Table 1. Clinical, echocardiographic and cardiac magnetic resonance imaging characteristics in different infiltrative cardiomyopathies.
| Variables | FA (n=20) | FD (n=23) | CA (n=14) | P value |
|---|---|---|---|---|
| Age (years) | 33±14 | 42±14 | 66±9*† | <0.001 |
| Age range (years) | 19–68 | 20–68 | 48–83 | <0.001 |
| Male [n (%)] | 14 (70.0) | 10 (43.5) | 9 (64.3) | 0.183 |
| BMI (kg/m2) | 24±4 | 25±6 | 25±5 | 0.649 |
| hs-TNT (pg/mL) | 14 [9–21] | 18 [8–22] | 98 [61–139]*† | <0.001 |
| NT-proBNP (pg/mL) | 40 [16–77] | 131 [62–349]* | 4110 [2,453–8,735]*† | <0.001 |
| Creatinine (mg/dL) | 0.9±0.2 | 0.9±0.2 | 1.6±0.8*† | <0.001 |
| eGFR (mL/min/1.73 m2) | 101±21 | 82±22* | 61±21*† | <0.001 |
| NYHA class [n (%)] | <0.001 | |||
| I | 16 (80.0) | 14 (60.9) | 0 (0) | |
| II | 2 (10.0) | 5 (21.7) | 10 (71.4) | |
| III | 1 (5.0) | 4 (17.4) | 3 (21.4) | |
| IV | 1 (5.0) | 0 (0) | 1 (7.1) | |
| NYHA class III–IV [n (%)] | 2 (10.0) | 4 (17.4) | 4 (28.6) | 0.375 |
| Echocardiography | ||||
| LV end-diastolic dimension (mm) | 45±6 | 46±4 | 44±5 | 0.296 |
| IVSd (mm) | 10.3±0.5 | 11.7±0.8* | 13.9±2.3*† | <0.001 |
| PWd (mm) | 10.1±0.5 | 11.2±1.0* | 13.6±2.0*† | <0.001 |
| LAD (mm) | 35±6 | 36±4 | 40±5* | 0.005 |
| LVEF (%) | 58±7 | 66±9* | 62±11 | 0.007 |
| E/A | 1.59±0.83 | 1.47±0.55 | 1.34±0.52 | 0.577 |
| E/E’ | 7.5±1.9 | 13.4±4.6* | 21.7±8.5*† | <0.001 |
| DT (ms) | 177±57 | 223±45*† | 181±60 | 0.013 |
| Global LSsys (%) | −15±2 | −15±4 | −12±5*† | 0.030 |
| Basal septal LSsys (%) | −13±3 | −14±3 | −8±5*† | <0.001 |
| Mid septal LSsys (%) | −15±4 | −16±4 | −11±5*† | 0.001 |
| Apical septal LSsys (%) | −18±5 | −20±6 | −19±7 | 0.644 |
| Ratio LSsysapi/bas | 1.5±0.7 | 1.6±0.7 | 2.9±1.3*† | <0.001 |
| Magnetic resonance imaging | ||||
| LE positive [n (%)] | 14/19 (73.7) | 16/23 (69.6) | 9/9 (100.0) | 0.177 |
| LVMI (g/m2) | 72±23 | 93±27* | 109±32* | 0.004 |
| End-diastolic LV volume index (mL/m2) | 71±20 | 75±18 | 81±26 | 0.538 |
| End-systolic LV volume index (mL/m2) | 31±13 | 24±12 | 33±9 | 0.089 |
| Stroke volume index (mL/m2) | 39±9 | 51±12* | 40±13 | 0.005 |
| Cardiac index (L/min/m2) | 2.9±0.7 | 3.2±0.6 | 3.0±0.9 | 0.471 |
| LVEF (%) | 57±9 | 68±9* | 58±7† | <0.001 |
*, P<0.05 vs. FA; †, P<0.05 vs. FD. FA, Friedreich’ Ataxia; FD, Fabry disease; CA, light-chain cardiac amyloidosis; BMI, body mass index; hs-TNT, high sensitive troponin T; NT-proBNP, N-terminal pro-brain natriuretic peptide; eGFR, estimated glomerular filtration rate; NYHA, New York Heart Association; LV, left ventricular; LVEDD, left ventricular end-diastolic dimension; IVSd, interventricular septal end-diastolic wall thickness; PWd, posterior wall thickness; LAD, left atrium anteroposterior diameter; LVEF, left ventricular ejection fraction; E/A, early (E-wave) to late (A-wave) mitral inflow velocity ratio; DT, E-wave deceleration time; E’, Tissue Doppler derived early-diastolic mitral annular velocity; LSsys, longitudinal systolic strain; LSsysapi/bas, the ratio between apical and basal septal LSsys; LE, late enhancement; LVMI, LV mass indexed to body surface area.