TNNI3 and Schnyder corneal dystrophy: In current clinical practice, risk stratification for SCD is based on clinical risk factors such as the degree of hypertrophy among others and family history.6 With few exceptions, the majority of genotype-phenotype correlations have failed to demonstrate that a gene variant alone could be used to predict risk of sudden death with enough certainty independent of the phenotype.5,6 In the specific case of TNNI3 p.Arg21Cys, our data highlight a malignant phenotype at a young age that justifies risk stratification for SCD based exclusively on genetic information.