Mixed models assessing the relation between variables and progression risk of the Fazekas score and infarctions
| Fixed effects | Fazekas score | Infarctions | ||
| OR (95% CI) | P value | OR (95% CI) | P value | |
| Model 1 (sex and phenotype separate) | ||||
| Age | 1.28 (1.24 to 1.32) | <0.0001 | 1.26 (1.17 to 1.35) | <0.0001 |
| Sex | ||||
| Women | 1 | – | 1 | – |
| Men | 7.4 (2.4 to 23.2) | 0.0006 | 169 (17 to 1697) | <0.0001 |
| Phenotype | ||||
| Non-classical | 1 | – | 1 | – |
| Classical | 52.9 (11.0 to 254.8) | <0.0001 | 64 (2.5 to 1625) | 0.0119 |
| Model 2 (sex and phenotype combined groups) | ||||
| Age | 1.28 (1.24 to 1.33) | <0.0001 | 1.26 (1.17 to 1.35) | <0.0001 |
| Sex, phenotype | ||||
| Women, classical | 1 | – | 1 | – |
| Men, classical | 11.0 (3.2 to 38.4) | 0.0002 | 224 (19 to 2618) | <0.0001 |
| Men, non-classical | 0.06 (0.01 to 0.49) | 0.009 | 1.42 (0.04 to 52.6) | 0.8483 |
| Women, non-classical | 0.05 (0.01 to 0.39) | 0.0037 | 0.07 (0.00 to 7.61) | 0.2619 |
| Model 2 + <6 months of ERT | 1 | – | 1 | – |
| ≥6 months of ERT | 1.34 (0.96 to 1.88) | 0.0821 | 1.43 (0.73 to 2.81) | 0.2951 |
| Model 2+ years treated with ERT | * | * | * | * |
| Model 2 + changes in eGFR | 0.99 (0.98 to 1.01) | 0.4997 | 0.96 (0.93 to 0.99) | 0.018 |
| Model 2 + changes in LVMi on MRI | 1.03 (1.00 to 1.05) | 0.0368 | 1.03 (0.99 to 1.07) | 0.1726 |
| Model 2+ no hypertension | 1 | – | 1 | – |
| Hypertension | 1.12 (0.26 to 4.82) | 0.8821 | 0.61 (0.05 to 6.88)† | 0.6859 |
| Model 2+ changes in BAD | 0.90 (0.57 to 1.43) | 0.6492 | 1.62 (0.65 to 4.05) | 0.2983 |
| Model 2 + LDL-cholesterol | 1.21 (0.58 to 2.64) | 0.6264 | 1.26 (0.31 to 5.13) | 0.7514 |
| Model 2 + no AF | 1 | – | 1 | – |
| AF | 0.77 (0.32 to 1.85) | 0.5617 | 0.45 (0.08 to 2.49) | 0.3584 |
| Model 2 + no ischaemic heart disease | 1 | – | 1 | – |
| Ischaemic heart disease | 1.92 (0.57 to 6.49) | 0.2917 | 0.75 (0.09 to 6.47) | 0.796 |
| Model 2 + no valve dysfunction | – | – | 1 | – |
| Valve dysfunction | – | – | 0.43 (0.10 to 1.86) | 0.2575 |
| Model 2 + no systolic dysfunction or LVOTO | – | – | 1 | – |
| Systolic dysfunction or LVOTO | – | – | 0.28 (0.04 to 2.09) | 0.2132 |
| Model 2 + MRI scanner Ingenia | 1 | – | 1 | – |
| Intera | 2.36 (1.56 to 3.57) | <0.0001 | 1.74 (0.75 to 4.03) | 0.1946 |
| Model 2 + Fazekas scale | – | – | 1.94 (1.32 to 2.85) | 0.0008 |
In all models adding additional variables to model 2, the effect sizes of age and sex and phenotype divided subgroups remained similar and are therefore not presented. To reduce the false positive rate (type I errors), we regarded p values <0.01 as significant.
*Models did not converge, probably due to collinearity.
†The model was unable to run with non-classical patients included, probably due to the low number of non-classical patients with infarctions. Thus, for this analysis only classical patients were included.
AF, atrial fibrillation; BAD, basilar artery diameter; eGFR, estimated glomerular filtration rate; ERT, enzyme replacement therapy; LDL, low-density lipoprotein; LVMi, left ventricular mass index; LVOTO, left ventricular outflow tract obstruction.