{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T13:33:48Z","timestamp":1782567228067,"version":"3.54.5"},"reference-count":20,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2023,6,29]],"date-time":"2023-06-29T00:00:00Z","timestamp":1687996800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3390\/children10071132","type":"journal-article","created":{"date-parts":[[2023,6,30]],"date-time":"2023-06-30T00:43:30Z","timestamp":1688085810000},"page":"1132","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Fetal Congenital Complete Heart Block: A Rare Case with an Extremely Low Ventricular Rate and Review of Current Management Strategies"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2906-0651","authenticated-orcid":false,"given":"Stefani","family":"Samples","sequence":"first","affiliation":[{"name":"Pediatric Cardiology, Ann & Robert Lurie Children\u2019s Hospital of Chicago, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Fitt","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology, Ann & Robert Lurie Children\u2019s Hospital of Chicago, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Satzer","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology, Ann & Robert Lurie Children\u2019s Hospital of Chicago, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9894-0484","authenticated-orcid":false,"given":"Ronald","family":"Wakai","sequence":"additional","affiliation":[{"name":"Department of Medical Physics, University of Wisconsin, Madison, WI 53705, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6986-2544","authenticated-orcid":false,"given":"Janette","family":"Strasburger","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology, Children\u2019s Hospital of Wisconsin, Milwaukee, WI 53226, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4257-1103","authenticated-orcid":false,"given":"Sheetal","family":"Patel","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology, Ann & Robert Lurie Children\u2019s Hospital of Chicago, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,6,29]]},"reference":[{"key":"ref_1","first-page":"85","article-title":"Congenital complete heart block: An international study of the natural history","volume":"4","author":"Engle","year":"1972","journal-title":"Cardiovasc. Clin."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1016\/j.clp.2005.09.011","article-title":"Prenatal Diagnosis of Fetal Arrhythmias","volume":"32","author":"Strasburger","year":"2005","journal-title":"Clin. Perinatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.jacc.2007.06.060","article-title":"Electrophysiological Characteristics of Fetal Atrioventricular Block","volume":"51","author":"Zhao","year":"2008","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1016\/j.amjcard.2007.03.081","article-title":"Atrial and ventricular rate response and patterns of heart rate acceleration during maternal\u2013fetal terbutaline treatment of fetal complete heart block","volume":"100","author":"Cuneo","year":"2007","journal-title":"Am. J. Cardiol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1016\/j.hrthm.2012.12.030","article-title":"Pathophysiology, clinical course, and management of congenital complete atrioventricular block","volume":"10","author":"Bordachar","year":"2013","journal-title":"Heart Rhythm."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1097\/GRF.0b013e3181fbb747","article-title":"Doppler echocardiography for managing fetal cardiac arrhythmia","volume":"53","author":"Matta","year":"2010","journal-title":"Clin. Obstet. Gynecol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1542\/peds.106.1.86","article-title":"Short- and long-term outcome of children with congenital complete heart block diagnosed in utero or as a newborn","volume":"106","author":"Eronen","year":"2000","journal-title":"Pediatrics"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1007\/s00431-021-04293-8","article-title":"Cohort study of congenital complete heart block among preterm neonates: A single-center experience over a 15-year period","volume":"181","author":"Hernstadt","year":"2021","journal-title":"Eur. J. Pediatr."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1007\/s002460010256","article-title":"Congenital Complete Heart Block in the Fetus: Hemodynamic Features, Antenatal Treatment, and Outcome in Six Cases","volume":"22","author":"Eronen","year":"2001","journal-title":"Pediatr. Cardiol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1002\/uog.22","article-title":"Successful outcome in a fetus with an extremely low heart rate due to isolated complete congenital heart block","volume":"21","author":"Vesel","year":"2003","journal-title":"Ultrasound Obstet. Gynecol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1002\/bdr2.1459","article-title":"Prenatal diagnosis and management of congenital complete heart block","volume":"111","author":"Pruetz","year":"2019","journal-title":"Birth Defects Res."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1055\/s-0034-1372430","article-title":"Diagnosis and Treatment of Fetal Arrhythmia","volume":"31","author":"Cuneo","year":"2014","journal-title":"Am. J. Perinatol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"S53","DOI":"10.1002\/jcph.2129","article-title":"Fetal Arrhythmia Diagnosis and Pharmacologic Management","volume":"62","author":"Strasburger","year":"2022","journal-title":"J. Clin. Pharmacol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"e025224","DOI":"10.1161\/JAHA.121.025224","article-title":"Contribution of Fetal Magnetocardiography to Diagnosis, Risk Assessment, and Treatment of Fetal Arrhythmia","volume":"11","author":"Strasburger","year":"2022","journal-title":"J. Am. Heart Assoc."},{"key":"ref_15","first-page":"957","article-title":"Ascites not due to congestive heart failure in a fetus with lupus-induced heart block","volume":"76 Pt 2","author":"Richards","year":"1990","journal-title":"Obstet. Gynecol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1111\/aogs.13338","article-title":"Maternal steroid therapy for fetuses with second-degree immune-mediated congenital atrioventricular block: A systematic review and meta-analysis","volume":"97","author":"Ciardulli","year":"2018","journal-title":"Acta Obstet. et Gynecol. Scand."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1016\/j.ajog.2016.07.002","article-title":"Detection and successful treatment of emergent anti-SSA mediated fetal atrioventricular block","volume":"215","author":"Cuneo","year":"2016","journal-title":"Am. J. Obstet. Gynecol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1055\/s-0038-1637764","article-title":"Predicting the Future: Delivery Room Planning of Congenital Heart Disease Diagnosed by Fetal Echocardiography","volume":"35","author":"Donofrio","year":"2018","journal-title":"Am. J. Perinatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1965","DOI":"10.3109\/14767058.2014.974536","article-title":"Prediction of fetal outcome without intrauterine intervention using a cardiovascular profile score: A systematic review and meta-analysis","volume":"28","author":"Li","year":"2015","journal-title":"J. Matern. Neonatal Med."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1038\/sj.jp.7211038","article-title":"Congenital complete heart block: Fetal management protocol, review of the literature, and report of the smallest successful pacemaker implantation","volume":"24","author":"Donofrio","year":"2004","journal-title":"J. Perinatol."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/10\/7\/1132\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T20:03:15Z","timestamp":1760126595000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/10\/7\/1132"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,6,29]]},"references-count":20,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2023,7]]}},"alternative-id":["children10071132"],"URL":"https:\/\/doi.org\/10.3390\/children10071132","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,6,29]]}}}