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Eleventh International Symposium HEART FAILURE & Co. MORTE IMPROVVISA SUDDEN DEATH

L’amiloidosi: il danno cardiaco e le conseguenze C. Quarta, C. Rapezzi. Eleventh International Symposium HEART FAILURE & Co. MORTE IMPROVVISA SUDDEN DEATH Reggia di Caserta, 29-30 aprile 2011. “.. an infiltrative CMP that looks like hypertrophic or hypertensive heart disease..”. Apo A.

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Eleventh International Symposium HEART FAILURE & Co. MORTE IMPROVVISA SUDDEN DEATH

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  1. L’amiloidosi: il danno cardiaco e le conseguenze C. Quarta, C. Rapezzi Eleventh International Symposium HEART FAILURE & Co. MORTE IMPROVVISA SUDDEN DEATH Reggia di Caserta, 29-30 aprile 2011

  2. “.. an infiltrative CMP that looks like hypertrophic or hypertensive heart disease..”

  3. Apo A immunoglobulin Lysozime TTR

  4. Terminology and Classification of Amyloidoses Syndrome or involved tissue Primary / Myeloma associated Familial (PAF) Senile (wilde type TTR) Secondary, reactive Hemodyalisis associated Familial Familial Familial …. Alzheimer’s desease, aging Spongioform encephalopathies Precursor immunoglobulin light chain Transthyretin Serum AA ß2 microglobulin Apolipoprotein AII Fibrinogen  chain Lysozyme …. Aß protein precursor Prion protein Amyloid protein AL ATTR AA A ß2M AApo AII AFib Alys …. Aß APrP

  5. Amyloid Heart Disease: Therapeutic Strategies chemotherapy AL high dose chemotherapy autologous stem cell transplantation heart transplantation Hereditary ATTR Liver transplantation Heart & liver transplantation “new stabilizer drugs” treatment of heart failure SSA heart transplantation

  6. Rapezzi et al, Circulation 2009:120:1203-1212

  7. CONIGLIO

  8. Perugini et al, Heart 2006;92:343-349

  9. Baseline characteristics of pts with amyloidotic CMP (Bologna & Pavia, n=233) Rapezzi et al, Circulation 2009;120:1203-1212

  10. OVERALL SURVIVAL MACE FREE SURVIVAL Rapezzi et al, Circulation 2009;120:1203-1212

  11. Vascular localization AL 79% ATTR 21% SSA 0% p<0,0001 Inflammation AL 14% ATTR 3% SSA 33% p 0,014 Rapezzi et al, Circulation 2009;120:1203-1212

  12. Different prognosis and functional impairment • Direct toxic effect of circulating light chains • Coronary (microvascular) involvement • Duration/rapidity of amyloid accumulation • ………

  13. Sudden death ?

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