Epidemiology of the inherited cardiomyopathies

WJ McKenna, DP Judge - Nature Reviews Cardiology, 2021 - nature.com
In the absence of contemporary, population-based epidemiological studies, estimates of the
incidence and prevalence of the inherited cardiomyopathies have been derived from …

Arrhythmogenic cardiomyopathy

D Corrado, C Basso, DP Judge - Circulation research, 2017 - Am Heart Assoc
Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to
sudden cardiac death, particularly in young patients and athletes. Pathological features …

Molecular mechanisms of arrhythmogenic cardiomyopathy

KM Austin, MA Trembley, SF Chandler… - Nature Reviews …, 2019 - nature.com
Arrhythmogenic cardiomyopathy is a genetic disorder characterized by the risk of life-
threatening arrhythmias, myocardial dysfunction and fibrofatty replacement of myocardial …

Arrhythmogenic right ventricular cardiomyopathy

C Basso, D Corrado, FI Marcus, A Nava, G Thiene - The Lancet, 2009 - thelancet.com
Arrhythmogenic right ventricular cardiomyopathy is a rare inherited heart-muscle disease
that is a cause of sudden death in young people and athletes. Causative mutations in genes …

Identification of a deletion in plakoglobin in arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratoderma and woolly hair (Naxos disease)

G McKoy, N Protonotarios, A Crosby, A Tsatsopoulou… - The Lancet, 2000 - thelancet.com
Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an autosomal
dominant heart muscle disorder that causes arrhythmia, heart failure, and sudden death …

[HTML][HTML] Suppression of canonical Wnt/β-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy

E Garcia-Gras, R Lombardi… - The Journal of …, 2006 - Am Soc Clin Investig
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is a genetic disease
caused by mutations in desmosomal proteins. The phenotypic hallmark of ARVC is …

[HTML][HTML] Diagnosis and management of rare cardiomyopathies in adult and paediatric patients. A position paper of the Italian Society of Cardiology (SIC) and Italian …

G Limongelli, R Adorisio, C Baggio, B Bauce… - International journal of …, 2022 - Elsevier
Cardiomyopathies (CMPs) are myocardial diseases in which the heart muscle is structurally
and functionally abnormal in the absence of coronary artery disease, hypertension, valvular …

Arrhythmogenic cardiomyopathy

K Pilichou, G Thiene, B Bauce, I Rigato… - Orphanet journal of rare …, 2016 - Springer
Arrhythmogenic cardiomyopathy (AC) is a heart muscle disease clinically characterized by
life-threatening ventricular arrhythmias and pathologically by an acquired and progressive …

Remodeling of myocyte gap junctions in arrhythmogenic right ventricular cardiomyopathy due to a deletion in plakoglobin (Naxos disease)

SR Kaplan, JJ Gard, N Protonotarios, A Tsatsopoulou… - Heart rhythm, 2004 - Elsevier
OBJECTIVES: We tested the hypothesis that defective interactions between adhesion
junctions and the cytoskeleton caused by the plakoglobin mutation in Naxos disease lead to …

Induced Deletion of the N-Cadherin Gene in the Heart Leads to Dissolution of the Intercalated Disc Structure

I Kostetskii, J Li, Y Xiong, R Zhou, VA Ferrari… - Circulation …, 2005 - Am Heart Assoc
The structural integrity of the heart is maintained by the end-to-end connection between the
myocytes called the intercalated disc. The intercalated disc contains different junctional …