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[Polycystic endodermal heterotopy of the atrioventricular node]

F J Monzón Muñoz1, B Aguilera Tapia, J M Martínez-Peñuela Virseda

  • 1Servicio de Anatomía Patólogica, Hospital de Navarra, Pamplona.

Medicina Clinica
|February 25, 1995
PubMed
Summary

This study details two Spanish cases of endodermic heterotopia of the atrioventricular node, a rare congenital heart condition. The lesion, linked to heart blocks and sudden death, showed endodermic differentiation in both patients.

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Area of Science:

  • Cardiovascular Pathology
  • Developmental Biology
  • Histopathology

Background:

  • Endodermic heterotopia of the atrioventricular node is an infrequent congenital lesion with poorly understood histogenesis.
  • This condition is associated with heart blocks and sudden cardiac death, predominantly affecting women.

Observation:

  • Two cases in Spain are presented: a 55-year-old woman with acute leukemia and a 25-year-old woman with pre-existing heart blocks.
  • Both patients died suddenly, and comprehensive post-mortem examinations, including cardiac conduction system analysis, were performed.
  • Histopathological and ultrastructural studies revealed multicystic and tubular lesions in the atrioventricular node region.

Findings:

  • Lesions were lined by cubic and polyhedral cells, with lumina containing eosinophilic material positive for acid and neutral mucosubstances.

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  • Immunohistochemistry demonstrated strong positivity for cytokeratins, EMA, CEA, and focal positivity for chromogranin A and calcitonin.
  • These findings indicate endodermic differentiation with a neuroendocrine phenotype in both cases.

    • Implications:

    • These cases contribute to the understanding of endodermic heterotopia of the atrioventricular node, particularly its endodermic and neuroendocrine characteristics.
    • The findings highlight the potential association of this lesion with severe cardiac events, including sudden death.
    • Further research into the histogenesis and clinical implications of this rare condition is warranted.