Fetal Doppler echocardiographic diagnosis and successful steroid therapy of Luciani-Wenckebach phenomenon and endocardial fibroelastosis related to maternal anti-Ro and anti-La antibodies.
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.
Raboisson
Marie-Josée
MJ
Department of Pediatrics, St-Justine Hospital, Montreal, Quebec, Canada.
Fouron
Jean-Claude
JC
Sonesson
Sven-Erik
SE
Nyman
Margareta
M
Proulx
Francine
F
Gamache
Sylvie
S
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
D001323:Autoantibodies; D001324:Autoantigens; D005938:Glucocorticoids; D003907:Dexamethasone