Table 1. Classification of amyloidosis with cardiac involvement.

	Main disorders	Amyloidogenic proteins	Main organ involvement	Extra-cardiac presentations	Outcomes and management
AL	Plasma cell dyscrasia	Light chain (Plasma cell dyscrasia)	Heart (~70%), kidney (~70%), liver (~40%), nerve (~20%)	Fatigue, ecchymosis, shoulder pad pain, Macroglossia, diarrhea	Chemotherapy, ASCT
ATTRv	Inherited ATTR gene mutation	Transthyretin	Nerve (major, ~70%), heart (30~100%), kidney (~20%)	Autonomic dysfunction, Spinal stenosis, CTS	TTR stabilizer, oligonucleotide therapy, liver transplantation
ATTRwt	Aging	Transthyretin	Heart (100%), kidney (~20%), nerve (~20%)	Spinal stenosis, CTS, Biceps tendon rupture	TTR stabilizer
AA	Systemic or inflammatory diseases	Serum amyloid A	Kidney (major, ~70%), heart (~5%), liver (~20%)	Nephropathy, hepatomegaly	Control of inflammatory disease
Aβ2M	Hemodialysis	β2-microglobulin	Heart (~80%), nerve (~16%),	CTS	No specific therapy
HGA	AD inherited form	Gelsolin	Nerve (~70%), eyes (~80%), skin (~80%), heart (~5%)	Cranial and peripheral neuropathy, corneal lattice dystrophy, cutis laxa (loose skin)	No specific therapy (similar life scan)

AA, serum amyloid A amyloidosis; AD, autosomal dominant; AL, amyloid light-chain; ASCT, autologous stem cell transplant; ATTRv, hereditary transthyretin amyloidosis; ATTRwt, wild-type transthyretin amyloidosis; Aβ2M, β2-microglobulin; CTS, carpal tunnel syndrome; HGA, hereditary gelsolin amyloidosis; TTR, transthyretin.