Overall | With neurological Onset | Without neurological onset | P value | |
---|---|---|---|---|
Number of patients | 40 | 14 (35) | 26 (65) | |
Number of families | 31 | 12 (39) | 19 (61) | |
Males | 22 (55) | 7 (50) | 15 (58) | 0.744 |
Age at symptom onset, yrs | 33 (21–42) | 11 (8–30) | 39 (32–46) | < 0.0001 |
Age at first contact at our center, yrs | 39 (29–74) | 31 (20–44) | 43 (36–49) | |
Follow-up duration, months | 30 (6–70) | 24 (12–101) | 32 (8–63) | |
Diagnostic pathway | ||||
Signs or symptoms | 12 (86) | 19 (73) | 0.452 | |
Screening | 2 (14) | 7 (27) | ||
Cardiomyopathy | 24 (60) | 5 (36) | 19 (73) | 0.040 |
Dilated CMP | 13 (32) | 2 (14) | 11 (42) | 0.089 |
Hypokinetic non dilated CMP | 8 (20) | 2 (14) | 6 (23) | 0.688 |
Restrictive CMP | 2 (5) | 0 | 2 (8) | 0.533 |
Right ventricle CMP | 1 (2) | 1 (7) | 0 | 0.350 |
History of atrial fibrillation | 17 (42) | 6 (43) | 11 (42) | 1.000 |
Sinus node dysfunction | 4 (10) | 3 (21) | 1 (4) | 0.114 |
Atrio-ventricular block | 23 (57) | 6 (43) | 17 (65) | 0.197 |
1st degree | 8 (20) | 2 (14) | 6 (23) | |
2nd degree | 7 (17) | 2 (14) | 5 (19) | |
3rd degree/high degree | 8 (20) | 2 (14) | 6 (23) | |
Positive familial history for | ||||
Sudden death | 7 (17) | 3 (21) | 4 (15) | 0.678 |
PM implantation (high degree AVB) | 11 (27) | 3 (21) | 8 (30) | 0.715 |
CMP | 16 (40) | 5 (36) | 11 (42) | 0.746 |
NYHA class III- IV | 7 (17) | 4 (28) | 3 (12) | 0.214 |
PM recipients | 8 (20) | 2 (14) | 6 (23) | 0.688 |
ICD recipients | 8 (20) | 1 (7) | 7 (27) | 0.221 |