Table 1.
Demographics, Clinical, and Genetic Characteristics of BrS Decedents
| Case ID | Antemortem Dx | Sex | Ethnicity | Age at Death, y | Circumstances of Death | Type 1 ECG | FH BrS | FH Unexplained SCD Age <45 y | Syncope | Shanghai Score | First Degree Relatives Screened, n | SCN5A (P/LP/VUS) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| BrSA1 | BrS | F | White | 60 | Died at rest | 1 | 0 | 1 | 0 | 7 (proband) | NA | Negative |
| BrSA2 | BrS | M | White | 25 | Died in sleep | 1 | 0 | 0 | 0 | 6.5 (proband) | NA | Negative |
| BrSA3 | BrS | M | White | 44 | Died at rest | 1 | 0 | 0 | 0 | 7 (proband) | NA | Pathogenic nonsense c.3944C>G (p.S1315X) |
| BrSA4 | BrS | M | White | 25 | Died in sleep | 1 | 0 | 0 | 0 | 6.5 (proband) | NA | Negative |
| BrSA5 | BrS | M | Asian | 45 | Died at rest | 1 | 0 | 0 | 0 | 6.5 (proband) | NA | VUS missense c.50C>T (p.T17I) |
| BrSA6 | BrS | M | White | 37 | Died at rest | 0 | 1 | 1 | 0 | 6 (proband) | NA | Not performed |
| BrSF7 | None | M | White | 31 | Died at rest | 0 | 0 | 0 | 0 | 2.5 (mother) | 2 | Negative |
| BrSF8 | None | F | White | 17 | Died in sleep | 0 | 0 | 0 | 0 | 3 (mother) | 3 | Not performed |
| BrSF9 | None | F | White | 24 | Died in sleep | 0 | 0 | 0 | 1 | 3 (mother) | 1 | Negative |
| BrSF10 | None | M | White | 50 | Died immediately after exertion | 0 | 0 | 0 | 0 | 2 (daughter) | 3 | Negative |
| BrSF11 | None | F | White | 25 | Died in sleep | 0 | 0 | 0 | 0 | 3 (father) | 4 | Negative |
| BrSF12 | None | M | White | 27 | Died at rest | 0 | 0 | 0 | 0 | 2 (mother and father positive ajmalines) | 3 | Not performed |
| BrSF13 | None | M | White | 15 | Died in sleep | 0 | 0 | 0 | 0 | 2.5 (father and brother positive ajmalines) | 3 | Not performed |
| BrSF14 | None | M | White | 23 | Died immediately after exertion | 0 | 0 | 0 | 0 | 4 (mother and sister positive ajmalines) | 3 | Not performed |
| BrSF15 | None | M | White | 27 | Died in sleep | 0 | 0 | 0 | 0 | 3 (mother and brother positive ajmalines) | 3 | Negative |
| BrSF16 | None | M | White | 56 | Died in sleep | 0 | 0 | 0 | 0 | 2 (son) | 5 | Negative |
| BrSF17 | None | M | White | 19 | Died in sleep | 0 | 0 | 0 | 0 | 3 (father) | 5 | Negative |
| BrSF18 | None | M | White | 40 | Died at rest | 0 | 0 | 0 | 0 | 2 (sister) | 4 | Negative |
| BrSF19 | None | M | White | 17 | Died in sleep | 0 | 0 | 0 | 0 | 3 (father) | 3 | Not performed |
| BrSF20 | None | M | White | 24 | Died in sleep | 0 | 0 | 0 | 0 | 3 (father) | 3 | Not performed |
| BrSF21 | None | M | White | 37 | Died in sleep | 0 | 0 | 0 | 0 | 3 (father) | 3 | Not performed |
| BrSF22 | None | M | White | 25 | Died during exertion | 0 | 0 | 0 | 0 | 3 (father) | 3 | Negative |
| BrSF23 | None | M | White | 23 | Died in sleep | 0 | 0 | 0 | 0 | 3 (father) | 4 | Not performed |
| BrSF24 | None | M | White | 23 | Died during exertion | 0 | 0 | 0 | 0 | 2 (father) | 2 | Not performed |
| BrSG25 | None | F | Asian | 27 | Died in sleep | 0 | 0 | 0 | 0 | NA | NA | Likely pathogenic missense c.5038G>A (p.A1680T) |
| BrSG26 | None | F | White | 24 | Died in sleep | 0 | 0 | 0 | 0 | NA | NA | Pathogenic missense c.673C>T (p.R225W) |
| BrSG27 | None | M | White | 23 | Died in sleep | 0 | 0 | 0 | 0 | NA | NA | Likely pathogenic missense c.3665T>G (p.L1222R) |
| BrSG28 | None | F | White | 47 | Died at rest | 0 | 0 | 0 | 0 | NA | NA | Likely pathogenic in-frame c.4850_4852delTCT p.Phe1617 del |
BrS = Brugada syndrome; BrSA = Brugada syndrome antemortem; BrSF = Brugada syndrome familial; BrSG = SADS cases harboring pathogenic/likely pathogenic SCN5A variants; Dx = diagnosis; FH = family history; NA = not available; P/LP/VUS = pathogenic/likely pathogenic/variant of uncertain significance; SCD = sudden cardiac death.