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Arrhythmogenic right ventricular dysplasia C U V I.
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Transcript of Arrhythmogenic right ventricular dysplasia C U V I.
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MIOCARDITE:
PRESENTAZIONE CLINICA ED EVOLUZIONE
DISRITMIE
ASSENZA DI DOLORE aritmie ipocinetiche FORMA
SINTOMI TORACICO aritmie ipercinetiche SCC FULMIN.
Guarigione CMPD Guarigione Morte CMPD Morte(completa o (completa o Improvvisaincompleta) incompleta)
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Arrhythmogenic right ventricular dysplasia
C UVI
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ARVD
• Dilatazione e disfunzione del ventricolo destro• Infiltrazione fibro-adiposa o fibrosa• Talora espansione al ventricolo sinistro• CHF prevalentemente destro• Aritmie ventricolari, sincope, SD• Familiare > 50%, trasmission AD• Frequenza: variabile, founder effect• Diagnosi: Guidelines (criteri maggiori e minori)
McKenna et alC UVI
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Arrhythmogenic right ventricular dysplasia
C UVI
LOCUS PROTEIN FREQUENCY CHROMOSOMEARVD1 ? ? 14q23-q24ARVD2 cardiac ryanodine receptor rare? 1q42-q43ARVD3 ? 14q12-q22ARVD4 desmoplakin ? 2q32.1-q32.3ARVD-AR plakoglobin Naxos (AR) 17q21ARVD6 ? (muscular dystrophy) 10p12-p14
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AMILOIDOSI• (fibrille 7-10 nm; aggregati in 2-5 filamenti in strutture
antiparallele o beta-pieghettate);• PRIMARIA (AL);85%; s. nefrosica; s. tunnel carpale;
neuropatia sensori-motoria; ipotensione ortostatica (trombocitosi, porpora, macroglossia, raucedine;etc)
• SECONDARIA (AA) AR; S. Sjogren; mal. infiammatorie croniche intestinali; (M. di Crohn); TBC; Lebbra;
• AMILOIDOSI FAMILIARE (FAP); autosomica dominante; <4° decade; PNP; disturbi GI o vescicali;
• SENILE; assenza componente monoclonale; assente interessamento extracardiaco; 20-65% cuori espiantati >90 anni;
• IRC; beta2mcg.
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A Trial of Three Regimens for Primary Amyloidosis: Colchicine
alone, Melphalan and Prednisone, and Melphalan, Prednisone and Colchicine
N Engl J Med 1997; 336: 1202-7
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