Failure of intravenous immunoglobulin to prevent congenital heart block: Findings of a multicenter, prospective, observational study

Arthritis Rheum. 2010 Apr;62(4):1147-52. doi: 10.1002/art.27350.

Abstract

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).

Conclusion: IVIG at the dose and frequency used in this study was not effective as prophylactic therapy for CHB in high-risk mothers.

Publication types

  • Multicenter Study

MeSH terms

  • Autoantigens / immunology
  • Dexamethasone / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Heart Block / prevention & control*
  • Heart Defects, Congenital / immunology*
  • Heart Defects, Congenital / prevention & control
  • Humans
  • Hydroxychloroquine / therapeutic use
  • Immunoglobulins, Intravenous / therapeutic use*
  • Infant
  • Infant, Newborn
  • Prednisone / therapeutic use
  • Pregnancy
  • Prospective Studies
  • Racial Groups
  • Recurrence
  • Ribonucleoproteins / immunology
  • Treatment Failure*

Substances

  • Autoantigens
  • Immunoglobulins, Intravenous
  • Ribonucleoproteins
  • SS-A antigen
  • SS-B antigen
  • Hydroxychloroquine
  • Dexamethasone
  • Prednisone