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Related Experiment Videos

Neonatal lupus.

Antonio Brucato1, Rolando Cimaz, Marco Stramba-Badiale

  • 1Divisione Medica Brera and Rheumatology, Ospedale Niguarda, Milan, Italy. brucato.guareschi@libero.it

Clinical Reviews in Allergy & Immunology
|October 31, 2002
PubMed
Summary

Congenital heart block (CHB) is a rare disorder linked to maternal anti-Ro/SSA and anti-La/SSB antibodies. Early diagnosis and monitoring in at-risk pregnancies are crucial for fetal outcomes.

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Area of Science:

  • Cardiology
  • Immunology
  • Neonatology

Background:

  • Congenital heart block (CHB) is a rare condition diagnosed in utero or neonatally.
  • It is strongly associated with maternal antibodies anti-Ro/SSA and anti-La/SSB, crossing the placenta.
  • These antibodies can cause fetal myocarditis, conduction system disturbances, or apoptosis, impacting fetal survival and long-term health.

Purpose of the Study:

  • To summarize the pathophysiology, clinical features, recurrence risks, and management recommendations for congenital heart block.
  • To highlight the importance of maternal antibody screening and fetal monitoring in at-risk pregnancies.

Main Methods:

  • Review of existing literature on congenital heart block, maternal autoantibodies, and fetal cardiac assessment.
  • Analysis of clinical features, diagnostic criteria, and management strategies for CHB.
  • Evaluation of recurrence rates and maternal/neonatal outcomes.

Main Results:

  • CHB is linked to maternal anti-Ro/SSA and anti-La/SSB antibodies, potentially causing fetal myocarditis or conduction abnormalities.
  • Survivors may require pacemakers; transient features include skin lesions, hematologic disorders, and hepatic cholestasis.
  • Recurrence risk is 10-17%; most mothers are asymptomatic.
  • Prevalence of CHB in newborns of anti-Ro/SSA positive women with connective tissue disease is 2%.

Conclusions:

  • Serial echocardiograms and obstetric sonograms are recommended for anti-Ro/SSA positive pregnant women starting at 16 weeks gestation.
  • While a standard in-utero therapy for CHB is lacking, vigilant monitoring and early detection are vital.
  • Maternal long-term outcomes are generally reassuring, with arthralgias and dry eyes being common symptoms.

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