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Published on: May 5, 2018

An expanded phenotype of maternal SSA/SSB antibody-associated fetal cardiac disease

Bettina F Cuneo1, Janette F Strasburger, Alisa Niksch

  • 1The Heart Institute for Children, Hope Children's Hospital, Oak Lawn, IL 60453, USA. cuneo@thic.com

The Journal of Maternal-Fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians
|March 31, 2009
PubMed

Insights

This study details rare cardiac conditions in fetuses exposed to Sjogren's antibodies (SSA/SSB). Findings expand the known spectrum of fetal cardiac disease linked to maternal autoantibodies.

Area of Science:

  • Cardiology
  • Immunology
  • Neonatology

Background:

  • Maternal anti-Sjogren's syndrome A/B (SSA/SSB) antibodies are linked to fetal cardiac disease.
  • Typical manifestations include atrioventricular block, bradycardia, endocardial fibroelastosis, and dilated cardiomyopathy.

Observation:

  • Three fetuses with unique myocardial and conduction system abnormalities were studied.
  • Case 1: Isolated endocardial fibroelastosis with subsequent valve chordal avulsion, leading to severe neonatal disease.
  • Case 2: Sinoatrial and infrahissian conduction system disease, progressing to life-threatening neonatal illness.
  • Case 3: Sinus node dysfunction and atrial flutter.

Findings:

  • The study identified novel presentations of fetal cardiac disease associated with SSA/SSB antibodies.
  • These include isolated endocardial fibroelastosis with valve complications and complex conduction system defects.
  • Neonatal progression to critical illness was observed in two cases.

Implications:

  • These findings broaden the recognized clinical spectrum of SSA/SSB antibody-associated fetal cardiac disease.
  • Highlights the need for increased awareness and surveillance for atypical cardiac manifestations.
  • Informs potential diagnostic and management strategies for affected pregnancies.
Abstract

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