Imaging Features of Arrhythmogenic Cardiomyopathies

• DOI: 10.1148/rg.230154
• Publication Date: 2024-03-21T16:49:16Z
• Authors: Mauricio S. Galizia; Anil K. Attili; William R. Truesdell; Eric D. Smith; Adam S. Helms; Abdulbaset M. A. ...; Chaitanya Madamanchi; Prachi P. Agarwal
• Keywords: Restrictive cardiomyopathy

Arrhythmogenic cardiomyopathy (ACM) is a genetic disease characterized by replacement of ventricular myocardium with fibrofatty tissue, predisposing the patient to arrhythmias and/or sudden cardiac death. Most cases ACM are associated pathogenic variants in genes that encode desmosomal proteins, an important cell-to-cell adhesion complex present both heart and skin tissue. Although was first described as predominantly right ventricle, it now acknowledged can also primarily involve left ventricle or ventricles. The original right-dominant phenotype traditionally diagnosed using 2010 task force criteria, multifactorial algorithm divided into major minor criteria consisting structural based on two-dimensional echocardiographic, MRI, angiographic findings; tissue characterization endomyocardial biopsy results; repolarization depolarization abnormalities electrocardiographic arrhythmic features; family history. Shortfalls due modern understanding have led development Padua which include updated for diagnosis new left-predominant biventricular phenotypes. In addition incorporating MRI findings dilatation, systolic dysfunction, regional wall motion abnormalities, emphasize late gadolinium enhancement at key feature imaging-based characterization. Conditions consider differential various other causes dilatation such left-to-right shunts normal anatomy be misinterpreted abnormalities. left-dominant mimic myocarditis imaging clinical examination. Additional considerations ACM, particularly phenotype, sarcoidosis dilated cardiomyopathy.