Table 1.
Characteristics | All patients (n = 31) | ERVA status | p valuea | |
---|---|---|---|---|
ERVA (+) (n = 7) | ERVA (−) (n = 24) | |||
Age (years) | 38 ± 13 | 37 ± 6 | 38 ± 15 | NS |
Male gender, n (%) | 20 (65) | 5 (71) | 15 (63) | NS |
More than 1 SCD risk factor, n (%) | 21 (68) | 7 (100) | 14 (28) | 0.04 |
Mutations, n (%) | NS | |||
MYH7 | 12 (37) b | 2 (29) | 10 (40) b | |
MYBPC3 | 7 (23) | 4 (57) | 3 (13) | |
TPM1 | 9 (29) | 1 (14) | 8 (33) | |
MYL2 | 2 (7) | 0 (0) | 2 (8) | |
CSRP3 | 2 (5) b | 0 (0) | 2 (6) b | |
NT-proBNP, pmol/l | 70 ± 56 | 87 ± 53 | 62 ± 57 | NS |
CMR | ||||
LVEF, % | 67 ± 5 | 69 ± 3 | 67 ± 6 | NS |
LVMi, g/m2 | 76 ± 23 | 95 ± 33 | 71 ± 17 | NS |
LVEDWT(max), mm | 20 ± 7 | 23 ± 7 | 19 ± 7 | NS |
Fibrosis, n (%) | 21 (68) | 7 (100) | 14 (58) | 0.04 |
Extent of fibrosis, % | 5 ± 4 | 8 ± 4 | 3.4 ± 3.6 | 0.02 |
Exercise test, n (%) | ||||
VT/VF | 0 (0) | 0 | 0 | |
VPBs | 7 (27) | 7 | 0 | |
Bigeminy and couplet | 1 (3) | 1 | 0 | |
β-blocker therapy | 9 (29) | 3 (43) | 6 (25) | NS |
CMR cardiac magnetic resonance imaging, CSRP3 cysteine and glycine-rich protein 3, ERVA exercise related ventricular arrhythmias, LV left ventricle, LVEDWT(max) maximal end-diastolic left ventricle wall thickness, LVEF left ventricle ejection fraction, LVMi left ventricle mass index, MYBPC3 myosin binding protein C, MYH7 myosin heavy chain 7, MYL2 myosin light chain 2, NS not significant, SCD sudden cardiac death, TPM1 alpha tropomyosin 1, VF ventricular fibrillation, VPB ventricular premature beat, VT ventricular tachycardia
aThe p values reflect the comparison of patients with ERVA vs. those without ERVA
bOne patient carried double heterozygous gene mutations