Clinical and echocardiographic data of mutation carriers in the three FHC families (ZY, ZG, and T) carrying MYL2 mutations
| Family | ID | Age (years) | Echocardiography | ECG | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| LVIVS (mid) (mm) | LVIVS (lvot) (mm) | LVPWT (mm) | E/A | Gradient LVOT (H) (mmHg) | Gradient LVOT (V) (mmHg) | Q wave (yes/no) | Voltage (yes/no) | ST/T (yes/no) | ||||
| ZY | II.1 | 69 | 14 | 15 | 11 | 0.6 | 5 | 5 | — | LBBB | — | |
| II.2 | 68 | 8 | 11 | 9 | 0.9 | 6 | 6 | No | No | No | ||
| II.4 | 60 | 9 | 11 | 10 | 1.0 | 5 | 5 | Yes | No | No | ||
| III.1 | 42 | 10 | 10 | 9 | 0.6 | 6 | 6 | No | No | No | ||
| III.2 | 35 | 23 | 24 | 11 | 0.6 | 11 | 11 | No | No | Yes | ||
| ZG | II.1 | 60 | 31 | 21 | 17 | 0.4 | 732-150 | 1142-150 | Yes | Yes | Yes | |
| T | II.1 | 46 | 15 | 20 | 9 | 0.7 | 4 | 25 | No | Yes | Yes | |
| II.2 | 49 | 10 | 14 | 10 | 1.4 | 5 | 5 | No | No | No | ||
| III.1 | 10 | 5 | 5 | 5 | 1.3 | 4 | 4 | Yes | Yes | No | ||
LVIVS—interventricular septal diameter at mid and LVOT level; LVPWD—left ventricle posterior wall diameter; LVIRT—left ventricle isovolumetric relaxation time; peak E/A—transmitral flow velocity; E/A—EA-ratio; gradient LVOT (H, rest and V, valsalva)—left ventricle outflow tract gradient; Q wave/Voltage/ST/T (segment)—yes/no refer to the fulfilment of the respective diagnostic ECG criteria; LBBB—left bundle branch block.
↵2-150 Midventricular gradient.