Table 3.
Results of baseline investigations
| No. of patients (%) | |||
|---|---|---|---|
| Echocardiographic data at diagnosis (n = 677) | LVMWT (mm) [median (IQR)] | 13 (11–18) | |
| LVMWT (Z score) [median (IQR)] | +10.1 (3.6–37) | ||
| Left atrial diameter (mm) [mean (SD)] [n = 352] | 29 (9.2) | ||
| Left ventricular outflow gradient at rest (mmHg) [n = 604] | <30 | 444 (64.6%) | |
| 30–50 | 47 (6.8%) | ||
| 50–90 | 81 (11.8%) | ||
| >90 | 32 (12.1%) | ||
| Ambulatory ECG performed (n = 483) | Yes | NSVT | 7 (1.4%) |
| No NSVT | 476 (98.6%) | ||
| No | 204 (29%) | ||
| Exercise test performeda (n = 220) | Non-syndromic | 196 (45%) | |
| RASopathy | 12 (10%) | ||
| Friedreich ataxia | 7 (12%) | ||
| Inborn error of metabolism | 5 (8%) | ||
| Blood pressure response to exercisea | Normal (>20 mmHg rise in systolic BP) | 126 (57%) | |
| Flat (<20 mmHg rise in systolic BP) | 68 (31%) | ||
| Hypotensive (fall in systolic BP) | 16 (7%) | ||
Data expressed as number (%). Total number of patients is 687 unless otherwise stated.
SD, standard deviation.
a
Exercise test in patients ≥6 years.