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Sudden death with circumferential subepicardial fibrofatty replacement: left-sided arrhythmogenic ventricular

Shannon M Mackey-Bojack1, Susan J Roe, Jack L Titus

  • 1Jesse E Edwards Registry of Cardiovascular Disease, John Nasseff Heart Hospital, Saint Paul, MN 55102, USA.

The American Journal of Forensic Medicine and Pathology
|May 26, 2009
PubMed

Insights

This study details 5 young sudden cardiac death cases with left ventricular fibrofatty replacement, resembling arrhythmogenic right ventricular cardiomyopathy (ARVC). The findings suggest a potential spectrum of arrhythmogenic cardiomyopathy or a distinct inheritable condition.

Area of Science:

  • Cardiology
  • Pathology
  • Genetics

Background:

  • Sudden cardiac death (SCD) in the young is a critical concern.
  • Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a known cause of SCD.
  • Histological features of ARVC typically involve the right ventricle.

Purpose of the Study:

  • To describe 5 cases of SCD with unique cardiac findings.
  • To investigate the potential relationship between these findings and ARVC.
  • To consider a broader definition of arrhythmogenic cardiomyopathy or a new inheritable entity.

Main Methods:

  • Case series reporting 5 instances of sudden cardiac death.
  • Detailed cardiac histopathological examination of all cases.
  • Review of family histories for cardiac symptoms or sudden death.

Main Results:

  • All 5 cases exhibited circumferential left ventricular subepicardial fibrofatty replacement.
  • Cardiac findings predominantly affected the left ventricle, with minimal right ventricular involvement.
  • Four cases had siblings with a history of sudden death or cardiac issues.

Conclusions:

  • The findings in these cases resemble ARVC but predominantly involve the left ventricle.
  • These cases may represent a wider spectrum of arrhythmogenic cardiomyopathy, potentially termed "arrhythmogenic cardiomyopathy."
  • Alternatively, these cases could indicate a separate, inheritable cardiomyopathic condition.

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