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Congenital heart block in neonatal lupus: The pediatric cardiologist’s perspective

  • Symposium on Pediatric Cardiology-II
  • Published:
The Indian Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Clinical presentation. Congenital heart block (CHB) in the absence of major structural abnormalities is associated with maternal antibodies to Ro (SS-A) and La (SS-B). CHB is most commonly diagnosed between 18 and 24 wk of gestation, and may be first, second or third degree (complete). Mortality approaches ∼20%, and most surviving children require pacemakers. Affected infants may develop cardiomyopathy. Abnormalities in the skin, liver and blood of neonates are also associated with anti-Ro/La antibodies, and are usually self-limiting; these manifestations and CHB are collectively referred to as neonatal lupus syndromes (NLS).Investigation of pathogenesis. Recent studies demonstrate that Ro/La ribonucleoproteins appear on the surface of apoptotic fetal cardiocytes and are recognized by their cognate antibodies, promoting an inflammatory response. Mice immunized with Ro/La proteins have offspring with conduction abnormalities.In vitro, human serum and IgG with anti-Ro/La antibodies affect the conducting properties of isolated animal heart tissue.Diagnostic problems. If fetal bradycardia is identified, a 2-dimensional and M-mode fetal echocardiographic and Doppler ultrasound should be obtained to determine whether there is an atrial arrhythmia or atrioventricular (AV) block, and to what degree, and whether there are major structural abnormalities of the heart. The mother’s serum should be tested by ELISA for anti-Ro and/or anti-La antibodies.Therapeutic options. To date, only anecdotal and retrospective evidence guidesin utero therapy of CHB. A prospective trial is currently underway to evaluate the efficacy of maternal oral dexamethasone in treating newly identified first, second or third degree block. Established third-degree block appears to be irreversible. Dexamethasone and sympathomimetics may be of some benefit in treating hydrops fetalis. In pregnant women with anti-Ro/La antibodies, prophylactic therapy is not indicated but serial echocardiographic analysis is strongly recommended, with emphasis on the mechanical PR interval to identify a reversible block.Conclusion. CHB occurs in ∼1–5% of pregnancies in mothers with antiRo/La antibodies, independent of the mother’s disease status, and in ∼15–20% of pregnancies following the birth of a child with NLS. Treatment of CHB identifiedin utero is not established but guidelines are provided. Serial echocardiographic monitoring of high-risk pregnancies, using the mechanical PR interval to identify first degree block, may afford the earliest opportunities for therapeutic intervention

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Department of Pediatrics, St. Luke’s-Roosevelt Hospital Center, New York

    Deborah M. Friedman

  2. Department of Rheumatology, Hospital for Joint Diseases, New York University School of Medicine, New York

    Ann Rupel & Jill P. Buyon

  3. Division of Pediatric Cardiology, Department of Pediatrics, Children Hospital of New York, Presbyterian Hospital, Columbia University College of Physicians and Surgeons, New York, New York, USA

    Julie Glickstein

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  1. Deborah M. Friedman
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  2. Ann Rupel
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  4. Jill P. Buyon
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Correspondence to Deborah M. Friedman.

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Friedman, D.M., Rupel, A., Glickstein, J. et al. Congenital heart block in neonatal lupus: The pediatric cardiologist’s perspective. Indian J Pediatr 69, 517–522 (2002). https://doi.org/10.1007/BF02722656

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