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LMNA Cardiomyopathy: Important Considerations for the Heart Failure Clinician.

Publication ,  Journal Article
Rosario, KF; Karra, R; Amos, K; Landstrom, AP; Lakdawala, NK; Brezitski, K; Kim, H; Devore, AD
Published in: J Card Fail
December 2023

BACKGROUND: A diagnosis of Lamin proteins A and C cardiomyopathy (LMNA-CM) not only impacts disease prognosis, but also leads to specific guideline-recommended treatment options for these patients. This etiology is fundamentally different from other genetic causes of dilated CM. METHODS AND RESULTS: LMNA-CM often presents early in the third to fourth decades and there is an age-dependent penetrance of nearly 90% among those with a positive genotype for LMNA-CM. Oftentimes, electrical abnormalities with either conduction disturbances and/or either atrial or ventricular arrhythmias manifest before there is imaging evidence of left ventricular dysfunction. Given these subtle early findings, cardiac magnetic resonance provides helpful guidance regarding patterns of enhancement associated with LMNA-CM, often before there is significant left ventricular dilation and/or a decrease in the ejection fraction and could be used for further understanding of risk stratification and prognosis of asymptomatic genotype-positive individuals. Among symptomatic patients with LMNA-CM, approximately one-quarter of individuals progress to needing advanced heart failure therapies such as heart transplantation. CONCLUSIONS: In the era of precision medicine, increased recognition of clinical findings associated with LMNA-CM and increased detection by genetic testing among patients with idiopathic nonischemic CM is of increasing importance. Not only does a diagnosis of LMNA-CM have implications for management and risk stratification, but new gene-based therapies continue to be evaluated for this group. Clinicians must be aware not only of the general indications for genetic testing in arrhythmogenic and dilated cardiomyopathies and of when to suspect LMNA-CM, but also of the clinical trials underway targeted toward the different genetic cardiomyopathies.

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Published In

J Card Fail

DOI

EISSN

1532-8414

Publication Date

December 2023

Volume

29

Issue

12

Start / End Page

1657 / 1666

Location

United States

Related Subject Headings

  • Mutation
  • Lamin Type A
  • Humans
  • Heart Failure
  • Cardiovascular System & Hematology
  • Cardiomyopathy, Dilated
  • Cardiomyopathies
  • Arrhythmias, Cardiac
  • 3202 Clinical sciences
  • 3201 Cardiovascular medicine and haematology
 

Citation

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Rosario, K. F., Karra, R., Amos, K., Landstrom, A. P., Lakdawala, N. K., Brezitski, K., … Devore, A. D. (2023). LMNA Cardiomyopathy: Important Considerations for the Heart Failure Clinician. J Card Fail, 29(12), 1657–1666. https://doi.org/10.1016/j.cardfail.2023.08.016
Rosario, Karen Flores, Ravi Karra, Kaitlyn Amos, Andrew P. Landstrom, Neal K. Lakdawala, Kyla Brezitski, Han Kim, and Adam D. Devore. “LMNA Cardiomyopathy: Important Considerations for the Heart Failure Clinician.J Card Fail 29, no. 12 (December 2023): 1657–66. https://doi.org/10.1016/j.cardfail.2023.08.016.
Rosario KF, Karra R, Amos K, Landstrom AP, Lakdawala NK, Brezitski K, et al. LMNA Cardiomyopathy: Important Considerations for the Heart Failure Clinician. J Card Fail. 2023 Dec;29(12):1657–66.
Rosario, Karen Flores, et al. “LMNA Cardiomyopathy: Important Considerations for the Heart Failure Clinician.J Card Fail, vol. 29, no. 12, Dec. 2023, pp. 1657–66. Pubmed, doi:10.1016/j.cardfail.2023.08.016.
Rosario KF, Karra R, Amos K, Landstrom AP, Lakdawala NK, Brezitski K, Kim H, Devore AD. LMNA Cardiomyopathy: Important Considerations for the Heart Failure Clinician. J Card Fail. 2023 Dec;29(12):1657–1666.
Journal cover image

Published In

J Card Fail

DOI

EISSN

1532-8414

Publication Date

December 2023

Volume

29

Issue

12

Start / End Page

1657 / 1666

Location

United States

Related Subject Headings

  • Mutation
  • Lamin Type A
  • Humans
  • Heart Failure
  • Cardiovascular System & Hematology
  • Cardiomyopathy, Dilated
  • Cardiomyopathies
  • Arrhythmias, Cardiac
  • 3202 Clinical sciences
  • 3201 Cardiovascular medicine and haematology