Wahbi LMNA Risk Score — EECC MCQ

Correct answer: D — NSVT on Holter, LVEF, male sex, and type of LMNA mutation (non-missense/truncating); a score ≥7 at 5 years suggests ICD benefit even at LVEF >35%

The Wahbi LMNA risk calculator (validated in 2019) is specifically designed for LMNA cardiomyopathy patients to guide primary prevention ICD decisions. It incorporates: (1) NSVT on Holter monitoring; (2) LVEF (continuous variable); (3) male sex; (4) type of LMNA mutation (non-missense mutations, particularly truncating variants, carry higher risk). A predicted 5-year risk of malignant ventricular arrhythmia ≥7% is the threshold at which ICD should be considered (Class IIa, ESC 2023 Cardiomyopathy Guidelines). This score is important because it allows ICD consideration at LVEF above the standard 35% threshold — many LMNA patients experience sudden death at relatively preserved LVEF.

Reference: ESC (2023): Cardiomyopathies Guidelines; Wahbi et al., Eur Heart J 2019