Total | No LE | LE | P values | |
n=146 | n=89 | n=57 | ||
Age at baseline (years) | 39±14 | 33±12 | 48±11 | <0.001 |
Male (n (%)) | 57 (39.0) | 34 (38.2) | 23 (40.4) | 0.795 |
BMI (kg/m²) | 24±5 | 23±4 | 25±5 | 0.006 |
BSA (m²) | 1.8±0.2 | 1.8±0.2 | 1.8±0.2 | 0.033 |
Heart rate (beats/min) | 67±12 | 68±12 | 66±12 | 0.228 |
Systolic blood pressure (mm Hg) | 123±19 | 120±19 | 127±19 | 0.040 |
Diastolic blood pressure (mm Hg) | 80±12 | 79±11 | 83±12 | 0.035 |
NYHA class (n (%)) | <0.001 | |||
I | 112 (76.7) | 79 (88.8) | 33 (57.9) | |
II | 24 (16.4) | 8 (9.0) | 16 (28.1) | |
III | 9 (6.2) | 2 (2.2) | 7 (12.3) | |
IV | 1 (0.7) | 0 | 1 (1.8) | |
Lyso-Gb3 (ng/mL) | 9.5 (4.1–24.1) | 8.9 (2.8–63.0) | 10.6 (6.9–19.0) | 0.782 |
Reduced enzyme activity (n (%)) | 123 (84.2) | 76 (85.4) | 47 (82.5) | 0.635 |
Enzyme replacement therapy | 79 (54.1) | 42 (53.2) | 37 (46.8) | 0.036 |
Kidney function | ||||
GFR (DTPA) (mL/min) | 105±32 | 112±28 | 93±35 | 0.001 |
Creatinine (mg/dL) | 0.8 (0.7–0.9) | 0.8 (0.7–0.9) | 0.9 (0.7–1.0) | 0.002 |
Cystatin C (mg/L) | 0.78 (0.70–0.90) | 0.76 (0.64–0.84) | 0.83 (0.73–1.08) | 0.001 |
Proteinuria (n (%)) | 50 (35.0) | 23 (25.8) | 27 (50.0) | 0.003 |
CKD stage III–V (n (%)) | 17 (11.6) | 9 (10.1) | 8 (14.0) | 0.471 |
Dialysis (n (%)) | 7 (4.8) | 2 (2.2) | 5 (8.8) | 0.110 |
Kidney transplantation (n (%)) | 3 (2.1) | 0 | 3 (5.3) | 0.058 |
NT-proBNP (pg/mL) | 91 (41–261) | 69 (30–132) | 267 (81–773) | <0.001 |
Cardiovascular history (n (%)) | ||||
Hypertensive heart disease | 40 (27.4) | 18 (20.2) | 22 (38.6) | 0.015 |
ICD/PM | 1 (0.7) | 0 | 1 (1.8) | 0.390 |
Neurological manifestations (n (%)) | ||||
Depression | 13 (8.9) | 4 (4.5) | 9 (15.8) | 0.034 |
TIA/stroke | 27 (18.5) | 15 (16.9) | 12 (21.1) | 0.524 |
Tinnitus | 53 (36.3) | 28 (31.5) | 25 (43.9) | 0.129 |
Hearing loss | 28 (19.2) | 15 (16.9) | 13 (22.8) | 0.373 |
Dysarthria | 4 (2.7) | 0 | 4 (7.0) | 0.011 |
Heat intolerance | 72 (49.3) | 40 (44.9) | 32 (56.1) | 0.235 |
Continuous variables are presented as mean±SD or median (quartiles), and categorical variables are presented with counts and percentages. P<0.05 vs no LE group.
BMI, body mass index; BSA, body surface area; CKD, chronic kidney disease; GFR (DTPA): glomerular filtration rate from 99mTc-diethylene triamine penta-acetic acid renography; ICD, implantable cardioverter defibrillator; LE, late gadolinium enhancement; Lyso-Gb3: plasma globotriaosylsphingosine; NT-proBNP, N-terminal pro-brain natriuretic peptide; NYHA, New York Heart Association; PCI, percutaneous coronary intervention; PM, pacemaker; TIA, transient ischaemic attack.