Table 1.
Baseline characteristics of 62 paediatric ARVC patients, comparing probands and relatives
| Overall (n = 62) |
Proband (n = 11) |
Relative (n = 51) |
P-value | |
|---|---|---|---|---|
| Female sex | 34 (55) | 4 (36) | 30 (59) | 0.18 |
| Age at inclusion, years | 9.8 (5.0–14.0) | 12.3 (11.4–16.4) | 9.0 (4.3–12.9) | 0.02 |
| Mutation carrier | 57 (92) | 7 (64) | 50 (98) | <0.01 |
| Duration of follow-up, years | 6.0 (2.9–9.6) | 6.6 (1.8–9.9) | 5.8 (3.5–9.2) | 0.72 |
| Clinical phenotype | ||||
| Repolarization criteriaa | 13 (21) | 9 (82) | 4 (8) | <0.01 |
| Major | 0 (0) | 0 (0) | 0 (0) | 1.00 |
| Minor | 13 (21) | 9 (82) | 4 (8) | <0.01 |
| Depolarization criteria | 6 (10) | 4 (36) | 2 (4) | <0.01 |
| Epsilon wave | 0 (0) | 0 (0) | 0 (0) | 1.00 |
| Late potentials | 6 (10) | 4 (36) | 2 (4) | <0.01 |
| Arrhythmia criteria | 16 (26) | 9 (82) | 7 (14) | <0.01 |
| Major | 0 (0) | 0 (0) | 0 (0) | 1.00 |
| Holter monitor, >500 PVCs/24 h | 16 (26) | 9 (82) | 7 (14) | <0.01 |
| Imaging results abnormal | 12 (19) | 10 (91) | 2 (4) | <0.01 |
| Major | 12 (19) | 10 (91) | 2 (4) | <0.01 |
| Minor | 0 (0) | 0 (0) | 0 (0) | 1.00 |
ARVC, arrhythmogenic right ventricular cardiomyopathy; PVC, premature ventricular complex; h, hours.
Values are frequency (%) or median (interquartile range). P-values by Student’s t-test, chi-square test or Fisher’s exact test, as appropriate.
a
T-wave inversions were not counted in patients under the age of 14 years.