Table 1 Cardiovascular magnetic resonance findings in Anderson-Fabry disease
From: CMR demonstration of multiple morphological phenotypes in Anderson-Fabry disease
| Â | Males (n=22) | Females (n=19) | p-value |
|---|---|---|---|
Age (years) | 47.4 (36.5-55.5) | 44.9 (34.9-60.8) | 0.896 |
Extracardiac involvement | 21 (95.5%) | 15 (78.9%) | 0.164 |
Prior enzyme replacement therapy | 17 (77.3%) | 11 (57.9%) | 0.313 |
EDWTmax (mm) | 14.6 (13.2-17.2) | 11.6 (8.9-13.5) | 0.013 |
No wall thickening | 4 (18.2%) | 12 (63.2%) | 0.005 |
Concentric hypertrophy | 14 (63.6%) | 5 (26.3%) | 0.027 |
Asymmetric septal hypertrophy | 3 (13.6%) | 1 (5.3%) | 0.610 |
Apical hypertrophy | 1 (4.5%) | 1 (5.3%) | 1.000 |
LV ejection fraction (%) | 58.3 (54.2-63.0) | 60.4 (57.3-65.6) | 0.200 |
LV end diastolic volume index (ml/m2) | 92.8 (72.1-111.3) | 83.3 (76.2-92.0) | 0.151 |
LV mass index (g/m2) | 78.0 (64.8-94.7) | 56.7 (51.5-63.0) | 0.001 |
Elevated LV mass index | 9 (40.9%) | 4 (21.1%) | 0.200 |
Myocardial scar | 16 (72.7%) | 13 (68.4%) | 1.000 |
Typical midwall lateral wall scar | 6 (27.3%) | 8 (42.1%) | 0.346 |
Patients with concentric hypertrophy and typical midwall lateral wall scar | 5 (22.7%) | 4 (21.1%) | 1.000 |
Patients with concentric hypertrophy and typical midwall lateral wall scar Scar as percentage of total myocardium at 4SD calculated from 16 males and 13 females with myocardial scar (%) | 4.9 (2.0-10.8) | 1.9 (1.2-10.2) | 0.381 |