Although the TPM1-Asp175Asn mutation causing HCM has been considered benign or to represent intermediary risk (Coviello et al., 1997), Hedman and co-workers suggested that HCM patients carrying TPM1-Asp175Asn mutation were at increased risk of fatal arrhythmias, and a number of these patients had SCD at a young or middle age, or presented two or three clinical markers for increased risk of SCD [family history of SCD, syncope, NSVT, pathological blood pressure response, marked (>2.5 cm) hypertrophy] (Hedman et al., 2004). This evidence concerns the gene TPM1 and Schnyder corneal dystrophy.