Eleventh International Symposium HEART FAILURE & Co. MORTE IMPROVVISA SUDDEN DEATH Reggia di...
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Transcript of Eleventh International Symposium HEART FAILURE & Co. MORTE IMPROVVISA SUDDEN DEATH Reggia di...
Eleventh International SymposiumHEART FAILURE & Co.
MORTE IMPROVVISASUDDEN DEATH
Reggia di Caserta, 29-30 aprile 2011
L’amiloidosi: il danno cardiaco e le conseguenze
C. Quarta, C. Rapezzi
“.. an infiltrative CMP that looks like hypertrophic or hypertensive heart disease..”
Apo A
immunoglobulin
Lysozime
TTR
Terminology and Classification of Amyloidoses
Amyloid protein
AL
ATTR
AA
A ß2M
AApo AIIAFibAlys
….AßAPrP
Syndrome or involved tissue
Primary / Myeloma associated
Familial (PAF) Senile (wilde type TTR)
Secondary, reactive
Hemodyalisis associated
FamilialFamilialFamilial
….Alzheimer’s desease, agingSpongioform encephalopathies
Precursor
immunoglobulinlight chain
Transthyretin
Serum AA
ß2 microglobulin
Apolipoprotein AIIFibrinogen chainLysozyme
….Aß protein precursorPrion protein
Amyloid Heart Disease: Therapeutic Strategies
Heart & liver transplantation
high dose chemotherapy
autologous stem cell transplantation
Hereditary
ATTR
Liver transplantation
chemotherapy
AL
heart transplantation
“new stabilizer drugs”
SSAtreatment of heart failure
heart transplantation
AL(n=157)
ATTR(n=61)
SSA(n=15)
PValu
e
Restrictive filling pattern, n (%)
82/150 (55)
22/59 (37)
4/14 (29) 0.024
Dip-plateau morphology of RV pressure tracing, n (%)
1 (2) 1 (3) 1/12 (8) 0.329
Rapezzi et al, Circulation 2009:120:1203-1212
CONIGLIO
Perugini et al, Heart 2006;92:343-349
Baseline characteristics of pts with amyloidotic CMP (Bologna & Pavia, n=233)
AL(n=157)
SSA(n=15)
ATTR(n=61)
p Value
LBBB, n (%) 6/146 (4) 6 (40) 4/60 (7) <0.001
Total QRS score, mV 92 ± 36 120 ± 37
112 ± 34 <0.001
Low QRS voltage, n (%)
88/146 (60) 6 (40) 15/60 (25) <0.001
Any infarct pattern, %
101/146 (69)
10 (66) 41/59 (69) 0.98
LV mass among men (g)
339 ± 106 513 ± 162
392 ± 150 <0.0001
Diastolic IVS (mm) 16 ± 3 20 ± 4 17 ± 4 <0.0001
LV ejection fraction (%)
53 ± 13 44 ± 15 58 ± 13 <0.0001
Restrictive filling pattern, n (%)
82/150 (55) 4/14 (29)
22/59 (37) 0.02
Rapezzi et al, Circulation 2009;120:1203-1212
Log Rank p = 0.00010%
25%
50%
75%
100%
Su
rviv
al
15 7 2 1SSA61 28 9 3 1ATTR
157 69 36 15 7ALat risk
0 2 4 6 8years
ALATTRSSA
OVERALL SURVIVAL
Rapezzi et al, Circulation 2009;120:1203-1212
MACE FREE SURVIVAL
Log Rank p = 0.00480%
25%
50%
75%
100%
Su
rviv
al
14 6 1SSA57 26 10 4 1ATTR
131 53 27 10 5ALat risk
0 2 4 6 8years
ALATTRSSA
Vascular
localization AL 79%
ATTR 21% SSA 0%
p<0,0001
InflammationAL 14%ATTR 3%SSA 33% p 0,014
Rapezzi et al, Circulation 2009;120:1203-1212
•Direct toxic effect of circulating light chains
•Coronary (microvascular) involvement
•Duration/rapidity of amyloid accumulation
•………
Different prognosis and functional impairment
Sudden death?