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Congenital heart disease linked to maternal autoimmunity against cardiac myosin

Charles R Cole1, Katherine E Yutzey, Anoop K Brar

  • 1Division of Molecular Cardiovascular Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229;

Journal of Immunology (Baltimore, Md. : 1950)
|March 28, 2014
PubMed

Insights

This study introduces a novel autoimmune model for congenital heart disease (CHD), specifically hypoplastic left heart syndrome (HLHS). It suggests maternal autoantibodies against cardiac myosin may cause prenatal HLHS in fetuses.

Area of Science:

  • Immunology
  • Cardiology
  • Developmental Biology

Background:

  • Structural congenital heart disease (CHD) lacks established links to autoimmunity.
  • Hypoplastic left heart syndrome (HLHS) is a severe left ventricular defect with unknown pathogenesis.
  • Cardiac myosin (CM) is a known autoantigen in autoimmune heart conditions.

Purpose of the Study:

  • To develop an autoimmune model of structural CHD resembling HLHS.
  • To investigate the role of maternal autoantibodies against CM in prenatal HLHS development.
  • To explore potential immune-mediated mechanisms in HLHS pathogenesis.

Main Methods:

  • Developed a maternal-fetal autoimmune model of CHD using CM immunization.
  • Assessed maternal and fetal serum autoantibody titers (anti-CM, anti-β-adrenergic receptor).
  • Examined IgG reactivity in fetal myocardium and cardiac protein kinase A activity.

Main Results:

  • Maternal immunization with CM induced anti-CM autoantibodies that crossed the placenta.
  • Elevated maternal and fetal anti-CM autoantibodies correlated with structural CHD and diminished left ventricular dimensions.
  • Fetuses with HLHS phenotype showed increased anti-β-adrenergic receptor Abs and protein kinase A activity.

Conclusions:

  • Presents the first evidence of an immune-mediated mechanism for structural CHD pathogenesis, specifically HLHS.
  • Suggests autoimmunity against CM may play a role in HLHS development via transplacental autoantibody transfer.
  • Highlights potential implications for future diagnosis and treatment of HLHS through an immune-mediated lens.

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