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 …

Association of genetic variants with outcomes in patients with nonischemic dilated cardiomyopathy

L Escobar-Lopez, JP Ochoa, JG Mirelis… - Journal of the American …, 2021 - jacc.org
Background The clinical relevance of genetic variants in nonischemic dilated
cardiomyopathy (DCM) is unsettled. Objectives The study sought to assess the prognostic …

Natural History of MYH7-Related Dilated Cardiomyopathy

F De Frutos, JP Ochoa, M Navarro-Peñalver… - Journal of the American …, 2022 - jacc.org
Background Variants in myosin heavy chain 7 (MYH7) are responsible for disease in 1% to
5% of patients with dilated cardiomyopathy (DCM); however, the clinical characteristics and …

Clinical Profile, Arrhythmias, and Adverse Cardiac Outcomes in Emery–Dreifuss Muscular Dystrophies: A Systematic Review of the Literature

AC Valenti, A Albini, JF Imberti, M Vitolo, N Bonini… - Biology, 2022 - mdpi.com
Simple Summary Cardiolaminopathies portend an augmented risk of atrial and ventricular
arrhythmias, thromboembolic events, ventricular dysfunction, and mortality. A high level of …

REALM-DCM: A Phase 3, Multinational, Randomized, Placebo-Controlled Trial of ARRY-371797 in Patients With Symptomatic LMNA-Related Dilated …

P Garcia-Pavia, JFR Palomares, G Sinagra… - Circulation: Heart …, 2024 - ahajournals.org
BACKGROUND: LMNA (lamin A/C)-related dilated cardiomyopathy is a rare genetic cause
of heart failure. In a phase 2 trial and long-term extension, the selective p38α MAPK …

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 …

[HTML][HTML] Timing of cardioverter-defibrillator implantation in patients with cardiac laminopathies—external validation of the LMNA-risk ventricular tachyarrhythmia …

C Rootwelt-Norberg, AH Christensen, ET Skjølsvik… - Heart Rhythm, 2023 - Elsevier
Background LMNA genotype-positive patients have high risk of experiencing life-threatening
ventricular tachyarrhythmias (VTAs). The LMNA-risk VTA calculator published in 2019 has …

Combination of late gadolinium enhancement and genotype improves prediction of prognosis in non‐ischaemic dilated cardiomyopathy

JG Mirelis, L Escobar‐Lopez, JP Ochoa… - European journal of …, 2022 - Wiley Online Library
Aims Genotype and left ventricular scar on cardiac magnetic resonance (CMR) are
increasingly recognized as risk markers for adverse outcomes in non‐ischaemic dilated …

Characterization and natural history of patients with LMNA‐related dilated cardiomyopathy in the phase 3 REALM‐DCM trial

P Garcia‐Pavia, NK Lakdawala, G Sinagra… - ESC heart …, 2024 - Wiley Online Library
Aims LMNA‐related dilated cardiomyopathy (DCM) is a rare disease with an incompletely
defined phenotype. The phase 3 REALM‐DCM trial evaluated a potential disease‐modifying …

Genetic testing as a guide for treatment in dilated cardiomyopathies

S Garcia-Hernandez, LM Iglesias - Current Cardiology Reports, 2022 - Springer
Abstract Purpose of Review Dilated cardiomyopathy (DCM) is one of the most prevalent
primary cardiomyopathies and may be caused by genetic and non-genetic etiologies. DCM …