Benefits of fetal echocardiographic surveillance in pregnancies at risk of congenital heart block: single‐center study of 212 anti‐Ro52‐positive pregnancies

• Published in: Ultrasound in obstetrics & gynecology Vol. 54; no. 1; pp. 87 - 95
• Main Authors: Sonesson, S.‐E., Ambrosi, A., Wahren‐Herlenius, M.
• Format: Journal Article
• Language: English
• Published: Chichester, UK John Wiley & Sons, Ltd 01-07-2019; Wiley Subscription Services, Inc
• Subjects: Antibodies; atrioventricular block; Atrioventricular Block - classification; Atrioventricular Block - epidemiology; Atrioventricular Block - physiopathology; Atrioventricular Block - prevention & control; Autoantibodies; Autoimmune Diseases - blood; Autoimmune Diseases - drug therapy; Betamethasone - administration & dosage; Betamethasone - therapeutic use; Cardiology; Conduction; Congenital heart block; Doppler; echocardiography; Echocardiography - methods; Echocardiography, Doppler - methods; EKG; Electrocardiography; Female; Fetal Heart - diagnostic imaging; Fetal Heart - physiopathology; fetus; Fetus - diagnostic imaging; Fetus - pathology; Fetuses; Gestation; Gestational Age; Glucocorticoids - administration & dosage; Glucocorticoids - therapeutic use; Health surveillance; Heart; Heart Block - congenital; Heart Block - diagnosis; Heart Block - immunology; Heart Block - physiopathology; Humans; Implantation; Incidence; Infant; Infant, Newborn; Intervals; Medicin och hälsovetenskap; neonatal lupus erythematosus; Newborn babies; postnatal outcome; Pregnancy; Pregnancy - blood; Pregnancy - immunology; Prospective Studies; Restoration; Risk; treatment; Treatment Outcome; Ventricle; treatment; atrioventricular block; echocardiography; neonatal lupus erythematosus; congenital heart block; postnatal outcome; autoantibodies; Doppler; fetus
• ISSN: 0960-7692; 1469-0705; 1469-0705
• DOI: 10.1002/uog.20214

ABSTRACT
Objectives
Assuming that autoimmune congenital heart block (CHB) is a progressive disease amenable to therapeutic modulation, we introduced a surveillance program for at‐risk pregnancies with the dual aim of investigating if fetal atrioventricular block (AVB) could be detected and treated before becoming complete and irreversible, and to establish the incidence of AVB I, II and III in a large prospective cohort.
Methods
This was a prospective study of 212 anti‐Ro52 antibody‐exposed pregnancies at risk of fetal AVB that were followed weekly between 18 and 24 weeks' gestation at our tertiary fetal cardiology center from 2000 to 2015. A 12‐lead electrocardiogram (ECG) was recorded within 1 week after birth. Fetal Doppler atrioventricular (AV) intervals were converted to Z‐scores using reference standard values derived from normal pregnancies. Each fetus was represented by the average value of the two recordings, obtained at two consecutive visits, which resulted in the longest AV interval. AV interval values were classified into normal AV conduction (Z‐score ≤ 2.0) and three levels of delayed AV conduction: Z‐score > 2.0 and ≤ 3.0, Z‐score > 3.0 and ≤ 4.0, and Z‐score > 4.0.
Results
AVB II or III developed in 6/204 (2.9%) pregnancies without a CHB history and 1/8 (12.5%) of those with a CHB history. AV intervals > 2 and ≤ 3, > 3 and ≤ 4, and > 4 were detected in 16.0%, 7.5% and 2.8% of cases, respectively, and were related to the PR interval on 185 available ECGs. Three of the five cases with AVB III and one of two cases with 2:1 AVB II developed within 1 week of AV interval Z‐score of 1.0, 1.9, 2.8 and 1.9, respectively. Transplacental treatment with betamethasone was associated with restoration of 1:1 AV conduction in the two fetuses with AVB II, with a better long‐term result (normal ECG vs AVB I or II) observed in the case in which treatment was started within 1 week after AVB developed. Betamethasone treatment did not reverse AVB III, although a temporary effect on AV conduction was observed in 1/5 cases. Notably, the three cases in which treatment was started within 1 week after AVB III development responded with a higher ventricular rate than the other two cases and did not require pacemaker implantation until a later age (2–5 years vs 1.5–2 months).
Conclusion
Fetal AV interval is a poor predictor of CHB progression, but CHB surveillance still allows detection of fetuses with AVB II or III shortly after its development, allowing for timely treatment initiation and potentially better outcome. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.
Linked Comment: Ultrasound Obstet Gynecol 2019; 54: 7–9