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Absence of pathognomonic or inflammatory patterns in cardiac biopsies from patients with Brugada syndrome

Sven Zumhagen1, Tilmann Spieker, Julia Rolinck

  • 1Department of Cardiology and Angiology, Hospital of the University of Münster, Münster, Germany.

Circulation. Arrhythmia and Electrophysiology
|October 8, 2009
PubMed

Insights

Brugada syndrome (BrS) patients often show moderate, uncharacteristic right ventricular abnormalities on imaging and biopsy. Few patients had significant histopathologic changes, suggesting diverse causes for the Brugada ECG phenotype.

Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Brugada syndrome (BrS) presents with specific ECG changes and is linked to ion channel gene mutations.
  • BrS can cause conduction disturbances and ventricular fibrillation.
  • The underlying cause of BrS, whether purely electrical or involving right ventricular (RV) histopathology, is debated.

Purpose of the Study:

  • To investigate histopathologic abnormalities in the RV of BrS patients.
  • To correlate histopathologic findings with clinical, genetic, and imaging data.

Main Methods:

  • Retrospective analysis of RV biopsy samples from 21 BrS patients.
  • Evaluation for hypertrophy, fibrosis, inflammation, and fatty tissue.
  • Correlative analysis with clinical, genetic, and imaging data (angiography, echocardiography, cardiac MRI).

Main Results:

  • Most myocardial biopsies showed moderate, uncharacteristic abnormalities.
  • Predominant fatty tissue was found in 19% of RV myocardium.
  • No evidence of inflammatory tissue changes or myocarditis was detected.

Conclusions:

  • Moderate cardiac abnormalities are detectable in BrS patients but are uncharacteristic.
  • Arrhythmogenic RV cardiomyopathy and overt myocarditis were not observed.
  • Specific RV histopathologic changes were rare, suggesting multifactorial causes for the Brugada ECG phenotype.
Abstract

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