Other than BrS, Scn5a mutations have been associated with sick sinus syndrome (SSS; Benson et al., 2003), progressive cardiac conduction defect (PCCD, or Lenègre disease; Schott et al., 1999; Tan et al., 2001; Probst et al., 2003) and idiopathic VF without BrS findings (Akai et al., 2000). This evidence concerns the gene SCN5A and progressive familial heart block, type 1A.