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Published on: August 30, 2024
Arrhythmogenic right ventricular dysplasia.
1Baltimore, Maryland, USA. hcalkins@jhmi.edu
Summary
Arrhythmogenic right ventricular dysplasia (ARVD) primarily affects the right ventricle, though rare left ventricular involvement occurs. The "accordion sign" on cardiac MRI shows promise for early ARVD diagnosis.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD) is a genetic heart condition causing ventricular arrhythmias and right ventricular (RV) abnormalities.
- Desmosomal mutations are linked to ARVD, prompting investigation into potential left ventricular (LV) involvement.
Purpose of the Study:
- To provide an overview of ARVD.
- To investigate morphological variants of ARVD using cardiac magnetic resonance imaging (CMR).
Main Methods:
- Genotyping and CMR were performed on 38 family members of ARVD probands.
- CMR investigators were blinded to genetic and clinical data.
- Analysis focused on RV and LV abnormalities, particularly the "accordion sign".
Main Results:
- Twenty-five individuals carried mutations (PKP2, DSP, DSG2).
- RV abnormalities correlated with mutation presence and ARVD diagnostic criteria severity.
- The "accordion sign" was observed in 60% of mutation carriers versus 0% of non-carriers (P<0.001) and increased with ARVD severity.
- Intramyocardial fat was the only LV abnormality detected, present in four mutation carriers.
Conclusions:
- While LV involvement in ARVD is possible, it is rare, and overall LV structure/function remain preserved.
- The "accordion sign" shows potential as an early diagnostic tool for ARVD.
- Further validation in larger patient cohorts is recommended.
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