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Lamin A/C Cardiomyopathy: Implications for Treatment

  • Myocardial Disease (A Abbate and G Sinagra, Section Editors)
  • Published:
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Abstract

Purpose of Review

The purpose of this review is to provide an update on lamin A/C (LMNA)-related cardiomyopathy and discuss the current recommendations and progress in the management of this disease. LMNA-related cardiomyopathy, an inherited autosomal dominant disease, is one of the most common causes of dilated cardiomyopathy and is characterized by steady progression toward heart failure and high risks of arrhythmias and sudden cardiac death.

Recent Findings

We discuss recent advances in the understanding of the molecular mechanisms of the disease including altered cell biomechanics, which may represent novel therapeutic targets to advance the current management approach, which relies on standard heart failure recommendations. Future therapeutic approaches include repurposed molecularly directed drugs, siRNA-based gene silencing, and genome editing.

Summary

LMNA-related cardiomyopathy is the focus of active in vitro and in vivo research, which is expected to generate novel biomarkers and identify new therapeutic targets. LMNA-related cardiomyopathy trials are currently underway.

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Authors and Affiliations

  1. Molecular Genetics, Cardiovascular Institute, University of Colorado Denver Anschutz Medical Campus, 12700 E 19th Ave #F442, Aurora, CO, 80045-2507, USA

    Suet Nee Chen, Orfeo Sbaizero, Matthew R. G. Taylor & Luisa Mestroni

  2. Department of Engineering and Architecture, University of Trieste (Italy), Trieste, Italy

    Orfeo Sbaizero

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  1. Suet Nee Chen
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Suet Nee Chen, Orfeo Sbaizero, and Luisa Mestroni declare that they have no conflict of interest.

Matthew RG Taylor reports grants from Array Biopharma.

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Chen, S.N., Sbaizero, O., Taylor, M.R.G. et al. Lamin A/C Cardiomyopathy: Implications for Treatment. Curr Cardiol Rep 21, 160 (2019). https://doi.org/10.1007/s11886-019-1224-7

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