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Genetic Risk Stratification in Arrhythmogenic Left Ventricular Cardiomyopathy
Yaanik B Desai1, Victoria N Parikh1
1Department of Medicine, Division of Cardiovascular Medicine, Stanford University School of Medicine, Falk CRVC, 300 Pasteur Drive, Stanford, CA 94305, USA.
None:
Arrhythmogenic left ventricular cardiomyopathy is characterized by early malignant ventricular arrhythmia associated with varying degrees and times of onset of left ventricular dysfunction. Variants in numerous genes have been associated with this phenotype. Here, the authors review the literature on recent cohort studies of patients with variants in desmoplakin, lamin A/C, filamin-C, phospholamban, RBM20, TMEM43, and selected channelopathy genes also associated with structural disease. Unlike traditional sudden cardiac death risk assessment in nonischemic cardiomyopathy, left ventricular systolic function is an insensitive predictor of risk in patients with these genetic diagnoses.
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