Review Article

Cardiac Ion Channelopathies and the Sudden Infant Death Syndrome

Table 6

Prevalence of cardiac ion channel-related mutations in SIDS cohorts.

Number of SIDS cases
GeneTotalwith mutationFunctionally significant

related genes
SCN5A 54228 (5.2%)26 (4.8%)
SCN1B 29200
SCN1Bb 2921 (0.3%)1 (0.3%)
SCN2B 29200
SCN3B 2922 (0.7%)2 (0.7%)
SCN4B 2921 (0.3%)1 (0.3%)
CAV3 3356 (1.8%)5 (1.5%)
GPD1-L 2212 (0.9%)2 (0.9%)
SNTA1 2928 (2.7%)3 (1.0%)
related genes
KCND3 2921 (0.3%)0
related genes
KCNQ1 4099 (2.2%)4 (1.0%)
KCNE1 3671 (0.3%)0
related genes
KCNH2 4098 (2.0%)2 (0.5%)
KCNE2 3672 (0.5%)1 (0.3%)
related genes
KCNJ2 20100
related genes
KCNJ8 2922 (0.7%)2 (0.7%)
Intracellular calcium cycling related genes
RYR2 1342 (1.5%)2 (1.5%)
Gap junctional current related genes
GJA1 2922 (0.7%)1 (0.3%)

: ATP-sensitive potassium current; : inward rectifier potassium current; : rapid delayed rectifier potassium current; : slow delayed rectifier potassium current; : fast sodium current; : transient outward potassium current.
Based on data listed in Table 5.