Samples Stefani, Fitt Catherine, Satzer Michael, Wakai Ronald, Strasburger Janette, Patel Sheetal
Pediatric Cardiology, Ann & Robert Lurie Children's Hospital of Chicago, Chicago, IL 60611, USA.
Department of Medical Physics, University of Wisconsin, Madison, WI 53705, USA.
Children (Basel). 2023 Jun 29;10(7):1132. doi: 10.3390/children10071132.
Congenital complete heart block (CCHB) is associated with high intrauterine and post-natal mortality. Prenatal detection and management, as well as appropriate delivery planning, may improve the outcomes in CCHB. We describe a rare case of CCHB that initially presented with fetal ascites and high-grade second-degree heart block noted on fetal echocardiography. The mother was noted to be positive for anti-SSA antibodies, and treatment with maternal steroids was started in an effort to reverse the fetal cardiac conduction abnormality. However, the fetal cardiac rhythm progressed to complete heart block by the follow up evaluation and the fetus had a continual declination of heart rate throughout the pregnancy to a low fetal heart rate of 25 beats per minute (bpm). This case demonstrates the lowest fetal ventricular rate documented in the literature and illustrates a severe presentation of a rare disease process. An overview of the existing knowledge related to etiology, prenatal evaluation with fetal echocardiography and fetal magnetocardiography, prenatal management, and delivery planning in fetuses with prenatally detected CCHB is included.
先天性完全性心脏传导阻滞(CCHB)与宫内及产后高死亡率相关。产前检测与管理以及适当的分娩计划可能会改善CCHB的预后。我们描述了一例罕见的CCHB病例,最初表现为胎儿腹水,胎儿超声心动图显示高度二度心脏传导阻滞。母亲抗SSA抗体检测呈阳性,遂开始使用母体类固醇进行治疗,试图逆转胎儿心脏传导异常。然而,在后续评估中胎儿心律进展为完全性心脏传导阻滞,且在整个孕期胎儿心率持续下降至每分钟25次的低胎儿心率。该病例展示了文献记载中最低的胎儿心室率,并说明了一种罕见疾病过程的严重表现。本文还综述了与产前诊断为CCHB的胎儿的病因、胎儿超声心动图和胎儿磁心动图的产前评估、产前管理及分娩计划相关的现有知识。