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[Arrhythmogenic right ventricular dysplasia].

Carmen Grigoriu1, Doina Butcovan, V Astărăstoae

  • 1Disciplina de Medicină Legală, Facultatea de Medicină, Universitatea de Medicină şi Farmacie Gr.T. Popa Iaşi.

Revista Medico-Chirurgicala a Societatii De Medici Si Naturalisti Din Iasi
|February 21, 2004
PubMed
Summary

Arrhythmogenic right ventricular dysplasia (ARVD) is a heart muscle disorder characterized by fibro-fatty tissue replacement in the right ventricle. This condition is an underrecognized cause of sudden cardiac death, particularly in young individuals.

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Area of Science:

  • Cardiology
  • Pathology
  • Genetics

Context:

  • Arrhythmogenic right ventricular dysplasia (ARVD) is a cardiomyopathy with unknown incidence.
  • Pathologically, it involves fibro-fatty replacement of the right ventricular myocardium.
  • ARVD is a significant cause of sudden cardiac death in young, healthy individuals.

Purpose:

  • To highlight the underrecognized frequency and pathological characteristics of ARVD.
  • To emphasize the importance of histological examination of the right ventricle in sudden death cases.
  • To inform about the familial nature and inheritance pattern of ARVD.

Summary:

  • ARVD is a heart muscle disorder characterized by fibro-fatty myocardial replacement, predominantly affecting the right ventricle.
  • While often familial with autosomal dominant inheritance (approx. 30%), its exact incidence remains unknown.

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  • It is a critical, though potentially underreported, cause of sudden unexpected cardiac death, especially in young, active people.

    • Impact:

    • Increased awareness of ARVD as a cause of sudden cardiac death.
    • Recommendations for comprehensive histological sampling in unexplained deaths.
    • Potential for improved diagnosis and genetic counseling in affected families.