cardiol. 2017 Jan 1;119(1):106-111. doi: 10.1016/j.amjcard.2016.09.022. Epub 2016 Sep 29.
In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.
在患有埃布斯坦畸形或三尖瓣发育异常（EA/TVD）的胎儿中，血流动力学状态不佳与新生儿预后较差相关。目前尚不清楚在妊娠早期生理状态更有利的EA/TVD胎儿在妊娠晚期是否会发展为更严重的疾病。我们评估了妊娠<24周的EA/TVD胎儿的超声心动图指标是否是妊娠后期生理状态的可靠指标。这项多中心回顾性研究纳入了51例妊娠<24周的EA/TVD胎儿，并进行了间隔≥4周的系列胎儿超声心动图检查。我们将以下情况指定为预后不良的标志物：肺动脉瓣无顺行血流、肺动脉瓣反流、心胸面积比>0.48、左心室（LV）功能障碍或三尖瓣（TV）瓣环Z评分>5.6。诊断时的中位孕周为21周（范围18至24周）。18例胎儿（35%）最初没有血流动力学状态不佳的标志物，而其中只有7例在妊娠晚期继续没有预后不良的标志物。首次超声心动图检查显示有顺行肺血流的27例胎儿中有9例（33%）发生了肺动脉闭锁；39例中有7例（18%）出现了新的肺动脉瓣反流。2例（4%）患者在<24周时出现LV功能障碍，但后来有14例（37%）出现（p<0.001）。从诊断到随访，TV瓣环Z评分和心胸面积均增加。总之，该队列中血流动力学逐渐受损很常见。我们的研究强调，在24周前进行咨询时必须谨慎，因为妊娠早期缺乏与预后不良相关的因素可能会给出错误的安慰信息。
