Details of included studies
| Study | HCM Risk-SCD 20143 | Vriesendorp et al
21 2015 | Magrì et al
23 2016 | Fernández et al
24 2016 | Leong et al
26 2018 | EVIDENCE-HCM 201825 |
| Patients (n) | 3675 | 706 | 623* | 502 | 288† | 3703 |
| Centres (n) | 6 | 2 | 5 | 1 | 1 | 14 |
| Follow-up (years) | 6.6±5 | 7.7±5.3 | 3.7 (2.2–5.1) | 8.6±4.3 | 5.6±3.8 | 7.6±6.5 |
| Annual SCD rate | 0.8 | 0.77 | 0.8 | NA | NA | 0.6 |
| Age (years) | 48±17 | 49±16 | 49±16 | 51±18 | 52±16 | 52±15 |
| MWT (mm) | 20±5 | 20±5 | 20±5 | 22±NA | 20±5 | 20±4 |
| LVOTg (mm Hg) | 31±38 | 48±44 | 12 (7–34) | 75±NA | 28±41 | 33±7 |
| LAd (mm) | 44±8 | 45±8 | 42±7 | 46±NA | 41±7 | 43±8 |
| NSVT (%) | 634 (17.3) | 157 (22.2) | 214 (34.3) | 58 (11.6) | 66 (22.9) | 582 (15.7) |
| Syncope (%) | 507 (13.8) | 72 (10.2) | 94 (15.1) | 45 (9) | 34 (11.8) | 474 (12.8) |
| FHSCD (%) | 886 (24.1) | 141 (20) | 71 (11.4) | 52 (10.4) | 40 (13.9) | 620 (16.7) |
| Validation type | Internal | External | External | External | External | External |
| C-index (95% CI) | 0.7 (0.68 to 0.72) | 0.69 (0.57 to 0.82) | 0.69 | 0.92 (0.89 to 0.95) | 0.86 | 0.7 (0.68 to 0.72) |
| C-index methodology | Uno | R Survival ROC | Harrell’s C | ROC curves SPSS | Stata ROC | Uno |
| Newcastle-Ottawa Score | 8 | 8 | 7 | 8 | 7 | 8 |
Continuous variables are presented as mean±SD or median (25th–75th centiles).
*32/623 patients were also reported in HCM Risk-SCD.
†9/288 patients had cardiac arrest prior to baseline evaluation.
FHSCD, family history of sudden cardiac death; HCM, hypertrophic cardiomyopathy; LAd, left atrial diameter; LVOTg: maximal instantaneous left ventricular outflow tract gradient at rest or Valsalva; MWT, maximal wall thickness; NA, not available; NSVT, non-sustained ventricular tachycardia; ROC, receiver operating characteristic; SCD, sudden cardiac death.