• Arrhythmogenic cardiomyopathy (AC) is an inherited cardiomyopathy predisposing to ventricular arrhythmias, sudden cardiac death, ventricular dysfunction and heart failure.
• The diagnostic 2010 Task Force Criteria (TFC 2010) combine data from imaging, electrical parameters (resting electrocardiogram (ECG) and Holter monitoring), family history, genetic testing, and tissue properties.
• A genetic mutation is found in up to 50-60% of probands, mostly affecting desmosomal genes, and inheritance is most frequently autosomal dominant.
• The disease is progressive, and repeated cardiac imaging is needed to follow disease progression and assess risk of arrhythmias.
• This expert consensus document will give clinical recommendations for how to use multi-modality imaging in the different aspects of AC disease, including diagnosis, family screening, follow -up, risk assessment and differential diagnosis.