Abstract
Exposure to maternal anti-Ro (SS-A) and anti-La (SS-B) antibodies is a well-described risk factor for the development of fetal atrioventricular (AV) block. The role of maternal fluorinated steroids in the treatment and prevention of antibody-mediated fetal AV block is controversial. Fetal atrial flutter has rarely been described in association with maternal antibodies. This report describes a case of fetal exposure to maternal anti-Ro antibodies with associated second-degree AV block and atrial flutter. Interestingly, the reported patient had 2:1 AV conduction during both normal atrial rates (consistent with AV node conduction disease) and episodes of flutter (consistent with physiologic AV node functionality). The fetus was treated with transplacental digoxin and dexamethasone, which resolved both rhythm disturbances. The case report is followed by a brief discussion of AV block and atrial flutter associated with maternal antibody exposure.
Abbreviations
- AV:
-
Atrioventricular
- AF:
-
Atrial flutter
- BPM:
-
Beats per minute
- EFE:
-
Endocardial fibroelastosis
References
Costedoat-Chalumeau N, Amoura Z, Villain E, Cohen L, Piette J-C (2005) Anti-SSA/Ro antibodies and the heart: more than complete congenital heart block? A review of electrocardiographic and myocardial abnormalities and of treatment options. Arthritis Res Ther 7:69–73
Cuneo BF, Strasburger JF, Niksch A, Ovadia M, Wakai RT (2009) An expanded phenotype of maternal SSA/SSB antibody-associated fetal cardiac disease. J Maternal Fetal Neonatal Med 22:233–238
Friedman DM, Kim MY, Copel JA, Llanos C, Davis C, Buyon JP (2009) Prospective evaluation of fetuses with autoimmune-associated congenital heart block followed in the PR interval and dexamethasone evaluation (PRIDE) study. Am J Cardiol 103:1102–1106
Jaeggi ET, Nii M (2005) Fetal brady- and tachyarrhythmias: new and accepted diagnostic and treatment methods. Semin Fetal Neonatal Med 10:504–514
Jaeggi E, Fouron JC, Drblik SP (1998) Fetal atrial flutter: diagnosis, clinical features. Pediatrics 132(2)
Jaeggi E, Laskin C, Hamilton R, Kingdom J, Silverman E (2010) The importance of the level of maternal anti-Ro/SSA antibodies as a prognostic marker of the development of cardiac neonatal lupus erythematosus a prospective study of 186 antibody-exposed fetuses and infants. J Am Coll Cardiol 55:2778–2784
Jaeggi ET, Silverman ED, Laskin C, Kingdom J, Golding F, Weber R (2011) 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 57:1487–1492
Lisowski LA, Verheijen PM, Benatar AA, Soyeur DJ, Stoutenbeek P, Brenner JI, Kleinman CS, Meijboom EJ (2000) Atrial flutter in the perinatal age group: diagnosis, management and outcome. J Am Coll Cardiol 35:771–777
Lockshin MD, Bonfa E, Elkon K, Druzin ML (1988) Arthritis and rheumatism: neonatal lupus risk to systemic lupus erythematosus. Arthritis Rheum 31(6)
Lopes LM, Tavares GMP, Damiano AP, Lopes MAB, Aiello VD, Schultz R, Zugaib M (2008) Perinatal outcome of fetal atrioventricular block: one hundred and sixteen cases from a single institution. Circulation 118:1268–1275
Nield LE (2002) Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis. Circulation 105:843–848
Rein AJJT, Mevorach D, Perles Z, Gavri S, Nadjari M, Nir A, Elchalal U (2009) 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 119:1867–1872
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Sacks, J.H., Samai, C., Gomez, K. et al. Maternal Antibody-Associated Fetal Second-Degree Heart Block and Atrial Flutter: Case Report and Review. Pediatr Cardiol 34, 2040–2043 (2013). https://doi.org/10.1007/s00246-012-0588-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-012-0588-x