Abstract
The objective of this study was to describe the clinical and immunological characteristics of maternal autoimmune-mediated fetal congenital heart block (CHB) in a cohort of pregnant women from an autoimmune disease pregnancy clinic. This is a retrospective observational study of all women presenting with CHB in our autoimmune disease pregnancy clinic from January 1997 to December 2014. In addition, perinatal outcome is also described. Fourteen patients accounting for 18 fetuses with CHB were identified. The median age was 32.5 years (range, 22–40). Seven (50 %) patients had Sjögren’s syndrome, and the remaining seven were asymptomatic carriers of autoantibodies. All patients had anti-Ro/SSA antibodies, and 11/13 (85 %) had anti-La/SSB antibodies. The median gestational age at the time of CHB was 22 weeks (range 18–28). Complete third degree CHB was detected in 12 (67 %). Seven cases of CHB were treated with dexamethasone, two with ritodrine, and one with the association of dexamethasone, ritodrine, and terbutaline. In 9 (50 %) cases that presented with, or developed, very poor prognosis factors, such as a ventricular rate below 50–55 bpm and/or the presence of fetal hydrops, parents opted for the termination of pregnancy, after dedicated counseling. Finally, there were nine newborns (seven males [78 %]) with median age at delivery of 37 weeks (range, 32–39). A definitive epicardial pacemaker was placed in six newborns, four of them within 2 weeks of life. CHB is a severe complication related to maternal anti-Ro/SSA and anti-La/SSB antibodies. Our results confirm previous data showing that therapy is ineffective, and most of the surviving patients will require neonatal pacemaker.
Similar content being viewed by others
References
Sirén MK, Julkunen H, Kaaja R (1998) The increasing incidence of isolated congenital heart block in Finland. J Rheumatol 25:1862–1864
Brito-Zerón P, Izmirly PM, Ramos-Casals M, Buyon JP, Khamashta MA (2015) The clinical spectrum of autoimmune congenital heart block. Nat Rev Rheumatol 11:301–312
Buyon JP, Hiebert R, Copel J et al (1998) Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol 31:1658–1666
Gleicher N, Elkayam U (2013) Preventing congenital neonatal heart block in offspring of mothers with anti-SSA/Ro and SSB/La antibodies: a review of published literature and registered clinical trials. Autoimmun Rev 12:1039–1045
Izmirly PM, Costedoat-Chalumeau N, Pisoni CN et al (2012) Maternal use of hdroxychloroquine is associated with a reduced risk of recurrent anti-SSA/Ro-antibody-associated cardiac manifestations of neonatal lupus. Circulation 126:76–82
Friedman DM, Llanos C, Izmirly PM et al (2010) 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 62:1138–1146
Pisoni CN, Brucato A, Ruffatti A et al (2010) Failure of intravenous immunoglobulin to prevent congenital heart block: findings of a multicenter, prospective, observational study. Arthritis Rheum 62:1147–1152
Izmirly PM, Kim MY, Llanos C et al (2010) 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 69:1827–1830
Tunks RD, Clowse MEB, Miller SG et al (2013) Maternal autoantibody levels in congenital heart block and potential prophylaxis with anti-inflammatory agents. Am J Obstet Gynecol 208:64.e1–7
Friedman DM, Kim MY, Copel JA, Davis C, Phoon CK, Glickstein JS, Buyon JP, Investigators PRIDE (2008) Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR interval and dexamethasone evaluation (PRIDE) prospective study. Circulation 117:485–493
Eliasson H, Sonesson SE, Sharland G et al (2011) Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients. Circulation 124:1919–1926
Levensque K, Morel N, Maltret A et al (2015) Description of 214 cases of autoimmune congenital heart block: results of the French neonatal lupus syndrome. Autoimmun Rev 14:1154–1160
Yoshida H, Iwamoto M, Sakakibara H, Shigeta H, Hirahara F, Sato K (2001) Treatment of fetal congenital complete heart block with maternal administration of beta-sympathomimetics (terbutaline): a case report. Gynecol Obstet Invest 52:142–144
Matsushita H, Higashino M, Sekizuka N, Kurabayashi T, Takakuwa K, Tanaka K (2002) Successful prenatal treatment of congenital heart block with ritodrine administered transplacentally. Arch Gynecol Obstet 267:51–53
Davey DL, Bratton SL, Bradley DJ, Yetman AT (2011) Relation of maternal anti-Ro/La antibodies to aortic dilation in patients with congenital complete heart block. Am J Cardiol 108:561–564
Altit G, Sarquella-Brugada G, Dahdah N et al (2014) Effect of dual-chamber pacemaker implantation on aortic dilatation in patients with congenital heart block. Am J Cardiol 114:1573–1577
Capone C, Buyon JP, Friedman DM, Frishman WH (2012) Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population. Cardiol Rev 20:72–76
Izmirly PM, Saxena A, Kim MY et al (2011) Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus. Circulation 124:1927–1935
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
The protocol was approved by the institutional review board of the Hospital Clinic of Barcelona.
Disclosures
None.
Funding source
GS-B is supported by the “Fundació Privada Daniel Bravo Andreu.”
Rights and permissions
About this article
Cite this article
Doti, P.I., Escoda, O., Cesar-Díaz, S. et al. Congenital heart block related to maternal autoantibodies: descriptive analysis of a series of 18 cases from a single center. Clin Rheumatol 35, 351–356 (2016). https://doi.org/10.1007/s10067-016-3174-4
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-016-3174-4