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Updated: May 13, 2026

Design and Synthesis of a Reconfigurable DNA Accordion Rack
Published on: August 15, 2018
An accordion not to be played
Riccardo Gorla1, Ornella Rimoldi, Francesco De Cobelli
1aCardio-Thoracic and Vascular Department, San Raffaele Hospital and Vita-Salute University San Raffaele bIBFM CNR cRadiology Department, San Raffaele Hospital and Vita-Salute University San Raffaele, Milan, Italy.
This case study highlights arrhythmogenic cardiomyopathy, a rare heart condition. Advanced imaging confirmed a left-dominant variant, crucial for understanding ventricular fibrillation causes.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Arrhythmogenic cardiomyopathy (ACM) is a genetic heart muscle disease.
- It typically affects the right ventricle but can involve the left.
Observation:
- A 52-year-old man presented with ventricular fibrillation and normal coronary arteries.
- Initial echocardiography suggested left-ventricular non-compaction.
- Cardiac MRI revealed right ventricular systolic bulgings and the 'accordion' sign in the outflow tract.
Findings:
- The 'accordion' sign is a specific marker for desmosomal gene mutations.
- Late gadolinium enhancement and fatty infiltration confirmed left-dominant ACM.
- This diagnosis is a rare variant of arrhythmogenic right-ventricular cardiomyopathy.
Implications:
- Accurate diagnosis of ACM variants is vital for risk stratification and management.
- Identifying desmosomal gene mutations aids in genetic counseling.
- This case expands understanding of ACM presentation and imaging findings.
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