Table 2.

Genotype–phenotype correlations and specific recommendations for implantable-cardioverter defibrillator implantation in the primary prevention of sudden cardiac death in patients with arrhythmogenic left ventricular cardiomyopathy.

Genotype	Phenotype	Recommendations for ICD
LMNA	ALVC, DCM, muscular dystrophy, conduction delay, VT, and SCD	-SCD risk ≥ 10% and NSVT or LVEF < 50% or AV delay -LVEF < 35%
PLN	ALVC, ARVC, DCM, VT/VF	-LVEF 35–50% and ≥ 2 risk factors -LVEF < 35%
FLNC	ALVC, DCM, SCD
RBM20	ALVC, DCM, VT

Abbreviations: ALVC, arrhythmogenic left ventricular cardiomyopathy; ARVC, arrhythmogenic right ventricular cardiomyopathy; AV, atrioventricular; DCM, dilated cardiomyopathy; LVEF, left ventricular ejection fraction; NSVT, non-sustained ventricular tachycardia; SCD, sudden cardiac death; VF, ventricular fibrillation; VT, ventricular tachycardia.