g registro guch
TRANSCRIPT
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I GUCH• CC: 0,8% dei nati vivi.
• 85% dei pz con CC raggiunge l’età adulta.
• Nei paesi occidentali le CC sono la prima causa di cardiopatia del giovane adulto e delle future madri.
• Negli USA i GUCH sono 20 milioni, in Inghilterra ci sono 1600 nuovi casi all’anno, in Canada il numero degli adulti con CC é aumentato del 270% in 10 anni.
• Un numero crescente di pazienti con CC complesse vive sino all’età adulta.
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I GUCH
• Gestione medico-chirurgica difficile e specializzata.• Collaborazione multidisciplinare.• Nuova specialità oggetto di raccomandazioni pubblicate da Società di Cardiologia nazionali ed internazionali. • In Europa ed nord America: centri dedicati, con supporto di reti, registri nazionali, protocolli diagnostico-terapeutici.
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I GUCH
• In Italia (dati ministeriali) ca80.000 GUCH
• Almeno la metà di essi ha bisogno di cure invasive
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I GUCH
• In Piemonte nascono ogni anno 280 bambini con CC.
• 238 raggiungono l’età adulta.• 48 hanno CC gravi.• Pazienti seguiti presso le
cardiologie pediatriche sino al 14° anno di vita.
• E poi????
In Piemonte ca 10.000 GUCH
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Alcuni esempi
Donna 60enne con TGA e SP nativa
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Alcuni esempi
Donna 40enne con ipoplasia ventricolare dx “ nativa”
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Alcuni esempi
Donna 60enne con CAVp nativo
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Donna 50enne con Fallot corretto e disfunzione ventricolare IMPARATO
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Eisenmenger
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REPORT REGISTRO GUCH PIEMONTE
G AGNOLETTI
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Registro nato nel gennaio 2013
• Inserimento a fine dicembre 2014: 461 pz
• Inserimento ad oggi: 536 pz
0
100
200
300
400
500
600
pz inseriti
giu-13
set-13
nov-13
gen-14
giu-14
dic-14
mar-15
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Centri attivi
0
2
4
6
8
10
12
centri
attivi
giu-13
settembre
novembre
gen-14
giu-14
dic-14
CARD OIRM S.ANNA 347 (TO)
CARD OSP SAVIGLIANO 100 (CN)
CARD2 OSP MOLINETTE 24 (TO)
CARD OSP BRA 19 (CN)
CARD CARD OSP NOVARA 10 (NO)
PNEU MOLINETTE 3 (TO)
CARD OSP MONDOVI 3 (CN)
CARD OSP MARTINI 3 (TO)
PNEU S.LUIGI ORBASSANO 1 (TO)
CARDCHIR MAURIZIANO 1 (TO)
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Pazienti
• Eta’ mediana 19 aa (18-83), età media 34.7 aa
• F 226
distribuzione dei sessi
maschifemmine
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Sindromi
N=59 (13%)
Sindromi n=59 (13%)
Sindromi
Tipi di Sindrome
Altre S T21
Alagille
Williams
Noonan
Di GeorgeVacterl
Turner
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Situs e punta del cuore
Situs e punta del cuore
1
2
3
SVS
SVI Destrocardia
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Tipi di cardiopatie
Distribuzione delle cardiopatie
DIA
Fallot
DIV
ArcoTGA
VU
SP
RVPA
CAV
DBP
AP
truncus
altro
tricuspide
DIA
Fallot
DIV
Arco
TGA
VU
SP
RVPA
CAV
DBP
AP
truncus
altro
tricuspide
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Palliazione
104 (22%)
apertura VD-APBH
BAP
BT
DCPP
fistola
unifocalizz.
waterston
apertura VD-AP
BH
BAP
BT
DCPP
fistola
unifocalizz.
waterston
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Chirurgia
N=452
bentall
DBP
DIA
DIV
DCPTCoAoKonnoRoss KonnoMustard/Senning
aortasubAo
mitrale
tricuspide
polmonare
Rastelli
Switch Altro
bentall
DBP
DIA
DIV
DCPT
CoAo
Konno
Ross Konno
Mustard/Senning
aorta
subAo
mitrale
tricuspide
polmonare
Rastelli
Switch
Altro
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Trattamento percutaneo
N=123 (27%)
PTA polmonare
PTA aortica
DBP
DIA
DIV
fenestrazione
stent aortico
stent polmonare
altro
PTA polmonare
PTA aortica
DBP
DIA
DIV
fenestrazione
stent aortico
stent polmonare
altro
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Clinica
problemi nel 26%
cianosi
endocardite
epatopatia
pb cerebrali
pb enteralipb polmonari
scompèenso
altro
PAH
Eisenmenger
cianosi
endocardite
epatopatia
pb cerebrali
pb enterali
pb polmonari
scompèenso
altro
PAH
Eisenmenger
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NYHA
NYHA I in 279 (60%)
NYHA I
NYHA II
NYHA III
NYHA IV
NYHA I
NYHA II
NYHA III
NYHA IV
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Clinica
Sovrappeso e obesità…
BMI >25 nel 26%...(e l’età media è <35 aa!)
BMI<25
BMI>25
BMI>30
BMI<25
BMI>25
BMI>30
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Aritmie
65 pz, 14%
FA
Flutter
rientro nodaleSSS
Tachi atriale
BAV 1-2
BAV 3
WPW
Flutter V
TVQt corto
FA
Flutter
rientro nodale
SSS
Tachi atriale
BAV 1-2
BAV 3
WPW
Flutter V
TV
Qt corto
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Trattamento interventistico delle aritmie
36 pz, 8%
EF interventistica
Ablazione
CVE
ICD
PM
altro
EF interventistica
Ablazione
CVE
ICD
PM
altro
••Ablazione:5Ablazione:5
••ICD:1ICD:1
••PM:18PM:18
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Farmaci
In 299 (65%)
antiaritmici
antiaggreganti
anticoagalanti
Ace-inibitori/sartanici
bosentan
sildenafil
BB
diuretici
calcio antagonisti
NO
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Pazienti > 30 aa
•N=224
•Lavoro:127 (57 %)
•Vita di coppia: 127 (57%)
F
lavoro
vita di coppia
gravidanze
figli
familiarita' per cc
F
lavoro
vita di coppia
gravidanze
figli
familiarita' per cc
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Vita al femminile
Donne > 30 aa
•F:118
•Lavoro: 63 (53%)
•3 laureate, 18 casalinghe, 23 impiegate….
•Vita di coppia: 65 (55%)
•Gravidanze:71
•Figli:107
•Familiarita’ per cc:6
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PAH N=21
Cardiopatie
SA
CAVp
DBP
DIA
DIV
ToF
IM
TGA
VU
SA
CAVp
DBP
DIA
DIV
ToF
IM
TGA
VU
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PAH
•PAH in 21(3.5%)
•Eisenmenger in 6 (1.3%)
•Sesso: 15 F
•Eta: mediana 61 aa, range 20-84 aa
•the prevalence of PAH among 1824 adult CHD
patients with septal defects was 6.1%, and 3.5%
had Eisenmenger syndrome
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PAH e aritmie
N= 14 (70%)
FA
rientro nodale
tachi atriale
flutter
TV
NOFA
rientro nodale
tachi atriale
flutter
TV
NO
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PAH
clinica
•Sat O2: 86+7 %
•BMI: 25.7++5.7
•NYHA I in 4, NYHA II in 8, NYHA 3 in 8
•1 decesso per scompenso CAVP nativo M 61 aa
•Sindromi: 1 Williams, 1 sindromi altro, 0 T21….
•Cateterismo in 13…RVP in 7…..
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PAH
Vita sociale
•Lavoro:7
•Vita di coppia: 11
•Gravidanze: 7 (15 donne)
•Figli: 9
Le donne con PAH possono avere Le donne con PAH possono avere figlifigli……
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FU
139 FU in 112 pz
I
II
III
I
II
III
NYHA I
NYHA II
NYHA III
NYHA IV
NYHA I
NYHA II
NYHA III
NYHA IV
NYHA II, I inserimento:84 (18%)NYHA II, I inserimento:84 (18%)
NYHA II, FU: 27 (24%)NYHA II, FU: 27 (24%)
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I inserimento-FU
FA
Flutter
Tachi atrialeSSS
TV
BAV
altro
FA
Flutter
Tachi atriale
SSS
TV
BAV
altro
FA
Flutter
rientro nodaleSSS
Tachi atriale
BAV 1-2
BAV 3
WPW
Flutter V
TVQt corto
FA
Flutter
rientro nodale
SSS
Tachi atriale
BAV 1-2
BAV 3
WPW
Flutter V
TV
Qt corto
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I inserimento-FU
Incidenza di aritmie da 14 a Incidenza di aritmie da 14 a
46%46%
i n c i d e n z a d i a r i t mi e a l p r i mo i n s e r i me n t o
ar it mie
n on ar it mie
i nc i denz a di ar i t mi e al FU
ar tmi e
non ar i tmi e
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Decessi
•2013 M 61aa CAVP: scompenso
•2013 M 52 aa BAV: emorragia cerebrale
•2012 M 45 aa VU DCPT: TEP
•2012 M 49 aa Fallot: morte improvvisa
•2012 F29 aa VU DCPT: decesso al TC
•2015 F 62 aa, Fallot nativo: evento cerebrale post KT
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Ad oggi inseriti
536 pazienti
(< 1/10 del previsto..)
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ATTIVITA DEL CENTRO GUCH•REGISTRO GUCH
•RIUNIONI GUCH bimestrali•CORSO GUCH annuale
•AMBULATORIO GUCH•AMBULATORIO multidisciplinare sul ventricolo unico
•AMBULATORIO multidisciplinare sulla cardiopatia e gravidanza•PUBBLICAZIONE Casi Clinici e materiale scientifico su : http://www.slideshare.net/guch-piemonte/presentations
Strutture coinvolteSC Cardiologia Pediatrica Presidio OIRM (Dr G Agnoletti) e SC Cardiologia 2,
Presidio Molinette (Dr S Marra)e-mail: [email protected], [email protected]
Segreteria OIRM 011-3135492 fax 0113135482Segreteria e fax Cardiologia 2: 0116335564
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58 PRESENTAZIONI PUBBLICATE
25.000 VISUALIZZAZIONI !
SLIDESHAREhttp://www.slideshare.net/guch-piemonte/presentations
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https://www.facebook.com/pages/Cardiopatie-Congenite-Adulti-GUCH-e-Bambini/1381961578706628?ref=stream
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• Ambulatorio GUCH (ogni lunedì pomeriggio, presidio OIRM. Tel 011 3135492)• Ricovero e valutazione emodinamica pazienteGUCH. Dr Agnoletti Tel 011 3135541 [email protected]• Ambulatorio gravida e cardiopatia Dr Agnoletti(Tel 0113135541) Dr Donvito (Tel 0113135734)
COME CONTATTARCI
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BUON LAVORO!!!