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Arrhythmogenic right ventricular dysplasia/cardiomyopathy as a cause of sudden infant death

Gaetano Nucifora1, Alessandra Benettoni, Giuseppe Allocca

  • 1Pediatric Cardiology Unit, IRCCS, Istituto per l'Infanzia, Burlo Garofolo, Trieste, Italy. gnucifora@cardionet.it

Journal of Cardiovascular Medicine (Hagerstown, Md.)
|March 13, 2008
PubMed

Insights

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is a rare cause of infant sudden death. A postmortem examination confirmed ARVC in a 4-month-old male infant who died unexpectedly in his sleep.

Area of Science:

  • Cardiology
  • Pediatric Pathology

Background:

  • Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is an inherited heart muscle disease.
  • It is characterized by fibrofatty replacement of the right ventricular myocardium.
  • ARVC is an uncommon cause of sudden cardiac death, particularly in infants.

Observation:

  • A case report of a 4-month-old male infant is presented.
  • The infant experienced sudden, unexplained death during sleep.
  • Autopsy was performed to determine the cause of death.

Findings:

  • Postmortem examination revealed findings consistent with arrhythmogenic right ventricular dysplasia/cardiomyopathy.
  • Histopathological analysis confirmed the fibrofatty infiltration of the right ventricular wall.

Implications:

  • This case highlights ARVC as a potential, albeit rare, cause of sudden infant death.
  • Early recognition and diagnosis of ARVC in infants may be crucial for risk stratification and family screening.
  • Further research into the genetic and clinical spectrum of ARVC in the pediatric population is warranted.

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