Zusammenfassung
Hintergrund: Evaluation des klinischen Verlaufes von Feten mit vermuteten oder bewiesenen Bradykardien auf dem Boden fetaler struktureller Herzfehler oder maternaler Kollagenose mit und ohne Therapie. Methode: Retrospektive Auswertung von fetalen Echokardiographien bei 14 Schwangerschaften (medianes Gestationsalter 25.5, Bereich 19 - 36 Schwangerschaftswochen). Die Indikation zur Untersuchung bestand in einer mütterlichen Kollagenose oder in einer fetalen Bradyarrhythmie mit oder ohne Verdacht auf Herzfehler. Der Nachweis mütterlicher Antikörper gegen Ribonukleinsäure (Anti-Ro/SS-A, Anti-La/SS-B) erfolgte mit ELISA. Ergebnisse: Die 14 untersuchten Feten wiesen eine Bradyarrhythmie oder einen AV-Block auf, bei 7 in Verbindung mit einem Herzfehler, bei 7 ohne strukturelle kardiale Anomalie mit Nachweis von maternalen Ro/La-Antikörpern. 6/7 Feten mit Vitium und 2/7 mit maternalen Autoantikörpern verstarben perinatal. Eine maternale Steroidtherapie hatte keinen Einfluss auf die Rhythmusstörung. Schlussfolgerung: Die fetale Echokardiographie bietet eine sichere Möglichkeit, Bradyarrhythmien und atrioventrikuläre Blocks zu erkennen. Assoziiert mit einem Herzfehler ist die fetale Prognose in unserem Kollektiv deutlich schlechter als in Kombination mit maternaler Kollagenose.
Abstract
Background: The purpose of this study was to evaluate the clinical outcome of fetuses with bradyarrhythmias or complete heart block (CHB) in the setting of fetal structural heart disease (CHD) or of maternal collagenosis with and without treatment. Methods: A retrospective analysis of echocardiographic studies performed in 14 fetuses (mean gestational age 25.5, range 19 - 36 weeks) referred for exclusion or diagnosis of bradyarrhytmias was performed. Maternal SS-A/Ro and SS-B/La antibodies were measured by ELISA. Results: 14 fetuses showed bradyarrhythmias or complete heart block in combination with severe cardiac malformations (n = 7) or with positive maternal antibodies (n = 7). Only one of the fetuses with CHD survived infancy as opposed to 5/7 fetuses with complete atrioventricular block in the setting of maternal collagenosis. Maternal treatment with corticosteroids did not seem to influence the rhythm disorder. Conclusion: Fetal echography is a safe method to detect bradyarrhythmias or complete atrioventricular block. When associated with structural heart defects, fetal prognosis is poorer than in combination with maternal collagenosis.
Schlüsselwörter
Kongenitaler atrioventrikulärer Block - fetale Doppler-Echokardiographie - Autoantikörper
Keywords
Congenital atrioventricular block - fetal doppler echocardiography - autoantibodies
Literatur
-
1
Allan L D.
Research protocol for fetuses with complete heart block.
Ultrasound Obstet Gynecol.
1995;
5
349-352
-
2
Allan L D, Anderson R H, Sullivan I D, Campbell S, Holt D W, Tynan M.
Evaluation of fetal arrhythmias by echocardiography.
Br-Heart-J.
1983;
50
240-5
-
3
Buyon J P, Waltuck J, Kleinman C, Copel J.
In utero identification and therapy of congenital heart block.
Lupus.
1995;
4(2)
116-21
-
4
Frohn-Mulder I M, Meilof J F, Szatmari A, Stewart P A, Swaak T J, Hess J.
Clinical significance of maternal anti-Ro/SS-A antibodies in children with isolated heart block.
J-Am-Coll-Cardiol.
1994;
23(7)
1677-81
-
5
Meilof J F, Frohn-Mulder M, Stewart P A, Szatmari A, Hess J, Veldhoven C H, Smeenk R J, Swaak A J.
Maternal autoantibodies and congenital heart block: no evidence for the existence of a unique heart block-associated anti-Ro/SS-A autoantibody profile.
Lupus.
1993;
2(4)
239-46
-
6
Buyon J P, Swersky S H, Fox H E, Bierman F Z, Winchester R J.
Intrauterine therapy for presumptive fetal myocarditis with acquired heart block due to systemic lupus erythematosus. Experience in a mother with a predominance of SS-B (La) antibodies.
Arthritis-Rheum.
1987;
30(1)
44-9
-
7 Rosenthal E. Fetal heart block. In: Allen L, Hornberger L, Sharland BSc, eds Textbook of Fetal Cardiolgy. London; GMM 2000: 438-451
-
8
Southall D P, Richards J, Hardwick R A, Shinebourne E A, Gibbens G L, Thelwall-Jones H, de-Swiet M, Johnston P G.
Prospective study of fetal heart rate and rhythm patterns.
Arch-Dis-Child.
1980;
55(7)
506-11
-
9
Silverman N H, Enderlein M A, Stanger P, Teitel D F, Heymann M A, Golbus M S.
Recognition of fetal arrhythmias by echocardiography.
J-Clin-Ultrasound.
1985;
13(4)
255-63
-
10
Calvin S E, Gaziano E P, Bendel R P, Knox G E, Brandt D G.
Evaluation of fetal cardiac arrhythmias. Ultrasound findings and neonatal outcome.
Minn-Med.
1992;
75(12)
29-31
-
11
Askanase A D, Friedmann D M, Copel J, Dische M R, Dubin A, Starc T J, Katholi M C, Buyon J P.
Spectrum and progression of conduction abnormalities in infants born to mothers with anti-SSA/Ro-SSA/La antibodies.
Lupus.
2002;
11
145-151
-
12
Ross B A.
Congenital complete atrioventricular block.
Pediatr-Clin-North-Am.
1990;
37(1)
69-78
-
13
Brucato A, Jonzon A, Friedman D, Allan L D, Vignati G, Gasparini M, Stein J I, Montella S, Michaelsson M, Buyon J.
Proposol for a new definition of congenital complete atrioventricular block.
Lupus.
2003;
12
427-435
-
14
Groves A M, Allan L D, Rosenthal E.
Outcome of isolated congenital complete heart block diagnosed in utero.
Heart.
1996;
75(2)
190-4
-
15
Schmidt K G, Ulmer H E, Silverman N H, Kleinman C S, Copel J A.
Perinatal outcome of fetal complete atrioventricular block: a multicenter experience.
J-Am-Coll-Cardiol.
1991;
17(6)
1360-6
-
16
Ramsey-Goldmann R, Hom D, Deng J S . et al .
Anti-SSA antibodies and fetal outcome in maternal systemic lupus erythematosus.
Arthritis & Rheumatism.
1986;
29
1269-1273
-
17
Brucato A.
Risk of congenitalcomplete heart block in newborns of mothers with anti-Ro/SSA antibodies detected by counterimmunoelectrophoresis.
Arthritis & Rheumatism.
2001;
44
1832-1835
-
18
Cimaz R, Spence D L, Hornberger L, Silverman E D.
Incidence and spectrum of neonatal lupus erythematosus. A prospective study of infants born to mothers with anti-Ro autoantibodies.
J Pediatr.
2003;
142
678-683
-
19
Friedmann D M, Rupel A, Glickstein J, Buyon J P.
Congenital heart block in neonatal lupus: the pediatric cardiologistŽs perspective.
Indian J Pediatr.
2002;
69(6)
517-22
-
20
Buyon J P, Hiebert R, Copel J, Craft J, Friedmann D, Katholi M, Lee L, Provost P, Reichlin M, Rider L, Rupel A, Saleeb S, Weston W, Skovron M L.
Autoimmune associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry.
JACC.
1998;
31(7)
1658-1666
-
21
Friedman R A.
Congenital AV block. Pace me now or pace me later?.
[editorial; comment] Circulation.
1995;
92(3)
283-5
-
22 Wells M, Fox H. Immunology and Immunopathology of the maternofetal interface. In: Coulam CB, Faulk WP, McIntyre JA, eds Immunological Obstetrics. New York; WW Norton & Co 1992: 166-172
-
23
Dörner T, Feist E, Pruss A, Chaoui R, Göldner B, Hiepe F.
Significance of autoantibodies in neonatal lupus erythematosus.
Int Arch Allergy Immunol.
2000;
123
58-66
-
24
Siren M K, Julkunen H, Kaaja R, Koskimies S.
Congenital heart block: HLA differences between affected children and healthy siblings in four Finnish families.
APMIS.
1997;
105(6)
463-8
-
25
Smeenk R J.
Immunological aspects of congenital atrioventricular block.
Pacing-Clin-Electrophysiol.
1997;
20(8 Pt 2)
2093-7
-
26
Saleeb S, Copel J, Friedman D, Buyon J P.
Comparison of treatment with fluorinated glucocorticoids to the natural history of autoantibody associated congenital heart block.
Arthritis & Rheumatism.
1999;
42(11)
2335-2345
-
27 Wimmer A G, Oberhoffer R, Schneider K TM, Fleck M, Schölmerich J, Müller-Ladner U. Interdisziplinäre Betreuung von schwangeren Patienten mit Kollagenose (zur Publikation eingereicht).
-
28
Julkunen H, Kaaja R, Siren M, Mack C, McCready S, Holthöfer H, Kurki P, Maddison P.
Immune Mediated Congenital Herat Block (CHB): Identifying and counseling Patients at risk for having children with CHB.
Semin Arthritis Rheum.
1998;
28
97-106
-
29
Eronen M, Sirèn M, Ekblad H, Tikanoja T, Julkunen H, Paavilainen T.
Short- and Long-Term Outcome of Children With Congenital Complete Heart Block Diagnosed In Utero or as a Newborn.
Pediatrics.
2000;
106
86-91
-
30
Jaeggi E T, Hamilton R M, Silvermann E D, Zamora S A, Hornberger L K.
Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block.
J Am Coll Cardiol.
2002;
39
130-7
Dr. Martin Tollens
Kinderklinik und Poliklinik der Technischen Universität München
Kölner Platz 1
D-80804 München
Phone: 089-3068-2272
Fax: 089-3068-3954
Email: Tollens@lrz.tu-muenchen.de
