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LMNA Cardiomyopathy — EECC MCQ

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HardCardiomyopathyLMNA CardiomyopathyEECC

A 50-year-old woman with dilated cardiomyopathy (LVEF 30%, LVESD 52 mm) undergoes genetic testing which reveals a pathogenic LMNA (lamin A/C) variant. She has had two episodes of non-sustained VT on Holter monitoring. According to the 2023 ESC Cardiomyopathy Guidelines, what specific consideration applies to ICD implantation in LMNA-related DCM?

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Correct answer: C — A gene-specific risk calculator should be used to guide ICD implantation

Explanation lettering: D = shown as C · C = shown as D

Per the 2023 ESC Cardiomyopathy Guidelines, specific gene-based risk calculators have been developed for LMNA-related cardiomyopathy to guide ICD implantation (Class IIa, LOE B). The LMNA risk calculator incorporates NSVT, LVEF, sex, and the variant to estimate 5-year risk of malignant arrhythmic events. LMNA mutations carry particularly high arrhythmic risk. Secondary prevention only (A) would miss at-risk patients. ICD in all carriers (C) is not justified. CRT-D preference (D) depends on QRS morphology. S-ICD (E) may be appropriate in some cases.

Reference: ESC (2023): Guidelines for the Management of Cardiomyopathies