Prevalence of indicators in symptomatic subjects with moderate to severe IVST depending on TTR mutation category
| IVST Moderate and severe | Val30Met Early onset | Val30Met Late onset | non-Val30Met | WT |
|---|---|---|---|---|
| N | 10 | 39 | 118 | 85 |
| Clinical | ||||
| Age ≥55 years, n (%) | 5 (50) | 39 (100) | 85 (72) | 85 (100) |
| NYHA II–IV, n (%) | 2 (100) | 13 (87) | 65 (93) | 72 (91) |
| Gender, male, n (%) | 6 (60) | 31 (80) | 88 (75) | 80 (94) |
| SBP ≥140 mm Hg, n (%) | 3 (33) | 13 (37) | 8 (8) | 6 (8) |
| Sensory neuropathy, n (%) | 9 (90) | 37 (95) | 75 (64) | 25 (29) |
| Motor neuropathy, n (%) | 8 (80) | 30 (77) | 57 (48) | 7 (8) |
| ECG | ||||
| Low voltage, n (%) | 1 (17) | 2 (11) | 27 (38) | 24 (35) |
| Q waves, n (%) | 1 (17) | 8 (44) | 31 (38) | 28 (38) |
| Echocardiography | ||||
| LVEF <50%, n (%) | 0 (0) | 2 (15) | 53 (51) | 43 (55) |
| Posterior wall hypertrophy*, n (%) | 1 (10) | 17 (53) | 80 (71) | 67 (79) |
| IVST/PWT ratio> 1.3, n (%) | 3 (30) | 13 (41) | 23 (21) | 10 (12) |
| E/A >2, n (%) | 0 (0) | 1 (20) | 3 (14) | 3 (30) |
| E/Ea mean >15, n (%) | 0 (0) | NA | 3 (75) | 1 (25) |
| RV free wall thickness ≥7 mm, n (%) | 1 (33) | 7 (88) | 14 (74) | 8 (89) |
| LAD >40 mm, n (%) | 5 (56) | 28 (80) | 69 (73) | 69 (89) |
| Sparkling, n (%) | 4 (67) | 25 (74) | 56 (65) | 38 (62) |
| Aortic thickening, n (%) | 1 (33) | 2 (18) | 33 (46) | 18 (30) |
| Mitral thickening, n (%) | 0 (0) | 2 (18) | 37 (51) | 26 (43) |
| Tricuspid thickening, n (%) | NA | 1 (9) | 12 (17) | 7 (12) |
*Moderate or severe hypertrophy=women >13 mm and men >14 mm; NA, no data available for tricuspid valve thickening in early-onset Val30Met and for E/Ea in late onset for Val30Met.
IVST, interventricular septal thickness; LAD, left atrial diameter; LVEF, left ventricular ejection fraction; NYHA, New York Heart Association; PWT, posterior wall thickness; RV, right ventricle; SBP, systolic blood pressure.