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

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Presentazione sul tema: "HEARTLINE HSM Genoa Cardiology Meeting Paolo Ciliberti Great Ormond Street Hospital for Children - London."— Transcript della presentazione:

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

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3 Apolipoproteina Catene leggere Ig Lisozima Transtiretina

4 Terminology and Classification of Amyloidoses Amyloid protein AL ATTR AA A ß 2 M AApo AII AFib Alys …. Aß APrP Syndrome or involved tissue Primary / Myeloma associated Familial (PAF) Senile (wilde type TTR) Secondary, reactive Hemodyalisis associated Familial …. Alzheimer’s desease, aging Spongioform encephalopathies Precursor immunoglobulin light chain Transthyretin Serum AA ß 2 microglobulin Apolipoprotein AII Fibrinogen  chain Lysozyme …. Aß protein precursor Prion protein

5 B C D J Am Coll Cardiol. 2005;46:

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

7 J Am Coll Cardiol. 2005;46:

8 TTR immunostaining TTR DNA analysis Plasma cell dyscrasia AL Hereditary TTR Wild type TTR (senile) Etiologic Diagnosis of Cardiac Amyloidosis

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

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

11 Overall survival of patients with the three main types of systemic amyloidosis (233 pts) Circulation 2009:120:

12 M.A.C.E. FREE SURVIVAL (adjusted for multiorgan involvement) Circulation 2009:120:

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

16 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 (GOSH Deutsches Herzzentrum, Munich) Principali progetti di ricerca

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18 DIAGNOSI DI CARDIOMIOPATIA AMILOIDOTICA

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