Table 3.
Echocardiographic characteristics of Fabry cohorts (AGAL-009-00 and AGAL-008-00)
| All patients | Male | Female | M vs. F, P-value | |
|---|---|---|---|---|
| Number (%) of patients | 139a | 92a (66.2%) | 47 (33.8%) | |
| Heat rate (b.p.m.), mean ± SD | 66.7 ± 12.9 | 65.4 ± 12.3 | 69.5 ± 13.7 | <0.132 |
| LVEDV (mL), mean ± SDa | 96.1 ± 26.19 | 105.4 ± 25.5 | 78.5 ± 16.8 | <0.001 |
| LVESV (mL), mean ± SDa | 33.7 ± 16.3 | 38.5 ± 17.2 | 24.5 ± 8.9 | <0.001 |
| SV (mL), mean ± SDa | 66.1 ± 8.19 | 66.9 ± 14.8 | 53.9 ± 10.9 | <0.001 |
| EF (%), mean ± SDa | 62.4 ± 14.9 | 64.4 ± 8.4 | 69.3 ± 6.9 | <0.001 |
| Pts with EF < 55%a | 9 (6.7%) | 9 (9.8%) | 0 (0%) | <0.027 |
| LVEDD (cm), mean ± SD | 4.39 ± 0.54 | 4.54 ± 0.524 | 4.12 ± 0.484 | <0.001 |
| LVESD (cm), mean ± SD | 2.80 ± 0.48 | 2.89 ± 0.520 | 2.62 ± 0.344 | <0.001 |
| IVS (cm)a, mean ± SD | 1.43 ± 0.39 | 1.49 ± 0.41 | 1.33 ± 0.34 | <0.024 |
| PWT (cm)a, mean ± SD | 1.40 ± 0.30 | 1.42 ± 0.30 | 1.35 ± 0.30 | <0.236 |
| Mean radial WT (cm)a, mean ± SD | 1.45 ± 0.31 | 1.49 ± 0.31 | 1.38 ± 0.30 | <0.052 |
| Relative WTa (eccentricity), mean ± SD | 0.65 ± 0.18 | 0.65 ± 0.18 | 0.67 ± 0.19 | <0.642 |
| LV mass (g)a, mean ± SD | 303.4±131.2 | 332.7 ± 139.1 | 248.5 ± 94.0 | <0.001 |
| LVMI (g/m2)a, mean ± SD | 165.5 ± 66.9 | 178.7 ± 70.1 | 140.3 ± 52.5 | <0.001 |
| RVH, n (%) | 35 (25.9%) | 20 (21.7%) | 14 (29.8%) | <0.296 |
| RV hypokinesis, n (%) | 1 (0.7%) | 1 (1.1%) | 0 (0%) | ns |
| LAE (≥mild), n (%) | 56 (40.3%) | 35 (38.0%) | 21 (44.7%) | <0.450 |
| RAE (≥mild), n (%) | 30 (21.6%) | 21 (22.8%) | 9 (19.1%) | <0.618 |
| Bi-atrial enlargement, n (%) | 24 (17.3%) | 15 (16.3%) | 9 (19.1%) | ns |
| MR (≥moderate), n (%) | 15 (11.0%) | 12 (13.5%) | 3 (6.4%) | <0.024 |
| AR (>mild), n (%) | 3 (2.2%) | 3 (3.5%) | 0 (0%) | ns |
| Dilated aortic root (>40 mm diameter), n (%) | 3 (2.1%) | 3 (3.3%) | 0 (0%) | ns |
| TR(>mild), n (%) | 1 (0.7%) | 1 (5.3%) | 0 (0%) | ns |
| PASP (mmHg), n (%) | 31 ± 7.3 | 31 ± 7.1 | 31 ± 7.9 | ns |
| n | (n = 58, AGAL-009 cohort) | 21 | 37 | |
| Mitral E velocity (m/s), n (%) | 0.9 ± 0.2 | 0.9 ± 0.2 | 0.9 ± 0.2 | ns |
| Mitral A velocity (m/s), n (%) | 0.5 ± 0.2 | 0.5 ± 0.2 | 0.6 ± 0.3 | |
| E:A ratio, n (%) | 1.9 ± 0.9 | 2.2 ± 0.9 | 1.7 ± 0.8 | |
| TDI E′ velocity (cm/s), n (%) | −11.7±3.6 (n = 6) | −14.0 ± 3.6 | −9.7 ± 2.3 | |
| E/E′ ratio, n (%) | 8.7 ± 2.8 | 10.3 ± 2.1 | 7.1 ± 2.5 | |
LVEDV, left ventricular end-diastolic volume; LVESV, left ventricular end-systolic volume; LVEDD, left ventricular end-diastolic diameter; LVESD, left ventricular end-systolic diameter; SV, stroke volume; EF, ejection fraction; IVS, interventricular septal thickness; PWT, posterior wall thickness; WT, wall thickness; RVH, right ventricular hypertrophy; RV, right ventricular; LAE, left atrial enlargement; RAE, right atrial enlargement; MR, mitral regurgitation; AR, aortic regurgitation; TDI, tissue-Doppler imaging (septal); TR, tricuspid regurgitation; PASP, estimated pulmonary artery systolic pressure; ns, non-significant.
aThe echocardiogram of one male subject was of insufficient quality for accurate 2D measurements.
Bold values indicate P ≤ 0.05.