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Article

Desmoplakin Cardiomyopathy in Pediatric Patients: A Distinct, Underrecognized Cohort of Arrhythmogenic Cardiomyopathy.

Circulation. Arrhythmia and electrophysiology - 1 Nov 2024

Choi Nak Hyun, Cherny Sara, Berul Charles I, Goodyer William R, Howard Taylor S, Joong Anna, Liberman Leonardo, Silver Eric S, Villa Chet R, Lee Teresa M, Zuckerman Warren A

Abstract excerpt

BACKGROUND: DSP cardiomyopathy is a distinct subset of arrhythmogenic cardiomyopathy, reported primarily in adults, that has predominantly left ventricular involvement and features of myocarditis. Clinical characteristics, risk stratification, and management of pediatric patients with DSP variants are not well known. We sought to identify phenotypic features and prognosis of pediatric patients with DSP pathogenic...

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PMID: 39523938
DOI: 10.1161/CIRCEP.124.013114

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