Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, USA.
Cohen Children's Medical Center, Zucker School of Medicine at Hofstra/Northwell, New York, USA.
Pediatr Cardiol. 2023 Aug;44(6):1397-1405. doi: 10.1007/s00246-023-03099-9. Epub 2023 Feb 28.
Truncus arteriosus (TA) is a rare congenital heart defect that can be prenatally detected by fetal echocardiography. However, prognostication and prenatal counseling focus primarily on surgical outcomes due to limited fetal and neonatal pre-surgical mortality data. We aimed to describe the incidence and identify predictors of pre-surgical mortality in prenatally detected TA. This two-center, retrospective cohort study included fetuses diagnosed with TA between 01/2010 and 04/2020. The primary outcome was pre-surgical mortality, defined by fetal or neonatal pre-surgical death or primary listing for transplantation prior to discharge. Univariable regression modeling, Chi-square tests, and t tests assessed for associations between prenatal clinical, demographic, and fetal echocardiographic (fetal-echo) variables and pre-surgical mortality. Of 23 pregnancies with prenatal diagnosis of TA, 4 (17%) were terminated. Of the remaining 19, pre-surgical mortality occurred in 4 (26%), including 2 (11%) fetal deaths and 2 (11%) neonatal pre-surgical deaths. No transplantation listings. Of liveborn fetuses (n = 17), 15 (88%) underwent a neonatal surgery, and 1 (6%) required ECMO. As compared to the survivors, the pre-surgical mortality group had a higher likelihood of having left ventricular dysfunction (0% vs. 40%; p = 0.01), right ventricular dysfunction (0% vs. 60%; p = 0.002), cardiovascular profile score < 7 (0% vs. 40%; p = 0.01), skin edema (0% vs. 40%; p = 0.01), and abnormal umbilical venous (UV) Doppler (0% vs. 60%; p = 0.002). The presence of truncal valve regurgitation or stenosis neared significance. In this cohort with prenatally diagnosed TA, there is significant pre-surgical mortality, including fetal death and neonatal pre-surgical death. Termination rate is also high. Fetal-echo variables associated with pre-surgical mortality in this cohort include ventricular dysfunction, low CVP, skin edema, and abnormal UV Doppler. Knowledge about prenatal risk factors for pre-surgical mortality may guide parental counseling and postnatal planning in prenatally diagnosed TA.
主脉干（TA）是一种罕见的先天性心脏病，可通过胎儿超声心动图在产前发现。然而，由于胎儿和新生儿围手术期死亡率数据有限，预后和产前咨询主要侧重于手术结果。我们旨在描述产前发现的 TA 围手术期死亡率的发生率，并确定其预测因素。这是一项在两个中心进行的回顾性队列研究，纳入了 2010 年 1 月至 2020 年 4 月期间被诊断为 TA 的胎儿。主要结局是围手术期死亡率，定义为胎儿或新生儿围手术期死亡或在出院前首次被列入移植名单。单变量回归模型、卡方检验和 t 检验评估了产前临床、人口统计学和胎儿超声心动图（胎儿超声心动图）变量与围手术期死亡率之间的关系。在 23 例产前诊断为 TA 的妊娠中，有 4 例（17%）终止妊娠。在其余 19 例中，有 4 例（26%）发生围手术期死亡，包括 2 例（11%）胎儿死亡和 2 例（11%）新生儿围手术期死亡。无移植名单。在存活的胎儿（n=17）中，有 15 例（88%）接受了新生儿手术，有 1 例（6%）需要 ECMO。与幸存者相比，围手术期死亡率组左心室功能障碍的可能性更高（0% vs. 40%；p=0.01），右心室功能障碍的可能性更高（0% vs. 60%；p=0.002），心血管状况评分<7 的可能性更高（0% vs. 40%；p=0.01），皮肤水肿的可能性更高（0% vs. 40%；p=0.01），脐静脉（UV）多普勒异常的可能性更高（0% vs. 60%；p=0.002）。三尖瓣反流或狭窄的存在接近显著水平。在本队列中，有 40%（4/10）的胎儿在产前诊断为 TA 后出现围手术期死亡，包括胎儿死亡和新生儿围手术期死亡。终止妊娠率也很高。与本队列中围手术期死亡率相关的胎儿超声心动图变量包括心室功能障碍、低 CVP、皮肤水肿和异常的 UV 多普勒。了解产前围手术期死亡率的危险因素可能有助于对产前诊断的 TA 患者进行父母咨询和产后规划。
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