Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy

D. Smith, Eric; K. Lakdawala, Neal; Papoutsidakis, Nikolaos; Aubert, Grégory; Mazzanti, Andrea; C. McCanta, Anthony; Agarwal, Prachi; Arscott, Patricia; Dellefave‐Castillo, Lisa; Vorovich, Esther; Nutakki, Kavitha; D. Wilsbacher, Lisa; G. Priori, Silvia; Jacoby, Daniel; M. McNally, Elizabeth; Helms, Adam

doi:10.1161/circulationaha.119.044934

Public

Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy

Published in Circulation • May 6, 2020

Authors:

Eric D. Smith

Neal K. Lakdawala

Nikolaos Papoutsidakis

Abstract

Background: Mutations in desmoplakin ( DSP ), the primary force transducer between cardiac desmosomes and intermediate filaments, cause an arrhythmogenic form of cardiomyopathy that has been variably associated with arrhythmogenic right ventricular cardiomyopathy. Clinical correlates of DSP cardiomy...

Subject

Medicine

Desmoplakin

Cardiology

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