Table 8. Imaging in prognosis.
| Light chain amyloidosis | Hereditary transthyretin amyloidosis (ATTRv) | Wild type transthyretin amyloidosis (ATTRwt) | |
| Clinical course | Rapid progression of HF, especially cardiac involvement | More variable, depends on the mutation | Conduction system disease present before HF |
| Worse than ATTR-CM | Presented with primary cardiomyopathy or peripheral autonomic polyneuropathy | Conduction system disease more common than ATTRv | |
| Worse outcome and EF than ATTRwt, especially in Val22Ile | One-third requires PPM | ||
| More common with atrial arrhythmias (40-60%) |
| Staging system | Kumar et al.117 (Mayo Clinic) | Gillmore et al.119 (NAC) (ATTRv and ATTRwt) | Grogan et al.32 (Mayo Clinic) (ATTRwt) |
| FLC-diff ≥ 18 mg/dL | eGFR < 45 mL/min/1.73 m2 | TnT > 0.05 ng/mL | |
| TnT ≥ 0.025 ng/mL | NT-proBNP > 3000 pg/mL | NT-proBNP > 3000 pg/mL | |
| NT-proBNP ≥ 1800 pg/mL |
| 5-year survival | Median survival | 4-year survival, median survival | ||||
| Stage I (0 parameters) | 68% | Stage I (0 parameters) | 69.2 months | Stage I (0 parameters) | 57%, 66 months | |
| Stage II (1 parameter) | 60% | Stage II (1 parameter) | 46.7 months | Stage II (1 parameter) | 42%, 40 months | |
| Stage III (2 parameters) | 28% | Stage III (2 parameters) | 24.1 months | Stage III (2 parameters) | 18%, 20 months | |
| Stage IV (3 parameters) | 14% |
| Lilleness et al.118 (Boston University) | Cheng et al.120 (Columbia University) (ATTRv and ATTRwt) | |
| TnI > 0.1 ng/mL | Mayo or NAC score (0-2 points) | |
| BNP > 81 pg/mL | Daily dose of furosemide or equivalent: | |
| 0 mg/kg (0 points); > 0-0.5 mg/kg (2 points); > 1 mg/kg (3 points) | ||
| NYHA class (I-IV) (1 to 4 points) |
| Median survival | Median survival | |||
| Stage I (0 parameters) | Not reached | 1-3 points | 90.5 months | |
| Stage II (1 parameter) | 112.8 months | 4-6 points | 38.5 months (Mayo Clinic) | |
| Stage III (2 parameters) | 51.6 months | 36.0 months (NAC) | ||
| Stage IIIb (2 parameters)* | 12 months | 7-9 points | 20.3 months (Mayo Clinic) | |
| 19.8 months (NAC) |
* TnI > 0.1 ng/mL and BNP > 700 pg/mL.
ATTRv, hereditary transthyretin amyloid cardiomyopathy; ATTRwt, wild type transthyretin amyloid cardiomyopathy; BNP, B-type natriuretic peptide; EF, ejection fraction; eGFR, estimated glomerular filtration rate calculated by the Modification of Diet in Renal Disease formula; FLC-diff, difference between involved and uninvolved free light chains; HF, heart failure; NAC, National Amyloidosis Center; NT-proBNP, N-terminal pro-B-type natriuretic peptide; NYHA, New York Heart Association; PPM, permanent pacemaker; TnI, troponin I; TnT, troponin T.