Hostname: page-component-76fb5796d-vfjqv Total loading time: 0 Render date: 2024-04-25T12:57:59.132Z Has data issue: false hasContentIssue false

The impact of treatment of the fetus by maternal therapy on the fetal and postnatal outcomes for fetuses diagnosed with isolated complete atrioventricular block

Published online by Cambridge University Press:  22 April 2009

Vlasta Fesslova*
Affiliation:
Centre of Fetal Cardiology, Policlinico San Donato IRCCS, Milano;
Gabriele Vignati
Affiliation:
Departments of Pediatric Cardiology,
Antonio Brucato
Affiliation:
Departments of Rheumatology,
Marina De Sanctis
Affiliation:
Department of Pediatrics, Policlinico San Matteo, Pavia;
Gianfranco Butera
Affiliation:
Centre of Fetal Cardiology, Policlinico San Donato IRCCS, Milano;
Maria Pia Pisoni
Affiliation:
Obstetrics and Gynecology, Ospedale Niguarda, Milano;
Enrico Chiappa
Affiliation:
Department of Pediatric Cardiology, Azienda Ospedaliera-Universitaria Meyer, Firenze;
Barbara Acaia
Affiliation:
Department of Obstetrics, Gynecology and Neonatology, Fondazione Ospedale Maggiore Policlinico, Mangiagalli, Regina Elena, Milano;
Pier Luigi Meroni
Affiliation:
Allergy and Immunology Unit, University of Milan, Istituto Auxologico Italiano, Milano, Italy
*
Correspondence to: Dr Vlasta Fesslova, Centre of Fetal Cardiology, Policlinico San Donato, Via Morandi 30, San Donato Milanese, Milano 20097, Italy. Tel: 0039 02 52774 511/393; Fax: 0039 02 52774 459; E-mail: cardiologiafetale@grupposandonato.it

Abstract

Objectives

to analyse retrospectively the data of fetuses diagnosed with isolated complete atrioventricular block and efficacy of treatment of the fetus by maternal therapy.

Materials

Between 1992 and 2004, we diagnosed complete atrioventricular block in 26 singleton and 2 twins fetuses of 27 pregnant women known to have anti Ro/La antibodies, 11 with autoimmune disease, one patient analysed in 2 pregnancies. At presentation, 20 of the fetuses were compensated and non-hydropic, while 8 had hydrops. Twenty patients were treated with dexamethasone, 2 with associated salbutamol and one mother with isoproterenol.

Results

Age at presentation was not different between the hydropic and non-hydropic fetuses. The fetuses with hydrops, however, had a lower mean heart rate at presentation, 48.5 ± 9.25 with a range from 32 to 60, compared to 59.95 ± 7.9 beats per minute, with a range from 50 to 80, in the non-hydropic fetuses (p less than 0.002). Equally, after birth the mean heart rate in hydropic fetuses was 42.6 ± 5.1, with a range from 38 to50, as opposed to 56.05 ± 11.8 beats per minute, with a range from 29 to 110, in the non-hydropic fetuses (p less than 0.015), The hydropic fetuses were delivered at 31.7 ± 3.8 weeks’ gestation, with a range from 29 to 38 weeks (p less than 0.003) compared to 35.5 weeks’ gestation ±2.04, with a range from 31 to 38, in the non-hydropic fetuses. Mortality was 37.5% in the hydropic fetuses, versus 5% of those without hydrops (p less than 0.02). Pacemakers were implanted in 22 of 26 infants born alive, at a median of 45 days, with a range from 1 day to 5 years, in those without hydrops during fetal life, and 3 days, with a range from 1 day to 8 months in those afflicted by hydrops, of whom 2 died despite the implant of the pacemaker. The presence and degree of hydrops had a significantly negative predictive value. No significant differences were observed between the treated and non treated cases, albeit that administration of steroids ameliorated rapidly the hydrops in 3 of 5 cases.

Conclusions

The outcome in our cases was mainly dependent on the presence and degree of fetal cardiac failure. Treatment of the fetus by maternal administration of steroids did not result in any regression of the conduction disorder, but had a favourable effect on fetal hydrops.

Type
Original Article
Copyright
Copyright © Cambridge University Press 2009

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Taylor, PV, Scott, JS, Gerlis, LM, Esscher, E, Scott, O. Maternal antibodies against fetal cardiac antigens in congenital complete heart block. New Engl J Med 1986; 315: 667672.CrossRefGoogle ScholarPubMed
2. Buyon, JP, Hiebert, R, Copel, J, et al. Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national lupus registry. J Am Coll Cardiol 1998; 31: 16581666.CrossRefGoogle ScholarPubMed
3. Ho, YS, Esscher, E, Anderson, RH, et al. Anatomy of congenital complete heart block and relation to maternal anti-Ro antibodies. Am J Cardiol 1986; 58: 291294.CrossRefGoogle ScholarPubMed
4. Lang, B, Wilhelm, C, Gildein, P, Vaith, P, Schillinger, H, Peter, HH. Fetal complete heart block with myocarditis and maternal SS-A-/AA-B/antibodies. Schweiz Med Wochenschr 1990; 120: 17411744.Google ScholarPubMed
5. Fesslova, V, Mannarino, S, Salice, P, et al. Neonatal lupus: fetal myocarditis progressing to atrioventricular block in triplets. Lupus 2003; 12: 775778.CrossRefGoogle ScholarPubMed
6. Nield, LY, Silverman, ED, Taylor, GP, et al. Maternal anti-Ro and anti-La antibody- associated endocardial fibrolelastosis. Circulation 2002; 105: 843848.CrossRefGoogle Scholar
7. Nield, LE, Silverman, ED, Smallhorn, JF, et al. Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block. J Am Coll Cardiol 2002; 40: 796802.CrossRefGoogle ScholarPubMed
8. Moak, JP, Barron, KS, Hougen, TJ, et al. Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela. J Am Coll Cardiol 2001; 37: 238242.CrossRefGoogle ScholarPubMed
9. Cimaz, R, Catelli, L, Luzzana, C, Panzeri, P, Meroni, PL. Neonatal lupus syndromes. IMAJ 2000; 2: 228231.Google ScholarPubMed
10. Schmidt, KG, Ulmer, HE, Silverman, NH, Kleinman, CS, Copel, JA. Perinatal outcome of fetal complete atrioventricular block: a multicenter experience. J Am Coll Cardiol 1991; 17: 13601366.CrossRefGoogle ScholarPubMed
11. Groves, AMM, Allan, LD, Rosenthal, E. Outcome of isolated congenital complete heart block diagnosed in utero. Heart 1996; 75: 190194.CrossRefGoogle ScholarPubMed
12. Bierman, FZ, Baxi, L, Jaffe, I. Fetal hydrops and congenital complete heart block: Response to maternal steroid therapy. J Pediatr 1988; 112: 646648.CrossRefGoogle ScholarPubMed
13. Rosenthal, D, Druzin, M, Chin, C, Dubin, A. A new therapeutic approach to the fetus with congenital complete heart block: preemptive, targeted therapy with dexamethasone. Obstet Gynecol 1998; 92: 689691.Google Scholar
14. Jaeggi, ET, Fouron, JC, Silverman, ED, Ryan, G, Smallhorn, J, Hornberger, LK. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation 2004; 110: 15421548.CrossRefGoogle ScholarPubMed
15. Groves, AM, Allan, LD, Rosenthal, E. Therapeutic trial of sympathomimetics in three cases of complete heart block in the fetus. Circulation 1995; 92: 33943396.CrossRefGoogle ScholarPubMed
16. Jaeggi, ET, Hamilton, RM, Silverman, ED, et al. Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block: a single institution’s experience of 30 years. J Am Coll Cardiol 2002; 39: 130137.CrossRefGoogle ScholarPubMed
17. Vignati, G, Brucato, A, Pisoni, MP, et al. Clinical course of pre- and post-natal isolated congenital atrioventricular block diagnosed in utero. G Ital Cardiol 1999; 29: 14781487.Google ScholarPubMed
18. Gladman, G, Silverman, ED, Yuk-Law, , et al. Fetal echocardiographic screening of pregnancies of mothers with anti-Ro and/or anti-La antibodies. Am J Perinatol 2002; 19: 7380.CrossRefGoogle ScholarPubMed
19. Brucato, A, Franceschini, F, Gasparini, M, et al. Isolated congenital complete heart block. Longterm outcome of mothers, maternal antibody specificity and immunogenetic background. J Rheumatol 1995; 22: 533540.Google ScholarPubMed
20. Costedoat-Chalumeau, N, Georgin-Lavialle, S, Amoura, Z, Piette, JC. Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block. Lupus 2005; 14: 660664.CrossRefGoogle ScholarPubMed
21. Watson, RM, Scheel, JN, Petri, M, et al. Neonatal lupus erythematosus. Report of serological and immunogenetic studies in twin discordant for congenital heart block. Brit J Dermatology 1994; 130: 342348.CrossRefGoogle ScholarPubMed
22. Cooley, HM, Keech, CL, Melny, BJ, Menaheim, S, Morahan, G, Kay, TWH. Monozygotic twins discordant for complete congenital heart block. Arthritis Rheum 1997; 40: 381384.CrossRefGoogle Scholar
23. Miyagawa, S, Dohi, K, Yoshioka, A, Shirai, T. Female predominance of immune response to SSA/Ro antigens and risk of neonatal lupus erythematosus. Br J Dermatol 1990; 123: 223227.CrossRefGoogle ScholarPubMed
24. Buyon, JP, Swersky, S, Fox, H, Bierman, F, Winchester, R. Intra-uterine therapy for presumptive fetal myocarditis with acquired heart block due to systemic lupus erythematosus: experience in a mother with a predominance of SSB (La) antibodies. Arthritis Rheum 1987; 30: 4449.CrossRefGoogle Scholar
25. Copel, JA, Buyon, JP, Kleinman, CS. Successful in utero therapy of fetal heart block. Am J Obstet Gynecol 1995; 173: 13841390.CrossRefGoogle ScholarPubMed
26. Saleeb, S, Copel, J, Friedman, D, et al. 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: 23352345.3.0.CO;2-3>CrossRefGoogle Scholar
27. Breur, JM, Visser, GHA, Kruize, AA, Steoutenbeek, O, Meijboom, . Treatment of fetal heart block with maternal steroid therapy: case report and review of the literature. Ultrasound Obstet Gynecol 2004; 24: 467472.CrossRefGoogle ScholarPubMed
28. Brucato, A, Gasparini, M, Vignati, G, et al. Isolated congenital complete heart block. Longterm outcome of children and immunogenetic study. J Rheumatol 1995; 22: 541543.Google ScholarPubMed
29. Brucato, A, Li Destri, M, Astori, MG, et al. Normal neuropsychological development of children with congenital complete heart block exposed or not exposed in utero to dexamethasone. Lupus 2004; 13: 762 (abs).Google Scholar
30. Tincani, A, Rebaioli, CB, Frassi, M, et al. Pregnancy and autoimmunity: maternal treatment and maternal disease influence on pregnancy outcome. Autoimmun Rev 2005; 4: 423428.CrossRefGoogle ScholarPubMed
31. Udink ten Cate, FE, Breur, JM, Cohen, MI, et al. Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children. J Am Coll Cardiol 2001; 37: 11291134.CrossRefGoogle ScholarPubMed