Fact-sheet: Arythmogenic right ventricul dysplasia
Updated on 02/06/2026 at 4:49 PM
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Definition
Chronic non-ischemic congenital cardiomyopathy due to fibro-fatty infiltration of the RV myocardium
Leading cause of sudden death in young athletes
Diagnosis based on a combination of findings: MRI, TTE, ECG, and clinical history
Epidemiology: prevalence 1/5,000, sex ratio 3/1 (M/F)
Annual mortality 2.5% (22% of sudden deaths <35 years)
Multiple hypotheses, including structural abnormalities of desmosomes (intercellular junctions)
Genetics: autosomal dominant, 5 genes identified to date, found in 30-50% of patients
Clinical features
Ventricular extrasystoles or ventricular tachycardia with left bundle branch block morphology, sudden death, atypical chest pain, paroxysmal dyspnea, RV dysfunction
MRI
MRI: reference examination for evaluation of the RV
Morphological assessment
- T1 "black blood" IR sequence
- Non-segmental subepicardial hyperintensity = dysplastic fatty infiltration
- Location: RV free wall and pulmonary infundibulum, extending to the septum and LV apex
- Low specificity (70%) and poor interobserver reproducibility (present in healthy subjects [atrioventricular and interventricular grooves] and in other cardiomyopathies [MI, amyloidosis, myocarditis...])
- "White blood" cine sequence
- Localized thinning of the RV wall < 2 mm
- Focal hypertrophy
- Dysplastic trabecular pattern (40%): "stacked coin" appearance
Functional assessment
- Global: RV dilation, RVEF impairment without other etiologies
- Segmental: triangle of dysplasia (pulmonary infundibulum, RV apex, RV free wall)
- Hypokinesia
- Akinesia: immobility between diastole and systole over a 1 cm segment
- Dyskinesia: systolic bulging >1 cm
- Saccular aneurysm: permanent bulging
- Contractile asynchrony: asymmetric contraction between segments
Delayed enhancement assessment
- Related to trapped gadolinium associated with fibrosis
- Predictive factor for arrhythmic events
A normal MRI does not rule out the diagnosis: follow-up imaging is warranted
Management
- Beta-blocker therapy
- Cessation of athletic activity: increased risk of arrhythmia and sudden death
- Defibrillator implantation
Classification
2010 modified Task Force criteria:
Major criteria
- Severe RV segmental wall motion abnormalities: dyskinesia or dyssynchronous contraction
AND one of the following criteria:
- Indexed EDV ≥110 mL/m² (M) ≥100 mL/m² (F)
- RVEF ≤ 40%
Minor criteria
- Severe RV segmental wall motion abnormalities: dyskinesia or dyssynchronous contraction
AND one of the following criteria:
- Indexed EDV:
110 ≤ EDVi (M)
90 ≤ EDVi (F) < 100 mL/m²
- 40% ≤ RVEF < 45%