skip to main content

LMNA Cardiomyopathy - ICD Decision — EECC MCQ

Instant feedback + full explanation. One question, done properly.

HardCardiomyopathyLMNA Cardiomyopathy - ICD DecisionEECC

A 38-year-old man with DCM (LVEF 35%) undergoes genetic testing which reveals a pathogenic LMNA (lamin A/C) variant. His ECG shows first-degree AV block (PR 240 ms) and left anterior fascicular block. He has no syncope and no family history of SCD. According to the 2023 ESC Cardiomyopathy Guidelines, which additional risk factor, combined with his LMNA variant, would most strongly support prophylactic ICD implantation?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: D — Non-sustained VT on 24-hour Holter monitoring

LMNA cardiomyopathy carries a significantly higher risk of SCD compared to other forms of DCM, often before severe LV dysfunction develops. The ESC 2023 Cardiomyopathy Guidelines recommend using the LMNA-specific risk calculator which incorporates: (1) NSVT, (2) LVEF, (3) sex (male higher risk), and (4) the type of LMNA variant (non-missense variants carry higher risk). NSVT is one of the strongest independent predictors of malignant arrhythmias in LMNA cardiomyopathy. Combined with his already reduced LVEF (35%) and conduction disease (a hallmark of LMNA), the presence of NSVT would significantly elevate his predicted 5-year SCD risk and strongly support prophylactic ICD implantation even at LVEF >35%.

Reference: ESC (2023): Guidelines on Cardiomyopathies