Table 1.
Clinical characteristics of HCM cohort
| HCM-cohort | Genotype negative | Single myofilament mutation |
MLP | TCAP | |
|---|---|---|---|---|---|
| No. of individuals | 389 | 233 | 140 | 12 | 4 |
| Sex, male/female | 215/174 | 127/106 | 79/61 | 7/5 | 2/2 |
| Age at Dx | 41.2 ± 19 | 45.1 ± 19 | 34.5 ± 17 | 48.5 ± 17 | 38.8 ± 9 |
| Cardiac symptoms | 216 (56%) | 128 (55%) | 74 (53%) | 9 (75%) | 4 (100%) |
| Max LVWT (mm) | 21.6 ± 6 | 20.6 ± 6 | 23.0 ± 7 | 20.1 ± 3 | 29.5 ± 12 |
| LVWT ≥ 25mm | 78 (20%) | 38 (16%) | 38 (28%) | 0 | 2 (50%) |
| Resting LVOTO (mmHg) | 46.6 ± 42 | 46.6 ± 42 | 42.8 ± 42 | 80 ± 43 | 75 ± 38 |
| Pos. FH for HCM | 121 (31%) | 54 (23%) | 61 (44%) | 3 (25%) | 1 (25%) |
| Pos. FH for SCD | 54 (14%) | 26 (11%) | 27 (19%) | 2 (17%) | 1 (25%) |
| Myectomy | 160 (41%) | 92 (39%) | 62 (44%) | 5 (42%) | 3 (75%) |
| Pacemaker | 67 (17%) | 35 (15%) | 26 (19%) | 5 (42%) | 2 (50%) |
| ICD | 60 (15%) | 23 (10%) | 36 (26%) | 1 (8%) | 0 |
| Multiple or concomitant myofilament mutation | 147 | — | 10/140 | 6/12 | 1/4 |
Values are means ± SD or % (n).
Dx indicates diagnosis; FH, family history; HCM, hypertrophic cardiomyopathy; ICD, implantable cardioverter-defibrillator; LVOTO, left ventricular outflow tract obstruction; LVWT, left ventricular wall thickness; SCD, sudden cardiac death.