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HEARTLINE HSM Genoa Cardiology Meeting Paolo Ciliberti

HEARTLINE HSM Genoa Cardiology Meeting Paolo Ciliberti Great Ormond Street Hospital for Children - London. Catene leggere Ig. Transtiretina. Lisozima. Apolipoproteina. Terminology and Classification of Amyloidoses. Amyloid protein AL ATTR AA A ß 2 M AApo AII AFib Alys …. A ß

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HEARTLINE HSM Genoa Cardiology Meeting Paolo Ciliberti

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  1. HEARTLINE HSM Genoa Cardiology Meeting Paolo Ciliberti Great Ormond Street Hospital for Children - London

  2. Catene leggere Ig Transtiretina Lisozima Apolipoproteina

  3. Terminology and Classification of Amyloidoses Amyloid protein AL ATTR AA A ß2M AApo AII AFib Alys …. Aß APrP Precursor immunoglobulin light chain Transthyretin Serum AA ß2 microglobulin Apolipoprotein AII Fibrinogen  chain Lysozyme …. Aß protein precursor Prion protein 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

  4. C D B J Am Coll Cardiol. 2005;46:1076-84

  5. Pattern of myocardial uptake of 99mTc-DPD RV LV LV RV IVS IVS RA RA LA LA J Am Coll Cardiol 2005;46:1076-84

  6. J Am Coll Cardiol. 2005;46:1076-84

  7. Etiologic Diagnosis of Cardiac Amyloidosis Hereditary TTR Wild type TTR (senile) AL TTR immunostaining TTR DNA analysis Plasma cell dyscrasia + + - - - + + - -

  8. NEJM 2002;346:1786-91 …fibrinogen A or TTR mutations identified in 34 / 350 pts (9.7%) previously diagnosed as AL…

  9. Amyloidotic CMP: therapeutic implications of etiologic diagnosis chemotherapy AL heart transplantation high dose chemotherapy autologous stem cell transplantation Liver transplantation Hereditary ATTR Heart-liver transplantation

  10. Overallsurvival of patients with the three main types of systemic amyloidosis (233 pts) Circulation 2009:120:1203-1212

  11. M.A.C.E. FREE SURVIVAL (adjusted for multiorgan involvement) Circulation 2009:120:1203-1212

  12. Attività clinica • GOSH ECHO LAB Lead Consultant Jan Marek -Complex congenital heart defect -3D echo -Dyssyncrony assesment in pediatric population

  13. Principali progetti di ricerca • Screening familiare della CMPI • in età pediatrica • Modalità di esecuzione • Timing • - Costo-efficacia • (GOSH-The Heart Hospital) • Outcome clinico-strumentale dei pazienti • con stenosi aortica critica neonatale • avviati a strategia riparativa biventricolare • (GOSH1970-2010- Deutsches Herzzentrum, Munich)

  14. DIAGNOSI DI CARDIOMIOPATIA AMILOIDOTICA

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