Akhtar MM, Lorenzini M, Pavlou M, et al. - Instead of currently recommended left ventricular ejection fraction (LVEF) values, higher values are advocated to be considered for a preventive implantable cardioverter-defibrillator in patients carrying truncating variants of the gene encoding filamin C (FLNCtv).

• A cohort study with 167 FLNCtv carriers and 244 controls with TTN truncating variants (TTNtv) matched for LVEF.

• 29 (17%) of 167 FLNCtv carriers reached the primary endpoint of malignant ventricular arrhythmia (MVA) or end-stage heart failure.

• Patients with FLNCtv with mild to moderate left ventricular systolic dysfunction had high frequency of MVA.

• In FLNCtv carriers, freedom from MVA did not differ significantly between patients with mild to moderate vs severely impaired LVEF.

• Independent risk factors linked with the primary endpoint were LVEF decrement and proband status.