Table 5.
Cardiac and extracardiac amyloidosis red flags
| Type | Red flag | Amyloidosis where it is most frequently found |
|---|---|---|
| Extracardiac | ||
| Clinical | Polyneuropathy | ATTRv, AL, AA, AGel |
| Dysautonomia | ATTR, AL | |
| Skin bruising | AL | |
| Skin discoloration | AApoAI | |
| Cutis laxa | AGel | |
| Macroglossia | AL | |
| Deafness | ATTRwt | |
| Bilateral carpal tunnel syndrome | ATTRv, ATTRwt | |
| Ruptured biceps tendon | ATTRwt | |
| Lumbar spinal stenosis | ATTRwt | |
| Vitreous deposits | ATTRv | |
| Corneal lattice dystrophy | AGel | |
| Family history | ATTRv, AApoAI, AApoAII | |
| Laboratory | Renal insufficiency | AL, AA, AApoAI, AApoAII, AApoAIV, Aβ2M, AFib |
| Proteinuria | AL, AA, AApoAI, AApoAII, AFib | |
| Cardiac | ||
| Clinical | Hypotension or normotensive if previous hypertensive | ATTR, AL |
| ECG | Pseudoinfarct pattern | All |
| Low/decreased QRS voltage to degree of LV thickness | All | |
| AV conduction disease | All | |
| Laboratory | Disproportionally elevated NT-proBNP to degree of HF | All |
| Persisting elevated troponin levels | ATTR, AL | |
| Echocardiogram | Granular sparkling of myocardium | All |
| Increased right ventricular wall thickness | All | |
| Increased valve thickness | All | |
| Pericardial effusion | All | |
| Reduced longitudinal strain with apical sparing pattern | All | |
| CMR | Subendocardial late gadolinium enhancement | All |
| Elevated native T1 values | All | |
| Increased extracellular volume | All | |
| Abnormal gadolinium kinetics | All |
AA, serum amyloid A amyloidosis; AApoAI, apolipoprotein AI amyloidosis; AApoAII, apolipoprotein AII amyloidosis; AApoAIV, apolipoprotein A-IV amyloidosis; Aβ2M, β2-microglobulin amyloidosis; AFib, fibrinogen amyloidosis; AGel, gelsolin amyloidosis; AL, light-chain amyloidosis; ATTRv, hereditary transthyretin amyloidosis; ATTRwt, wild-type transthyretin amyloidosis; AV, atrio-ventricular; CMR, cardiac magnetic resonance; ECG, electrocardiogram; HF, heart failure; LV, left ventricular; NT-proBNP, N-terminal pro-B-type natriuretic peptide.