Outcome in 212 anti‐Ro/<scp>SSA</scp>‐positive pregnancies and population‐based incidence of congenital heart block

Skog, Amanda; Lagnefeldt, Linda; Conner, Peter; Wahren‐Herlenius, Marie; Sonesson, Sven‐Erik

doi:10.1111/aogs.12785

Cited by 38 publications

(33 citation statements)

References 27 publications

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“…Given that the AV interval Z ‐score threshold of 3 for diagnosing AVB‐I corresponded to an AV interval ranging between 151 and 167 ms, depending on GA, it is important to consider GA before diagnosing AVB‐I. As some but not all fetuses with AVB‐I will progress to AVB‐II or ‐III, we agree with the approach of Skog et al . that repeat measurements, rather than a single prolonged AV interval, will identify more accurately fetuses that may benefit from treatment.…”

Section: Discussionsupporting

confidence: 79%

Assessment of atrioventricular conduction by echocardiography and magnetocardiography in normal and anti‐Ro/SSA‐antibody‐positive pregnancies

Cuneo

Bitant

Strasburger

et al. 2019

Ultrasound in Obstet & Gyne

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Objectives The objectives of this study were, first, to evaluate the association between fetal echocardiographic atrioventricular (AV) and magnetocardiographic (fMCG) PR intervals at different gestational ages (GAs) in normal and anti‐Ro/SSA‐antibody‐positive pregnancies; second, to determine if PR interval could be predicted by AV interval; and third, to assess the neonatal outcome of fetuses with prolonged AV and PR intervals, with the goal of developing criteria for fetal first‐degree AV block (AVB‐I). Methods This was a retrospective study of anti‐Ro/SSA‐antibody‐positive pregnancies (cases) and controls that underwent fMCG and fetal echocardiography at the same recording session. Cardiac cycle length, GA and AV (by mitral inflow/aortic outflow Doppler) and PR (by fMCG) intervals were measured. We tested for significant differences between AV and PR intervals using generalized estimating equations to account for repeat measurements, and assessed whether PR interval could be predicted reliably by AV interval. After delivery, infants with fetal AV or PR interval Z‐score ≥ 3 underwent 12‐lead electrocardiography. Results Thirty‐nine controls and 31 cases underwent 46 and 36 simultaneous fMCG and echocardiographic examinations, respectively; 101 controls and nine cases underwent fMCG only. AV and PR intervals increased with GA (P < 0.05 for both). Overall, AV and PR intervals were significantly different from each other (P < 0.001); this difference was not significant when compared between cases and controls (P = 0.222). PR interval could not be predicted accurately from AV interval and GA alone. Three of four cases with AV and PR interval Z‐scores > + 3 had postnatal AVB‐I despite treatment. The fourth fetus, which had predominately second‐degree AVB and rare periods of AVB‐I, progressed to third‐degree AVB despite treatment with dexamethasone. Conclusions The diagnostic threshold for AVB‐I, defined by AV interval Z‐score, is GA dependent. Based on the observed data, an AV interval Z‐score threshold of 3 (AV interval, 151–167 ms) may be appropriate. Echocardiographic AV interval was not predictive of fMCG‐PR interval. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

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Section: Discussionsupporting

confidence: 79%

Assessment of atrioventricular conduction by echocardiography and magnetocardiography in normal and anti‐Ro/SSA‐antibody‐positive pregnancies

Cuneo

Bitant

Strasburger

et al. 2019

Ultrasound in Obstet & Gyne

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“…NL is responsible for 80 to 95% of all cases of CHB diagnosed in utero or in the neonatal period [88]. Cardiac NL is less common than other NL manifestation and the risk of CHB is higher in anti-Ro/SSA positive women with pSS or other autoimmune diseases, than in women with SLE [11,89]. The incidence of cardiac NL manifestations increases in women with anti-Ro/SSA and/or anti-La/SSB antibodies who have had a previous child with CHB, being the recurrence estimated between 12% [90] and 17% [91].…”

Section: Lupus Anti-ro/ssa and Anti-la/ssb Antibodies And Neonatal Lmentioning

confidence: 99%

A comprehensive review of the clinical approach to pregnancy and systemic lupus erythematosus

Lazzaroni

Dall’Ara

Fredi

et al. 2016

Journal of Autoimmunity

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“…We reported previously that our surveillance cases of AVB II/III were diagnosed earlier in gestation (20.0 ± 2.3 vs 24.1 ± 4.4 weeks) and had a trend towards higher heart rate than cases referred when AVB was already present 31 . Other investigators have found that non-surveillance CHB cases were detected at a median or average of 24 weeks of gestation [9][10][11]18 , with heart rate reported to be below 50 bpm in about 20% of cases 9,10 , and 9-14% of cases being hydropic at diagnosis [8][9][10][11] .…”

Section: Discussionmentioning

confidence: 63%

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

Sonesson

Ambrosi

Wahren‐Herlenius

2019

Ultrasound in Obstet & Gyne

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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.

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Outcome in 212 anti‐Ro/SSA‐positive pregnancies and population‐based incidence of congenital heart block

Abstract: This study of CHB provides new information on the incidence of CHB and outcome of pregnancy in anti-Ro/SSA-positive women, which has clinical relevance when counseling rheumatic patients considering pregnancy.

Cited by 38 publications

References 27 publications

Assessment of atrioventricular conduction by echocardiography and magnetocardiography in normal and anti‐Ro/SSA‐antibody‐positive pregnancies

Assessment of atrioventricular conduction by echocardiography and magnetocardiography in normal and anti‐Ro/SSA‐antibody‐positive pregnancies

A comprehensive review of the clinical approach to pregnancy and systemic lupus erythematosus

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

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