Abstract
Anti-Ro/SSA antibodies are associated with a risk of 1–2 % to develop complete atrioventricular
block (AVB) in fetuses of positive mothers. Complete AVB is irreversible, but studies
suggest that anti-inflammatory treatment during the transition period from a normal
fetal heart rate (FHR) to an AVB might stop this progression and restore sinus rhythm.
The most efficient method for diagnostic evaluation of this arrhythmia is the pulsed‐Doppler
fetal echocardiography.
However, weekly or bi-weekly recommended fetal echocardiographic surveillance can
rarely identify an AVB in time for treatment success, also because the transition
from a normal rhythm to a third degree AVB is very fast.
Daily FHR monitoring in a medical facility could increase the chances of identifying
the AVB onset but is difficult to realize. For this reason, an alternative method
of FHR monitoring, performed directly by mothers in their home context, has been recently
proposed. We present a case report utilizing this approach and review the current
evidence about this condition.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to European Journal of Obstetrics and Gynecology and Reproductive BiologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Antinuclear, anticytoplasmic, and anti-Sjogren’s syndrome antigen A (SS-A/Ro) antibodies in female blood donors.Clin Immunol Immunopathol. 1985; 36: 120-128
- Prevalence of non-organ-specific autoantibodies in pregnant and non-pregnant healthy women.Lupus. 1992; 1: 141-144
- Risk of congenital complete heart block in newborns of mothers with anti-Ro/SSA antibodies detected by counter immunoelectrophoresis: a prospective study of 100 women.Arthritis Rheum. 2001; 44 (PMID: 11508435): 1832-1835https://doi.org/10.1002/1529-0131(200108)44:8<1832:AID-ART320>3.0.CO;2-C
- Congenital heart block not associated with anti-Ro/La antibodies: comparison with anti-Ro/La-positive cases.J Rheumatol. 2009; 36 (Epub 2009 Jun 30): 1744-1748https://doi.org/10.3899/jrheum.080737
- Autoimmune associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry.J Am Coll Cardiol. 1998; 31: 1658-1666
- The clinical spectrum of autoimmune heart block.Nat Rev Rheumatol. 2015; 11: 301-312
- Comparison of treatment with fluorinated glucocorticoids to the natural history of autoantibody associated congenital heart block: retrospective review of the research registry for neonatal lupus.Arthritis Rheum. 1999; 42: 2335-2345
- Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study.Circulation. 2008; 117: 485-493
- Detection and successful treatment of emergent anti- SSA-mediated fetal atrioventricular block.Am J Obstet Gynecol. 2016; 215: 527-528
- Repeated echocardiographic monitoring of fetuses exposed to maternal anti-SSA antibodies: time to question the dogma.Lancet Rheumatol. 2019; 1: e187-93
- Autoimmune-mediated congenital heart block.Best Pract Res Clin Obstet Gynaecol. 2020; 64 (Epub 2019 Oct 8. PMID: 31685414): 41-51https://doi.org/10.1016/j.bpobgyn.2019.09.001
- First report of the italian registry on immune-mediated congenital heart block (Lu.NE registry).Front Cardiovasc Med. 2019; 28 (PMID: 30873413; PMCID: PMC6404544): 11https://doi.org/10.3389/fcvm.2019.00011
- Proposal for a new definition of congenital complete atrioventricular block.Lupus. 2003; 12: 427-435https://doi.org/10.1191/0961203303lu408oa.)
- Association of natural killer cell ligand polymorphism HLA-C Asn80Lys with the development of Anti-SSA/Ro-Associated congenital heart block.Arthritis Rheumatol. 2017; 69 (Epub 2017 Oct 17. PMID: 29045069; PMCID: PMC5679096): 2170-2174https://doi.org/10.1002/art.40228
- Progress in the pathogenesis and treatment of cardiac manifestations of neonatal lupus.Curr Opin Rheumatol. 2017; 29 (PMID: 28520682; PMCID: PMC5578407): 467-472https://doi.org/10.1097/BOR.0000000000000414
- Autoimmune calcium channelopathies and cardiac electrical abnormalities.Front Cardiovasc Med. 2019; 6: 54https://doi.org/10.3389/fcvm.2019.0005
- Congenital heart block: identification of autoantibody binding site on the extracellular loop (domain I, S5-S6) of alpha(1D) L-type Ca channel.J Autoimmun. 2010; 34 (Epub 2009 Jul 28. PMID: 19640679; PMCID: PMC2822065): 80-86https://doi.org/10.1016/j.jaut.2009.06.005
- Anatomical and pathological findings in hearts from fetuses and infants with cardiac manifestations of neonatal lupus.Rheumatology (Oxford). 2012; 51: 1086-1092
- Immunohistologic evidence supports apoptosis, IgG deposition, and novel macrophage/fibroblast crosstalk in the pathologic cascade leading to congenital heart block.Arthritis Rheum. 2004; 50: 173-182
- Siglec-1 macrophages and the contribution of IFN to the development of autoimmune congenital heart block.J Immunol. 2019; 202: 48-55
- Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management.Eur J Pediatr. 2016; 175 (Springer: Berlin, Germany): 1235-1248
- Hydrops fetalis.Complications of pregnancy. IntechOpen, London, UK2019
- The many faces of hydrops.J Pediatr Surg. 2015; 50: 50-54
- Diagnosis and treatment of fetal cardiac disease: a scientific statement from the american heart association.Circulation. 2014; 129: 2183-2242
- EULAR recommendations for women’s health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome.Ann Rheum Dis. 2017; 76 (Epub 2016 Jul 25. PMID: 27457513; PMCID: PMC5446003): 476-485https://doi.org/10.1136/annrheumdis-2016-209770
- Pulsed Doppler echocardiographic assessment of the fetal PR interval.Am J Cardiol. 2000; 86: 236-239
- The fetal Doppler mechanical PR interval: a validation study.Fetal Diagn Ther. 2004; 19: 31-34
- Fetal Doppler mechanical PR interval: correlation with fetal heart rate, gestational age and fetal sex.Ultrasound Obstet Gynecol. 2009; 34: 538-542
- Reference values for time intervals between atrial and ventricular contractions of the fetal heart measured by two Doppler techniques.Am J Cardiol. 2001; 88: 1433-1436
- Signs of first‐degree heart block occur in one‐third of fetuses of pregnant women with anti‐SSA/Ro 52‐kd antibodies.Arthritis Rheum. 2004; 50: 1253-1261
- Outcomes of fetal echocardiographic surveillance in anti-SSA exposed fetuses at a large fetal cardiology center.Prenat Diagn. 2014; 34: 1207-1212
- Should we treat congenital heart block with fluorinated corticosteroids?.Autoimmun Rev. 2017; 16 (Epub 2017 Sep 9. PMID: 28899797.): 1115-1118https://doi.org/10.1016/j.autrev.2017.09.005
- Fluorinated steroids are not superior to any treatment to ameliorate the outcome of autoimmune mediated congenital heart block: a systematic review of the literature and meta-analysis.Clin Exp Rheumatol. 2020; 38 (Epub 2020 Jun 23. PMID: 32573408.): 783-791
- Heart sounds at home: feasibility of an ambulatory fetal heart rhythm surveillance program for anti- SSA-positive pregnancies.J Perinatol. 2017; 37: 226-230
- Home monitoring for fetal heart rhythm during anti-ro pregnancies.J Am Coll Cardiol. 2018; 72: 1940-1951
- Type I IFN system activation in newborns exposed to Ro/SSA and La/SSB autoantibodies in utero.RMD Open. 2020; 6e000989
- Mechanism of endosomal TLR inhibition by antimalarial drugs and imidazoquinolines.J Immunol. 2011; 186: 4794-4804
- Novel small molecule inhibitors of TLR7 and TLR9: mechanism of action and efficacy in vivo.Mol Pharmacol. 2014; 85: 429-440
- Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent anti‐SSA/ro‐antibody—associated cardiac manifestations of neonatal lupus.Circulation. 2012; 126: 76-82
- Evaluation of the risk of anti-SSA/Ro-SSB/La antibody-associated cardiac manifestations of neonatal lupus in fetuses of mothers with systemic lupus erythematosus exposed to hydroxychloroquine.Ann Rheum Dis. 2010; 69: 1827-1830
- Hydroxychloroquine to prevent recurrent congenital heart block in fetuses of Anti-SSA/Ro-Positive mothers.J Am Coll Cardiol. 2020; 76: 292-302https://doi.org/10.1016/j.jacc.2020.05.045
- The EULAR points to consider for use of antirheumatic drugs before pregnancy, and during pregnancy and lactation.Ann Rheum Dis. 2016; 75: 795-810
- Disease‐modifying anti‐rheumatic drug use in pregnant women with rheumatic diseases: a systematic review of the risk of congenital malformations.Clin Exp Rheumatol. 2016; 34: 172-183
- Fetal cardiac arrhythmias: current evidence.Ann Pediatr Cardiol. 2018; 11 (Erratum in: Ann Pediatr Cardiol. 2018 Sep-Dec;11(3):332. PMID: 29922012; PMCID: PMC5963229.): 148-163https://doi.org/10.4103/apc.APC_134_17
- Umbilical pressure measurement in the evaluation of nonimmune hydrops fetalis.Am J Obstet Gynecol. 1993; 168 (PMID: 8456887): 817-823https://doi.org/10.1016/s0002-9378(12)90827-3
- Electrophysiological characteristics of fetal atrioventricular block.J Am Coll Cardiol. 2008; 51 (PMID: 18174041; PMCID: PMC3296565): 77-84https://doi.org/10.1016/j.jacc.2007.06.060
- Prolongation of the atrioventricular conduction in fetuses exposed to maternal Anti‐Ro/SSA and Anti‐La/SSB antibodies did not predict progressive heart block: a prospective observational study on the effects of maternal antibodies on 165 fetuses.J Am Coll Cardiol. 2011; 57: 1487-1492
- The prevention, screening and treatment ofcongenital heart block from neonatal lupus:a survey of provider practices.Rheumatology. 2018; 57: v9-v17
- Early diagnosis and treatment of atrioventricular block in the fetus exposed to maternal anti‐SSA/Ro‐SSB/La antibodies a prospective, observational, fetal kinetocardiogram‐based study.Circulation. 2009; 119: 1867-1872
- Prevention of complete heart block in children of mothers with anti‐SSA/Ro and anti‐SSB/La autoantibodies: detection and treatment of first‐degree atrioventricular block.Curr Opin Rheumatol. 2009; 21: 478-482
- Maternal steroid therapy for fetuses with second‐degree immune‐mediated congenital atrioventricular block: a systematic review and meta‐analysis.Acta Obstet Gynecol Scand. 2018; 97: 787-794
- Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease.Circulation. 2004; 110 (Epub 2004 Sep 7. PMID: 15353508): 1542-1548https://doi.org/10.1161/01.CIR.0000142046.58632.3A
- Prevention and treatment in utero of autoimmune-associated congenital heart block.Cardiol Rev. 2014; 22 (PMID: 25050975; PMCID: PMC4539276): 263-267https://doi.org/10.1097/CRD.0000000000000026
- Plasmapheresis, intravenous immunoglobulins and bethametasone‐a combined protocol to treat autoimmune congenital heart block: a prospective cohort study.Clin Exp Rheumatol. 2016; 34: 706-713
- Plasma exchange effectively removes 52‐ and 60‐kDa anti‐RO/SSA and anti‐La/SSB antibodies in pregnant women with congenital heart block.Transfusion. 2015; 55: 1782-1786
- Failure of intravenous immunoglobulin to prevent congenital heart block: findings of a multicenter, prospective, observational study.Arthritis Rheum. 2010; 62: 1147-1152https://doi.org/10.1002/art.27350
- Evaluation of fetuses in a study of intravenous immunoglobulin as preventive therapy for congenital heart block: results of a multicenter, prospective, open-label clinical trial.Arthritis Rheum. 2010; 62: 1138-1146https://doi.org/10.1002/art.27308
- Pregnancy outcome in 100 women with autoimmune diseases and anti-Ro/SSA antibodies: a prospective controlled study.Lupus. 2002; 11: 716-721https://doi.org/10.1191/0961203302lu252oa
- Pregnancy outcomes in patients with autoimmune diseases and anti-Ro/SSA antibodies.Clin Rev Allergy Immunol. 2011; 40: 27-41https://doi.org/10.1007/s12016-009-8190-6.)
Article info
Publication history
Published online: January 28, 2021
Accepted:
January 18,
2021
Received:
December 4,
2020
Identification
Copyright
© 2021 Elsevier B.V. All rights reserved.
