2023 ESC Guidelines for the management of cardiomyopathies: Developed by the task force on the management of cardiomyopathies of the European Society of …

E Arbelo, A Protonotarios, JR Gimeno… - European heart …, 2023 - academic.oup.com
• The specific situation of the patient. Unless otherwise provided for by national regulations,
off-label use of medication should be limited to situations where it is in the patient's interest …

Pathophysiology of dilated cardiomyopathy: from mechanisms to precision medicine

M Gigli, D Stolfo, M Merlo, G Sinagra… - Nature Reviews …, 2024 - nature.com
Dilated cardiomyopathy (DCM) is a complex disease with multiple causes and various
pathogenic mechanisms. Despite improvements in the prognosis of patients with DCM in the …

A novel tool for arrhythmic risk stratification in desmoplakin gene variant carriers

RT Carrick, A Gasperetti, A Protonotarios… - European heart …, 2024 - academic.oup.com
Abstract Background and Aims Pathogenic desmoplakin (DSP) gene variants are
associated with the development of a distinct form of arrhythmogenic cardiomyopathy known …

Programmed ventricular stimulation as an additional primary prevention risk stratification tool in arrhythmogenic right ventricular cardiomyopathy: a multinational study

A Gasperetti, RT Carrick, S Costa, P Compagnucci… - Circulation, 2022 - Am Heart Assoc
Background: A novel risk calculator based on clinical characteristics and noninvasive tests
that predicts the onset of clinical sustained ventricular arrhythmias (VA) in patients with …

Phenotype and clinical outcomes in desmin-related arrhythmogenic cardiomyopathy

FJ Bermudez-Jimenez, A Protonotarios… - Clinical …, 2024 - jacc.org
Background Desmin (DES) pathogenic variants cause a small proportion of arrhythmogenic
cardiomyopathy (ACM). Outcomes data on DES-related ACM are scarce. Objectives This …

AAV-mediated delivery of Plakophilin-2a arrests progression of arrhythmogenic right ventricular cardiomyopathy in murine hearts: preclinical evidence supporting …

CJM van Opbergen, B Narayanan… - Circulation: Genomic …, 2024 - Am Heart Assoc
BACKGROUND: Pathogenic variants in PKP2 (plakophilin-2) cause arrhythmogenic right
ventricular cardiomyopathy, a disease characterized by life-threatening arrhythmias and …

Highly malignant disease in childhood-onset arrhythmogenic right ventricular cardiomyopathy

MK Smedsrud, M Chivulescu, MI Forså… - European Heart …, 2022 - academic.oup.com
Aims This study aimed to explore the incidence of severe cardiac events in paediatric
arrhythmogenic right ventricular cardiomyopathy (ARVC) patients and ARVC penetrance in …

The diagnostic value of the 12-lead ECG in arrhythmogenic left ventricular cardiomyopathy: novel ECG signs

L Calò, C Crescenzi, A Martino, M Casella… - Clinical …, 2023 - jacc.org
Background Electrocardiographic (ECG) findings in arrhythmogenic left ventricular
cardiomyopathy (ALVC) are limited to small case series. Objectives This study aimed to …

Arrhythmic risk stratification in arrhythmogenic right ventricular cardiomyopathy

A Gasperetti, CA James, RT Carrick, A Protonotarios… - Europace, 2023 - academic.oup.com
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable cardiomyopathy
characterized by a predominantly arrhythmic presentation. It represents the leading cause of …

State‐of‐the‐art document on optimal contemporary management of cardiomyopathies

PM Seferović, M Polovina, G Rosano… - European journal of …, 2023 - Wiley Online Library
Cardiomyopathies represent significant contributors to cardiovascular morbidity and
mortality. Over the past decades, a progress has occurred in characterization of the genetic …