title: Fetal Doppler echocardiographic diagnosis and successful steroid therapy of Luciani-Wenckebach phenomenon and endocardial fibroelastosis related to maternal anti-Ro and anti-La antibodies.
abstract: BACKGROUND
  Complete fetal heart block (HB) and endocardial fibroelastosis (EFE) are known to be associated with maternal anti-Ro and anti-La antibodies. Complete fetal HB is irreversible.
  
  
  OBJECTIVE
  We sought to (1) assess the value of the superior vena cava/ascending aorta Doppler approach in the early detection of abnormal delay in the fetal atrioventricular (AV) time of conduction, before appearance of complete fetal HB; and (2) report the effect of prenatal steroid therapy on EFE, HB, or both.
  
  
  RESULTS
  The clinical history, echocardiographic, and Doppler investigations of 3 fetuses and children born to mothers positive for anti-Ro and anti-La antibodies are reported. Two fetuses presented with EFE either isolated (29 weeks) or associated with AV block (25 weeks). In this last case, the superior vena cava/ascending aorta approach allowed the identification of a Luciani-Wenckebach phenomenon. In a third fetus, 2:1 AV block was noted at 23 weeks of gestation. Dexamethasone (4 mg/day) was administered to all 3 patients. Complete regression of the EFE and conduction abnormalities was documented in all cases.
  
  
  CONCLUSIONS
  Early prenatal detection of abnormal delay in fetal AV time conduction is possible with the Doppler superior vena cava/ascending aorta approach. Steroid therapy can cure fetal EFE and AV conduction delays associated with maternal anti-Ro and anti-La antibodies.
authors.affiliation: Department of Pediatrics, St-Justine Hospital, Montreal, Quebec, Canada.
authors.forename: Marie-Josée
authors.initials: MJ
authors.lastname: Raboisson
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Fouron
authors.forename: Sven-Erik
authors.initials: SE
authors.lastname: Sonesson
authors.forename: Margareta
authors.initials: M
authors.lastname: Nyman
authors.forename: Francine
authors.initials: F
authors.lastname: Proulx
authors.forename: Sylvie
authors.initials: S
authors.lastname: Gamache
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D003907:Dexamethasone / Q000627:therapeutic use; D015150:Echocardiography, Doppler; D004695:Endocardial Fibroelastosis / Q000000981:diagnostic imaging* / Q000276:immunology; D005260:Female; D005333:Fetus / Q000276:immunology; D005938:Glucocorticoids / Q000627:therapeutic use; D006327:Heart Block / Q000000981:diagnostic imaging* / Q000276:immunology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008431:Maternal-Fetal Exchange; D011247:Pregnancy; D011256:Pregnancy Outcome; D016216:Ultrasonography, Prenatal
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D005938:Glucocorticoids; D003907:Dexamethasone