Our clinical report describes a rare finding of a prenatally-detected congenital atrioventricular (AVB) block without associated maternal antibodies, which progressed from 1st/2nd degree AVB to complete heart block during second half of pregnancy. Obstetrical ultrasound at 12th week did not reveal any abnormalities and prenatal echocardiography (due to VSD in afamily member) at the 18th week of gestation detected 1st degree block, then bigeminy and bradycardia. Transplacental treatment with B-2-mimetics was introduced. The delivery was organized in a tertiary center and a pacemaker for the newborn baby was secured and implanted in 15th day of life. Currently the boy`s condition is good and stable. Before therapy with B-2-mimetics the mother underwent echocardiography and ECG which revealed clinically silent structural and conduction heart abnormalities. Literature findings suggest that parents of children with non-immune congenital or childhood AVB are more likely to carry clinically silent conduction abnormalities than general population. Given the corresponding findings in the mother and her son, they should be good candidates for genetic testing.
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1. Brucato A Jonzon A Friedman D Allan LD Vignati G Gasparini M et al. Proposal for a new definition of congenital complete atrioventricular block. Lupus. 2003;12(6):427-35.
2. Michaelsson M Riesenfeld T Jonzon A. Natural history of congenital complete atrioventricular block. Pacing Clin Electrophysiol. 1997;20(8 Pt 2):2098-101.
3. Michaëlsson M Engle MA. Congenital complete heart block: an international study of the natural history. Cardiovasc Clin. 1972;4(3):85-101.
4. Taylor P V Taylor KF Norman A Griffiths S Scott JS. Prevalence of maternal Ro (SS-A) and La (SS-B) autoantibodies in relation to congenital heart block. Br J Rheumatol. 1988 Apr;27(2):128-32.
5. Taylor P V Scott JS Gerlis LM Esscher E Scott O. Maternal antibodies against fetal cardiac antigens in congenital complete heart block. N Engl J Med. 1986 Sep 11;315(11):667-72.
6. Slodki M Szymkiewicz-Dangel J Tobota Z Seligman NS Weiner S Respondek-Liberska M. The Polish National Registry for Fetal Cardiac Pathology: organization diagnoses management educational aspects and telemedicine endeavors. Prenat Diagn. 2012 May;32(5):456-60.
7. Hua M Weiner S. Maternal Conditions Affecting the Fetal Heart. Prenat Cardiol. 2013;3(3):5-11.
8. Respondek-Liberska M Sysa A Gadzinowski J. [The cost of newborns transportation to the referral centers in comparison to the cost of the transport in-utero]. Ginekol Pol. 2004 Apr;75(4):326-31.
9. Eliasson H Sonesson S-E Sharland G Granath F Simpson JM Carvalho JS et al. Isolated atrioventricular block in the fetus: a retrospective multinational multicenter study of 175 patients. Circulation. 2011 Nov 1;124(18):1919-26.
10. Chang Y Hsieh PCC Chang S Chao A Liang CC Soong Y. Perinatal outcome of fetus with isolated congenital second degree atrioventricular block without maternal anti-SSA / Ro-SSB / La antibodies. Eur J Obstet Gynecol Reprod Biol. 2005;122:167-71.
11. Leszczyńska K Chojnicki M Haponiuk I Preis K Ciach K Gierat-Haponiuk K et al. Analiza przebiegu ciąży porodu i losów noworodków z prenatalnie diagnozowanym całkowitym blokiem przedsionkowo-komorowym. Ginekol Pol. 2015;(5):366-71.
12. Jaeggi ET Hamilton RM Silverman ED Zamora SA Hornberger LK. 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(1):130-7.
13. Villain E Coastedoat-chalumeau N Marijon E. Presentation and Prognosis of Complete Atrioventricular Block in Childhood According to Maternal Antibody Status. J Am Coll Cardiol. 2006;48(8):6-11.
14. Baruteau A Behaghel A Fouchard S Mabo P Schott J Dina C et al. Parental Electrocardiographic Screening Identifies a High Degree of Inheritance for Congenital and Childhood Nonimmune Isolated Atrioventricular Block. Circulation. 2012;
15. Syam N Ozhathil LC Sottas V Baruteau A Baron E Amarouch M et al. Variants of Transient Receptor Potential Melastatin Member 4 in Childhood Atrioventricular Block. J Am Heart Assoc. 2016;1-19.