Failure of intravenous immunoglobulin to prevent congenital heart block: Findings of a multicenter, prospective, observational study.
OBJECTIVE
Congenital heart block (CHB) is presumed to be caused by transplacental passage of maternal immunoglobulin against Ro and La ribonucleoproteins. The recurrence rate in subsequent pregnancies following the birth of a child with CHB is approximately 19%. The purpose of this study was to determine whether intravenous immunoglobulin (IVIG) therapy could prevent the development of CHB in the fetuses of high-risk pregnant women.


METHODS
A total of 24 pregnancies in 22 women who had a previous pregnancy in which CHB developed, were over the age of 18 years, were <12 weeks pregnant, and had anti-Ro, anti-La, or both antibodies were monitored in this multicenter, prospective, observational study. Fifteen patients received infusions of IVIG. The 9 pregnancies in the remaining 7 patients served as controls. IVIG was administered at a dose of 400 mg/kg at weeks 12, 15, 18, 21, and 24 of pregnancy. Echocardiograms were performed at least every 3 weeks from week 15 to week 30 of gestation. Electrocardiograms were obtained at birth. The outcome measure was the development of third-degree CHB detected by fetal echocardiogram.


RESULTS
CHB developed in 3 babies among the 15 pregnancies in the treatment group (20%) and in 1 baby among the 9 pregnancies in the control group (11%). CHB was detected at weeks 18, 23, and 26, respectively, in the 3 babies in the treated group and at week 19 in the baby in the control group. Three of the affected pregnancies ended in termination; 2 for reasons related to the fetal disease and 1 for reasons related to both maternal (severe pulmonary hypertension) and fetal disease (at 21 weeks of gestation).


CONCLUSIONS
IVIG at the dose and frequency used in this study was not effective as prophylactic therapy for CHB in high-risk mothers.
Pisoni
C N
CN
St. Thomas' Hospital, and King's College London, London, UK. ceciliapisoni@gmail.com
Brucato
A
A
Ruffatti
A
A
Espinosa
G
G
Cervera
R
R
Belmonte-Serrano
M
M
Sánchez-Román
J
J
García-Hernández
F G
FG
Tincani
A
A
Bertero
M T
MT
Doria
A
A
Hughes
G R V
GR
Khamashta
M A
MA
D001324:Autoantigens / Q000276:immunology; D003907:Dexamethasone / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006327:Heart Block / Q000517:prevention & control*; D006330:Heart Defects, Congenital / Q000276:immunology* / Q000517:prevention & control; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007223:Infant; D007231:Infant, Newborn; D011241:Prednisone / Q000627:therapeutic use; D011247:Pregnancy; D011446:Prospective Studies; D044469:Racial Groups; D012008:Recurrence; D012261:Ribonucleoproteins / Q000276:immunology; D017211:Treatment Failure; D000097585:SS-B Antigen; D000099207:SS-A Antigen
D001324:Autoantigens; D003907:Dexamethasone; D006886:Hydroxychloroquine; D016756:Immunoglobulins, Intravenous; D011241:Prednisone; D012261:Ribonucleoproteins; D000097585:SS-B Antigen; D000099207:SS-A Antigen