The Cardiovascular Magnetic Resonance Phenotype of Lamin Heart Disease

The CMR phenotype of LMNA carriers with preserved left ventricular systolic function consists of longer T<sub>2</sub>, higher serum troponin levels, higher extracellular volume, and impaired strain. CMR-derived focal fibrosis and strain biomarkers are prognostic, and future studies should explore their added clinical utility beyond the currently available MACE risk prediction tools. (The Deep Phenotype of Lamin A/C Cardiomyopathy; NCT03860454).

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Publication Details

Journal
JACC. Cardiovascular imaging
Published
2025-05-14
DOI
https://doi.org/10.1016/j.jcmg.2025.01.004
Citations
8
Primary Topic
Cardiomyopathy and Myosin Studies
Type
article
Field-Weighted Citation Impact
11.11

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article

The Cardiovascular Magnetic Resonance Phenotype of Lamin Heart Disease

Paolo Piras, James Moon, Iain Pierce, Fiona Chan et al.
8 citations
JACC. Cardiovascular imaging
Cardiomyopathy and Myosin Studies
11.11
article

The Cardiovascular Magnetic Resonance Phenotype of Lamin Heart Disease

Paolo Piras, James Moon, Iain Pierce, Fiona Chan, Konstantinos Savvatis, Constantin‐Cristian Topriceanu, Peter Kellman, Hunain Shiwani, Mashael Alfarih, Saidi Mohiddin, Gabriella Captur, Richard P. Steeds, Migma Dorji Tamang, George Joy, Paolo Emilio Puddu, Andrea Barison, Benjamin O’Brien, Alun David Hughes, Denis C. Ilie-Ablachim, Steffen Erhard Petersen, Catherine M. Banks, William E. Moody, Stephen Pettit, Matt Webber
article en
8 citations

Abstract

The CMR phenotype of LMNA carriers with preserved left ventricular systolic function consists of longer T<sub>2</sub>, higher serum troponin levels, higher extracellular volume, and impaired strain. CMR-derived focal fibrosis and strain biomarkers are prognostic, and future studies should explore their added clinical utility beyond the currently available MACE risk prediction tools. (The Deep Phenotype of Lamin A/C Cardiomyopathy; NCT03860454).

JACC. Cardiovascular imagingVol. 18(6)
St Bartholomew's Hospital (GB), Outcomes Research Consortium (US), Queen Mary University of London (GB), Barts Health NHS Trust (GB), Queen Elizabeth Hospital Birmingham (GB), Royal Papworth Hospital (GB), British Heart Foundation (GB), Deutsches Herzzentrum der Charité (DE), National Heart, Lung, and Blood Institute (US), MRC Unit for Lifelong Health and Ageing (GB), William Harvey Research Institute (GB), Fondazione Toscana Gabriele Monasterio (IT), University College London (GB), Charité - Universitätsmedizin Berlin (DE), Sapienza University of Rome (IT)
Society for Cardiovascular Magnetic Resonance, Barts Charity, National Institute for Health and Care Research, British Heart Foundation, British Medical Association
Good health and well-being
Openalex Percentile: Top 1%
Cardiomyopathy and Myosin Studies
11.11
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