Cadherin 2-Related Arrhythmogenic Cardiomyopathy: Prevalence and Clinical Features.

doi:10.1161/CIRCGEN.120.003097

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Article

Cadherin 2-Related Arrhythmogenic Cardiomyopathy: Prevalence and Clinical Features.

Circulation. Genomic and precision medicine - 1 Apr 2021

Ghidoni Alice, Elliott Perry M, Syrris Petros, Calkins Hugh, James Cynthia A, Judge Daniel P, Murray Brittney, Barc Julien, Probst Vincent, Schott Jean Jacques, Song Jiang-Ping, Hauer Richard N W, Hoorntje Edgar T, van Tintelen J Peter, Schulze-Bahr Eric, Hamilton Robert M, Mittal Kirti, Semsarian Christopher, Behr Elijah R, Ackerman Michael J, Basso Cristina, Parati Gianfranco, Gentilini Davide, Kotta Maria-Christina, Mayosi Bongani M, Schwartz Peter J, Crotti Lia

Abstract excerpt

BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiac disease characterized by fibrofatty replacement of the right and left ventricle, often causing ventricular dysfunction and life-threatening arrhythmias. Variants in desmosomal genes account for up to 60% of cases. Our objective was to establish the prevalence and clinical features of ACM stemming from pathogenic variants in the nondesmosomal...

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PMID: 33566628
DOI: 10.1161/CIRCGEN.120.003097
PMCID: PMC8284361

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