heartline hsm genoa cardiology meeting paolo ciliberti great ormond street hospital for children -...
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HEARTLINE HSM Genoa Cardiology Meeting
Paolo CilibertiGreat Ormond Street Hospital for Children - London
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Apolipoproteina
Catene leggere Ig
Lisozima
Transtiretina
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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
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B C D
J Am Coll Cardiol. 2005;46:1076-84
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LVLV
RVRV
RARALA LA
IVS IVS
Pattern of myocardial uptake of 99mTc-DPD
J Am Coll Cardiol 2005;46:1076-84
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J Am Coll Cardiol. 2005;46:1076-84
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TTR immunostaining
TTR DNA analysis
Plasma cell dyscrasia
AL Hereditary TTR
Wild type TTR
(senile)
-
- -
-+
+
-
+ +
Etiologic Diagnosis of Cardiac Amyloidosis
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NEJM 2002;346:1786-91
…fibrinogen A or TTR mutations identified in 34 / 350 pts (9.7%) previously diagnosed as AL…
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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
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Overall survival of patients with the three main types of systemic amyloidosis (233 pts)
Circulation 2009:120:1203-1212
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M.A.C.E. FREE SURVIVAL(adjusted for multiorgan involvement)
Circulation 2009:120:1203-1212
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Attività clinica• GOSH ECHO LABLead Consultant Jan Marek
-Complex congenital heart defect
-3D echo
-Dyssyncrony assesment in pediatric population
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• 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 neonataleavviati a strategia riparativa biventricolare(GOSH1970-2010- Deutsches
Herzzentrum, Munich)
Principali progetti di ricerca
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DIAGNOSI DI CARDIOMIOPATIA AMILOIDOTICA
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