Key result
Pathogenic LMNA variant drives rapid conduction disease and atrial standstill prior to LV dysfunction.
Why the study?
LMNA-related cardiomyopathy is a rare but malignant disease characterized by early conduction system disease and a high risk of sudden cardiac death, with electrical abnormalities often preceding structural dysfunction.
Case Report (n=1)
No
LMNA-related cardiomyopathy can manifest primarily with severe, progressive atrial electrical and hemodynamic dysfunction—eventually leading to atrial standstill—before significant left ventricular dysfunction occurs.
May warrant LMNA consideration in young adults with rapid atrial conduction disease; extends phenotype to atrial standstill preceding LV dysfunction.
Laminopathies caused by pathogenic variants in the LMNA gene are a rare but malignant form of cardiomyopathy, with early conduction system disease and a significantly increased risk of sudden cardiac death (SCD).Compared with other cardiomyopathies, LMNA-associated disease has a much more arrhythmogenic phenotype, with electrical abnormalities often preceding structural myocardial dysfunction [1].
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Dymek et al. (2026) conducted a case report in LMNA-related cardiomyopathy (n=1). LMNA c.1130G>T (p.Arg377Leu) pathogenic variant was evaluated. A pathogenic LMNA variant in a 38-year-old man caused rapidly progressive atrial arrhythmias, advanced atrioventricular block, and eventual atrial standstill prior to left ventricular dysfunction.
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