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MRI assessment of right ventricular dysplasia
1Department of Radiology, University of L'Aquila, via Vetoio 1, 67100, L'Aquila, Italy. ernesto.dicesare@cc.univaq.it
Insights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease causing arrhythmias and sudden death. Cardiac MRI aids in diagnosis, family screening, and locating the arrhythmogenic focus.
Area of Science:
- Cardiology
- Medical Imaging
Background:
- Arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as right ventricular dysplasia, is a progressive heart muscle disease.
- It is characterized by fibroadipose atrophy primarily affecting the right ventricle, leading to severe ventricular arrhythmias and sudden death, particularly in young individuals.
Purpose of the Study:
- To highlight the role of Magnetic Resonance Imaging (MRI) in diagnosing and managing arrhythmogenic right ventricular cardiomyopathy (ARVC).
- To clarify the diagnostic utility and potential pitfalls of identifying fatty infiltration in ARVC using MRI.
Main Methods:
- Utilizing cardiac MRI to assess ventricular morphology and myocardial composition.
- Evaluating signs such as ventricular dilatation, wall thinning/thickening, diastolic bulging, and fatty infiltration.
Main Results:
- Cardiac MRI reveals ventricular dilatation, wall abnormalities, and fatty substitution, particularly in the right ventricle.
- Fatty substitution alone can be misleading due to normal variations and imaging artifacts, necessitating a comprehensive assessment.
- MRI is effective for diagnosing idiopathic right ventricular outflow tract tachycardia and screening family members of ARVC patients.
Conclusions:
- Cardiac MRI is a valuable, non-invasive tool for diagnosing ARVC, monitoring disease progression, and screening at-risk family members.
- A thorough MRI evaluation, beyond just assessing fatty infiltration, is crucial for accurate ARVC diagnosis.
- MRI assists in electrophysiological studies to precisely locate arrhythmogenic foci, improving treatment outcomes.
Abstract:
Right ventricular dysplasia is a new entity of unknown origin in the classification of cardiomyopathies. Also known as arrhythmogenic right ventricular cardiomyopathy (ARVC) or arrhythmogenic right ventricular dysplasia, it is a disease of the heart muscle characterised by fibroadipose atrophy mainly involving the right ventricle and responsible for severe ventricular arrhythmias and sudden death also in young people. Magnetic resonance imaging provides evidence of ventricular dilatation at the outflow tract, thinning and thickening of the wall, diastolic bulging areas (especially located at the level of the right ventricle outflow tract) and fatty substitution of the myocardium mainly at the level of the right ventricle. Many radiologists erroneously consider the previously described fatty substitution as the main sign of ARVC, even though an evaluation of fat substitution alone may be a source of error for two reasons: firstly, because isolated areas of fatty replacement are not synonymous with ARVC since small non-transmural focal fatty areas of fat are also present in the normal patients; and secondly, because the MRI detection of fat may be overestimated due to partial-volume artefacts with normal subepicardial fat. Cardiac MRI can also be employed for the diagnosis of idiopathic right ventricular outflow tract tachycardia. Considering the evolutive nature of the disease, the non-invasiveness of MRI allows the follow-up of these patients and may be considered an excellent screening modality for the diagnosis of ARVC in family members. Finally, MRI can be employed in electrophysiological studies to locate the arrhythmogenic focus and reduce sampling errors.