Table 2.
Prevalence of adult risk factors for sudden death and previously reported pediatric risk factors for death in the study group
| Risk factor (overall % of patients) | Number of observations | Patients without primary end point (n = 89) | Patients with primary end point (n = 23) | p value | Cutoff point value | RR (95 % CI) |
|---|---|---|---|---|---|---|
| Family history of SCD (22 %) | 25/112 | 20 | 5 | 0.84 | – | 0.967 (0.399–2.343) |
| Prior cardiac arrest (3 %) | 3/112 | 0 | 3 | 0.004 | – | 5.450 (3.668–8.098) |
| Syncope (15 %) | 17/112 | 14 | 3 | 0.99 | – | 0.838 (0.280–2.514) |
| NSVT (15 %) | 17/112 | 11 | 6 | 0.19 | – | 1.972 (0.909–4.279) |
| Extreme LVH ≥ 30 mm (8.9 %) | 10/112 | 8 | 2 | 0.71 | – | 0.971 (0.266–3.553) |
| ABPR to exercise (31 %) | 22/70 | 17/62 | 5/8 | 0.11 | No cutoff | 0.439 (0.224–0.859) |
| LVOTO ≥ 30 mmHg (25 %) | 28/112 | 20 | 8 | 0.34 | No cutoff | 1.600 (0.760–3.367) |
| LVOT gradient, mmHg | 112 | 6.8 (5–19) | 10.9 (7–31.4) | 0.04 | No cutoff | – |
| Age at diagnosis, years | 112 | 5 (0.6–12) | 4 (0.2–10) | 0.48 | No cutoff | – |
| SWT, mm | 112 | 14.9 (11.1–21.2) | 16.5 (10.3–20) | 0.94 | 30 | 1.922 (0.292–12.657) |
| SWT, z-score | 112 | 7.6 (4.7–14.9) | 11.3 (5.6–16.8) | 0.37 | 7 | 0.856 (0.410–1.788) |
| LVPWT, mm | 112 | 8.6 (7.2–11.3) | 9.6 (7.4–11.8) | 0.39 | 10 | 1.315 (0.636–2.719 |
| LVPWT, z-score | 1.4 (0.1–3.2) | 3 (0.1–6.3) | 0.068 | 4 | 2.488 (1.234–5.013) | |
| SWT of >20, mm (26.8 %) | 30/112 | 25 | 5 | 0.54 | – | 0.759 (0.309–1.864) |
| SWT expressed as percent of 95th centile value for age | 112 | 146.9 (116.7–219.1) | 181.6 (125.8–222.2) | 0.72 | 195 | 0.680 (0.330–1.402) |
| SWT > 190 % of upper normal limit for age (38.4 %) | 43/112 | 32 | 11 | 0.3 | – | 1.471 (0.713–3.034) |
| QTc dispersion | 112 | 0.04 (0.04–0.06) | 0.06 (0.04–0.06) | 0.0042 | 0.055 | 3.153 (1.506–6.603) |
| QTc | 112 | 0.41 (0.38–0.43) | 0.42 (0.39–0.45) | 0.27 | 0.415 | 1.214 (0.586–2.518) |
| Heart failure at presentation (17.9 %) | 20/112 | 10 | 10 | 0.0003 | – | 3.538 (1.815–6.899) |
| LA size, mm | 112 | 34 (25.7–44.4) | 39 (33–47) | 0.1 | 38 | 2.496 (1.184–5.262) |
| LA size, z-score | 1.57 (0.34–3.82) | 4.8 (2.2–8.2) | 0.001 | 3 | 3.125 (1.450–6.736) | |
| LVPWT-to-cavity ratio | 112 | 0.22 (0.18–0.29) | 0.26 (0.18–0.37) | 0.15 | 0.6 | 2.650 (1.086–6.468) |
| LVPWT-to-cavity ratio >0.3 (26 %) | 29/112 | 19 | 10 | 0.03 | – | 2.202 (1.085–4.468) |
| E/A, cm/s | 101/112 | 1.56 (1.25–1.9) | 1.39 (1.03–1.55) | 0.24 | 3 | 1.516 (0.262–8.770) |
| E/A, z-score | −0.72 (−1.19) to (−0.27) | −1 (−1.57) to (−0.78) | 0.16 | 1 | 1.753 (0.596–5.155) | |
| E vel, cm/s | 101/112 | 85.2 (73.8–97) | 63 (46–91) | 0.04 | 70 | 0.220 (0.090–0.538) |
| E vel, z-score | −0.5 (−1) to (0.31) | −1.94 (−2.4) to (−0.25) | 0.027 | −1 | 0.265 (0.107–0.653) |
Bold values indicate statistically significant data
Data expressed as median (IQR) or frequencies (percentages). No cutoff = frequency distribution similar, no cutoff point can be assessed
LV Left ventricular, LA left atrial, E vel early mitral inflow E velocity, E/A early mitral inflow E to late mitral inflow A ratio, SCD sudden cardiac death, NSVT non-sustained ventricular tachycardia, LVH left ventricular hypertrophy, SWT septal wall thickness, LVPWT left ventricular posterior wall thickness, ABPR abnormal blood pressure response, LVOTO left ventricular outflow tract obstruction