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Ospedale Pediatrico Bambino Gesù, Cardiologia
G. Rinelli
Follow Up
a medio e lungo termine
delle
Cardiopatie Congenite
F.U...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prevalenza: 6-10/1000 nati vivi
Maschi/Femmine: Uguale
CC Isolata 80 -85%
C...
Urgenze Neonatali
Non Urgenze Neonatali
Gruppo Fisiopatologico
Classificazione
Clinica
F.Up delle Cardiopatie Congenite - G.Rinelli -
Complesse
Semplici
Gruppo Fisiopatologico
Anatomica
Prognosi
Classificazion...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Complesse
Semplici
Gruppo Fisiopatologico
Anatomica
Prognosi
Classificazion...
 Circuiti in parallelo
Trasposizione delle grandi Arterie
 Dotto dipendenza polmonare
Atresia Polmonare a setto intatt...
Epidemiologia
Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26.
CC S...
Epidemiologia
Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26.
12,5...
Epidemiologia
Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26.
12,5...
Epidemiologia
Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26.
CC S...
Epidemiologia
Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26.
F.Up...
Epidemiologia
Lazio = 45.000 nati/anno
Circa 60 urgenze neonatali/anno
Circa 365 cardiopatici/anno
Circa 300 CC semplici/a...
Prognosi
Dove stanno ?
Che fanno ?
Dove vanno ?
'Ndo state? Che fate? 'Nd'annate?
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346
AVSD atrioventricular septal defect; I/HAA, interrupted or hypoplasti...
Cumulative postoperative survival until 16 years of age or the
end of follow-up in the combined group of patients with com...
Gunnar Erikssen et al. Circulation. 2015;131:337-346
Number of deaths related to congenital heart defects (International C...
Kaplan–Meier plots of reoperation-free survival in the combined group of patients with complex
congenital heart defects wh...
One-year survival in patients with complex congenital heart defects born in 1975 to 1979, 1980
to 1984, 1985 to 1989, 1990...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza
1 anno di età 80%
15 anni di età 72%
Knowles RL et al. PLoS O...
Prognosi
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
81/10000 Prevalenza CC Congenite
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie semplici
1. Difetto I...
81/10000 Prevalenza CC Congenite
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Funzi...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Biventricolari
14/81 (ogni 10000 nati ...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Biventricolari
• Fallot 5/10000
• Cana...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Biventricolari
• Fallot 5/10000
• Cana...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Tetralogia di Fallot
Prognosi
Knowles RL et al. PLoS One. 2014 Sep 10;9(8):...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346
Tetralogia di...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Tetralogia di Fallot
Correzione
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Tetralogia di Fallot
Intervento correttivo (patch transanulare)
Pa...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Biventricolari
• Fallot 5/10000
• Cana...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Canale Atrio Ventricolare
Prognosi
Knowles RL et al. PLoS One. 2014 Sep 10;...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346
Canale Atrio ...
Ostruzioni destre
Canale Fallot
S.Down Fenotipo Normale
Ostruzioni sinistre
Ipoplasia VS
Mitrale a paracadute
Stenosi sott...
Giornate Akragantine di Cardiologia Pediatrica - CAV -
CAV Completo
Down Non Down
Secondo Clinica
Entro 4 Mesi Secondo
Cli...
CAV Parziale
In genere timing come un
Difetto Interatriale
Secondo
Clinica
Insufficienza Mitralica
Ipertensione Polmonare
...
CAV Parziale con S. Shone
1 Stadio Coartazione
2 Stadio CAVp
Mitrale ?
Prognosi da molto buona ad infausta a secondo
della...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Biventricolari
• Fallot 5/10000
• Cana...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Intervento
Switch (Intervento di Jatene)
Italia: eseguito fino al 90
Problemi:
Disfunzione Ventricolare dx
PMK a distanza 99% pts
Futuro Trapianto ?
F.Up delle Car...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Switch (Intervento di Jatene)
Prognosi
Knowles RL et al. PLoS One. 2014 Sep...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Switch (Intervento di Jatene)
Prognosi
Gunnar Erikssen et al. Circulation. ...
F.Up delle Cardiopatie Congenite - G.Rinelli -
DORV
Prognosi
• Switch
• Rastelli
• REV
• Nicaidoh
F.Up delle Cardiopatie Congenite - G.Rinelli -
DORV
Prognosi
Rastelli
REV
F.Up delle Cardiopatie Congenite - G.Rinelli -
DORV
Prognosi
Rastelli (OPBG) 43 (pts) 1984 -2014
Sopravvivenza 87.5 % a 10...
F.Up delle Cardiopatie Congenite - G.Rinelli -
DORV
Prognosi
Rastelli (OPBG) 43 (pts) 1984 -2014
Freedom from Reinterventi...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Biventricolari
• Fallot 5/10000
• Cana...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Truncus
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346
F.Up delle Cardiopatie Congenite - G.Rinelli -
Truncus
Prognosi
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Biventricolari
• Fallot 5/10000
• Cana...
F.Up delle Cardiopatie Congenite - G.Rinelli -
VD Piccolo
(sospetto ECO sinusoidi )
Chirurgia (1° Stadio)
Shunt SP
PGE1 (0...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Chirurgia (2° Stadio)
Glenn
(1 ventricolo e mezzo)
Biventricolare
Chiusura ...
APSI
Prognosi
Knowles RL et al. PLoS One. 2014 Sep 10;9(8):e106806. doi: 10.1371/journal.pone.0106806. eCollection 2014.
F.Up delle Cardiopatie Congenite - G.Rinelli -
APSI
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346
F.Up delle Cardiopatie Congenite - G.Rinelli -
Atresia Polmonare
con
Difetto Interventricolare
Prognosi
Tratto d’efflusso ventricolare destro:
- atresia infundibolare
- atresia giunzione infundibolo-polmonare
Difetto intervent...
No Mapcas (Tetralogia di Fallot con Atresia Polmonare)
Shunt
Correzione
(Dopo pochi mesi)
F.Up delle Cardiopatie Congenite...
Basi della Chirugia Correttiva
Necessità di convertire il pattern multifocale di
perfusione polmonare in una fonte unica:
...
Chirurgia
Diagnosi: Eco
Ricostruzione palliativa tratto efflusso VD
Procedure di unifocalizzazione:
Chiusura DIV (Correzio...
Timing Chirurgico
Diagnosi: Eco
1-5 Mesi Cateterismo
Dipende da Sat O2
Paziente Stabile Cianotico
1 ° Chirurgia 5-8 Kg
Paz...
January 1994 – September 2014
 Number of pts
 1994-2000
 2001-2009
118
21 (17.8%)
97 (82.2%)
 Chromosome 22q11 deletio...
1 year: 86.4 % (95%CI: 78.4-91.6)
5 and 10 years: 83.9 % (95%CI: 75.3-89.7)
Rehabilitation Unifocalization Total
Study Par...
Honjo 2009 20 18 0.63 15 0.53 2.5
AP + DIV
Prognosi OPBG
Carotti et all, J Thorac Cardiovasc Surg. 2010 Nov;140(5):1092-10...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Univentricolari
(Funzionalmente)
6/81 ...
1. Neonatale
2. Glenn dopo qualche mese
3. Fontan dopo 2-3 anni
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Unive...
Modulazione flusso Polmonare
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Univentricolare
Trattamento
Se Troppo  ...
Atresia Aortica (HLHS – HLHS like)
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Univentricolare
Trattamento
1. For...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Norwood Sano
HLHS
Trattamento 1 stadio
Norwood Classica
Ibrido 1° Stadio
F.Up delle Cardiopatie Congenite - G.Rinelli -
HLHS
Trattamento 1° Stadio
Norwood II stadio (Glenn)
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Univentricolare
Trattamento
Ibrido 2° Stadi...
Norwood III stadio (Fontan)
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Univentricolare
Trattamento
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore funzionalmente
Univentricolare
Prognosi
Cuore sinistro Ipoplasico/lik...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore funzionalmente
Univentricolare
Prognosi
Cuore destro Ipoplasico
 Atr...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore FunzionalmenteUniventricolare
Prognosi
Knowles RL et al. PLoS One. 20...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Funzionalmente Univentricolare
Prognosi
Gunnar Erikssen et al. Circul...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Funzionalmente Univentricolare
Prognosi
Gunnar Erikssen et al. Circul...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore sn Ipoplasico vs Cuore dx Ipoplasico
Prognosi
Yves d’Udekem et al. JA...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Ibrido vs Norwood I
Prognosi
Photiadis J et all Thorac Cardiovasc Surg. 201...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Ibrido vs Norwood I
Prognosi
Baba K et all Circulation 2012 Sep 11;126(11 S...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Ibrido vs Norwood I
Prognosi
Baba K et all Circulation 2012 Sep 11;126(11 S...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Ibrido vs Norwood I
Prognosi
Alexander A Brescia et all J Thorac Cardiovasc...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza dopo la Fontan OPBG
Prognosi
Salvatore Giannico et all JACC V...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza dopo la Fontan OPBG
Prognosi
Salvatore Giannico et all JACC V...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza dopo la Fontan OPBG
Prognosi
Salvatore Giannico et all JACC V...
F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza dopo la Fontan OPBG
Prognosi
Salvatore Giannico et all JACC V...
Conclusioni Come vanno
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Semplici
Benissimo !!!
Correzio...
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  • DOWNLOAD THIS BOOKS INTO AVAILABLE FORMAT (2019 Update) ......................................................................................................................... ......................................................................................................................... Download Full PDF EBOOK here { https://soo.gd/irt2 } ......................................................................................................................... Download Full EPUB Ebook here { https://soo.gd/irt2 } ......................................................................................................................... Download Full doc Ebook here { https://soo.gd/irt2 } ......................................................................................................................... Download PDF EBOOK here { https://soo.gd/irt2 } ......................................................................................................................... Download EPUB Ebook here { https://soo.gd/irt2 } ......................................................................................................................... Download doc Ebook here { https://soo.gd/irt2 } ......................................................................................................................... ......................................................................................................................... ................................................................................................................................... eBook is an electronic version of a traditional print book THIS can be read by using a personal computer or by using an eBook reader. (An eBook reader can be a software application for use on a computer such as Microsoft's free Reader application, or a book-sized computer THIS is used solely as a reading device such as Nuvomedia's Rocket eBook.) Users can purchase an eBook on diskette or CD, but the most popular method of getting an eBook is to purchase a downloadable file of the eBook (or other reading material) from a Web site (such as Barnes and Noble) to be read from the user's computer or reading device. Generally, an eBook can be downloaded in five minutes or less ......................................................................................................................... .............. Browse by Genre Available eBooks .............................................................................................................................. Art, Biography, Business, Chick Lit, Children's, Christian, Classics, Comics, Contemporary, Cookbooks, Manga, Memoir, Music, Mystery, Non Fiction, Paranormal, Philosophy, Poetry, Psychology, Religion, Romance, Science, Science Fiction, Self Help, Suspense, Spirituality, Sports, Thriller, Travel, Young Adult, Crime, Ebooks, Fantasy, Fiction, Graphic Novels, Historical Fiction, History, Horror, Humor And Comedy, ......................................................................................................................... ......................................................................................................................... .....BEST SELLER FOR EBOOK RECOMMEND............................................................. ......................................................................................................................... Blowout: Corrupted Democracy, Rogue State Russia, and the Richest, Most Destructive Industry on Earth,-- The Ride of a Lifetime: Lessons Learned from 15 Years as CEO of the Walt Disney Company,-- Call Sign Chaos: Learning to Lead,-- StrengthsFinder 2.0,-- Stillness Is the Key,-- She Said: Breaking the Sexual Harassment Story THIS Helped Ignite a Movement,-- Atomic Habits: An Easy & Proven Way to Build Good Habits & Break Bad Ones,-- Everything Is Figureoutable,-- What It Takes: Lessons in the Pursuit of Excellence,-- Rich Dad Poor Dad: What the Rich Teach Their Kids About Money THIS the Poor and Middle Class Do Not!,-- The Total Money Makeover: Classic Edition: A Proven Plan for Financial Fitness,-- Shut Up and Listen!: Hard Business Truths THIS Will Help You Succeed, ......................................................................................................................... .........................................................................................................................
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  • DOWNLOAD THIS BOOKS INTO AVAILABLE FORMAT (2019 Update) ......................................................................................................................... ......................................................................................................................... Download Full PDF EBOOK here { https://soo.gd/irt2 } ......................................................................................................................... Download Full EPUB Ebook here { https://soo.gd/irt2 } ......................................................................................................................... Download Full doc Ebook here { https://soo.gd/irt2 } ......................................................................................................................... Download PDF EBOOK here { https://soo.gd/irt2 } ......................................................................................................................... Download EPUB Ebook here { https://soo.gd/irt2 } ......................................................................................................................... Download doc Ebook here { https://soo.gd/irt2 } ......................................................................................................................... ......................................................................................................................... ................................................................................................................................... eBook is an electronic version of a traditional print book THIS can be read by using a personal computer or by using an eBook reader. (An eBook reader can be a software application for use on a computer such as Microsoft's free Reader application, or a book-sized computer THIS is used solely as a reading device such as Nuvomedia's Rocket eBook.) Users can purchase an eBook on diskette or CD, but the most popular method of getting an eBook is to purchase a downloadable file of the eBook (or other reading material) from a Web site (such as Barnes and Noble) to be read from the user's computer or reading device. Generally, an eBook can be downloaded in five minutes or less ......................................................................................................................... .............. Browse by Genre Available eBooks .............................................................................................................................. Art, Biography, Business, Chick Lit, Children's, Christian, Classics, Comics, Contemporary, Cookbooks, Manga, Memoir, Music, Mystery, Non Fiction, Paranormal, Philosophy, Poetry, Psychology, Religion, Romance, Science, Science Fiction, Self Help, Suspense, Spirituality, Sports, Thriller, Travel, Young Adult, Crime, Ebooks, Fantasy, Fiction, Graphic Novels, Historical Fiction, History, Horror, Humor And Comedy, ......................................................................................................................... ......................................................................................................................... .....BEST SELLER FOR EBOOK RECOMMEND............................................................. ......................................................................................................................... Blowout: Corrupted Democracy, Rogue State Russia, and the Richest, Most Destructive Industry on Earth,-- The Ride of a Lifetime: Lessons Learned from 15 Years as CEO of the Walt Disney Company,-- Call Sign Chaos: Learning to Lead,-- StrengthsFinder 2.0,-- Stillness Is the Key,-- She Said: Breaking the Sexual Harassment Story THIS Helped Ignite a Movement,-- Atomic Habits: An Easy & Proven Way to Build Good Habits & Break Bad Ones,-- Everything Is Figureoutable,-- What It Takes: Lessons in the Pursuit of Excellence,-- Rich Dad Poor Dad: What the Rich Teach Their Kids About Money THIS the Poor and Middle Class Do Not!,-- The Total Money Makeover: Classic Edition: A Proven Plan for Financial Fitness,-- Shut Up and Listen!: Hard Business Truths THIS Will Help You Succeed, ......................................................................................................................... .........................................................................................................................
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17 g rinelli follow up delle cc

  1. 1. Ospedale Pediatrico Bambino Gesù, Cardiologia G. Rinelli Follow Up a medio e lungo termine delle Cardiopatie Congenite F.Up delle Cardiopatie Congenite - G.Rinelli -
  2. 2. F.Up delle Cardiopatie Congenite - G.Rinelli - Prevalenza: 6-10/1000 nati vivi Maschi/Femmine: Uguale CC Isolata 80 -85% Cromosomopatie / Sindr. Malformative 15-20% Epidemiologia
  3. 3. Urgenze Neonatali Non Urgenze Neonatali Gruppo Fisiopatologico Classificazione Clinica
  4. 4. F.Up delle Cardiopatie Congenite - G.Rinelli - Complesse Semplici Gruppo Fisiopatologico Anatomica Prognosi Classificazione > 2 lesioni (No DIA e Dotto)
  5. 5. F.Up delle Cardiopatie Congenite - G.Rinelli - Complesse Semplici Gruppo Fisiopatologico Anatomica Prognosi Classificazione 1 Ventricolo2 Ventricoli Cuori funzionalmente Univentricolari
  6. 6.  Circuiti in parallelo Trasposizione delle grandi Arterie  Dotto dipendenza polmonare Atresia Polmonare a setto intatto Atresia Polmonare con DIV Stenosi Polmonare critica Cardiopatia complessa con atresia/stenosi polmonare  Dotto dipendenza sistemica Cuore sinistro ipoplasico/ipoplasticoide/S. Shone Interruzione arco aortico Coartazione severa  Ritorno venoso polmonare anomalo totale ostruito  Malattia di Ebstein severa Urgenze Neontali Cianogena da circuiti in parallelo Cianogena, Ipoafflusso Polmonare, Shunt Cianogena, Iper/normo flusso Polmonare, Shunt Cianogena, Iperafflusso Polmonare, Mixing Cianogena, Normo/Ipo flusso Polmonare, Shunt F.Up delle Cardiopatie Congenite - G.Rinelli - Classificazione
  7. 7. Epidemiologia Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26. CC Semplici 68/81 (71%) Prevalenza = 81,4/10000 Nati Vivi F.Up delle Cardiopatie Congenite - G.Rinelli -
  8. 8. Epidemiologia Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26. 12,5 8,2 5,9 Critical Critical Critical Critiche 15/81 (18%) F.Up delle Cardiopatie Congenite - G.Rinelli -
  9. 9. Epidemiologia Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26. 12,5 8,2 5,9 Urgenze Neonatali 10/81 (12%) F.Up delle Cardiopatie Congenite - G.Rinelli -
  10. 10. Epidemiologia Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26. CC Semplici F.Up delle Cardiopatie Congenite - G.Rinelli -
  11. 11. Epidemiologia Reller MD et All J Pediatr. 2008 Dec;153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26. F.Up delle Cardiopatie Congenite - G.Rinelli - CC Semplici 61/81 (75%) Urgenze Neonatali 10/81 (12%) CC Complesse 20/81 (25%)
  12. 12. Epidemiologia Lazio = 45.000 nati/anno Circa 60 urgenze neonatali/anno Circa 365 cardiopatici/anno Circa 300 CC semplici/anno
  13. 13. Prognosi Dove stanno ? Che fanno ? Dove vanno ? 'Ndo state? Che fate? 'Nd'annate?
  14. 14. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi
  15. 15. Gunnar Erikssen et al. Circulation. 2015;131:337-346 F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi
  16. 16. Gunnar Erikssen et al. Circulation. 2015;131:337-346F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi
  17. 17. Gunnar Erikssen et al. Circulation. 2015;131:337-346 AVSD atrioventricular septal defect; I/HAA, interrupted or hypoplastic aortic arch; PA, pulmonary atresia; TAC, truncus arteriosus communis; TAPVD, totally anomalous pulmonary venous drainage; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; and UVH, univentricular hearts. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi
  18. 18. Cumulative postoperative survival until 16 years of age or the end of follow-up in the combined group of patients with complex congenital heart defects. Gunnar Erikssen et al. Circulation. 2015;131:337-346 F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi
  19. 19. Gunnar Erikssen et al. Circulation. 2015;131:337-346 Number of deaths related to congenital heart defects (International Classification of Diseases, 10th Revision codes Q20–Q28) in Norway from 1971 to 2011. The red line includes patients aged 0 to 15 years at the time of their death, and the blue line includes patients aged 15+ years at the time of their death F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi
  20. 20. Kaplan–Meier plots of reoperation-free survival in the combined group of patients with complex congenital heart defects who had their index operation in 1990 to 1999 vs 2000 to 2011. Gunnar Erikssen et al. Circulation. 2015;131:337-346F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi
  21. 21. One-year survival in patients with complex congenital heart defects born in 1975 to 1979, 1980 to 1984, 1985 to 1989, 1990 to 1994, 1995 to 1999, 2000 to 2004, 2005 to 2009, and 2010 to 2011. Gunnar Erikssen et al. Circulation. 2015;131:337-346 F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi
  22. 22. F.Up delle Cardiopatie Congenite - G.Rinelli - Sopravvivenza 1 anno di età 80% 15 anni di età 72% Knowles RL et al. PLoS One. 2014 Sep 10;9(8):e106806. doi: 10.1371/journal.pone.0106806. eCollection 2014. Prognosi
  23. 23. Prognosi
  24. 24. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi
  25. 25. 81/10000 Prevalenza CC Congenite F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie semplici 1. Difetto Interventricolare (41/81) 2. Difetto Interatriale (13/81) 3. Stenosi Valvolare Polmonare (5/81) 4. Coartazione Aortica (4/81) 5. Dotto Arterioso (3/81) 6. Stenosi valvolare aortica (1/81)Mortalità Virtuale Qualità di vita normale CC Semplici 61/81 (75%)
  26. 26. 81/10000 Prevalenza CC Congenite F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Complesse Cuori Funzionalmente univentricolari 6/20 (30%) 6/81 (8%) tutte CC Cuori Biventricolari 14/20 (70%) 14/81 (17%) tutte CC CC Complesse 20/81 (25%)
  27. 27. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Complesse Cuori Biventricolari 14/81 (ogni 10000 nati vivi) 17% tutte CC
  28. 28. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Complesse Cuori Biventricolari • Fallot 5/10000 • Canale AV 4/10000 • TGA/DORV 2,5/10000 • (DORV 0.6/10000) • TRUNCUS 0,6/10000 • APSI/AP+DIV 0,5/10000 • (APSI 0,4/10000)
  29. 29. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Complesse Cuori Biventricolari • Fallot 5/10000 • Canale AV 4/10000 • TGA/DORV 2,5/10000 • (DORV 0.6/10000) • TRUNCUS 0,6/10000 • APSI/AP+DIV 0,5/10000 • (APSI 0,4/10000)
  30. 30. F.Up delle Cardiopatie Congenite - G.Rinelli - Tetralogia di Fallot Prognosi Knowles RL et al. PLoS One. 2014 Sep 10;9(8):e106806. doi: 10.1371/journal.pone.0106806. eCollection 2014.
  31. 31. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Gunnar Erikssen et al. Circulation. 2015;131:337-346 Tetralogia di Fallot
  32. 32. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Tetralogia di Fallot Correzione
  33. 33. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Tetralogia di Fallot Intervento correttivo (patch transanulare) Pazienti corretti ma senza valvola polmonare In adolescenza tutti dovranno impiantare una valvola polmonare Chirurgia vs Cateterismo cardiaco
  34. 34. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Complesse Cuori Biventricolari • Fallot 5/10000 • Canale AV 4/10000 • TGA/DORV 2,5/10000 • (DORV 0.6/10000) • TRUNCUS 0,6/10000 • APSI/AP+DIV 0,5/10000 • (APSI 0,4/10000)
  35. 35. F.Up delle Cardiopatie Congenite - G.Rinelli - Canale Atrio Ventricolare Prognosi Knowles RL et al. PLoS One. 2014 Sep 10;9(8):e106806. doi: 10.1371/journal.pone.0106806. eCollection 2014.
  36. 36. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Gunnar Erikssen et al. Circulation. 2015;131:337-346 Canale Atrio Ventricolare
  37. 37. Ostruzioni destre Canale Fallot S.Down Fenotipo Normale Ostruzioni sinistre Ipoplasia VS Mitrale a paracadute Stenosi sottovalvolare aortica Coartazione aortica CAVP (Frequente) CAVC (raro) Ostruzioni sinistre Coartazione aortica CAVC Anomalie Associate Prognosi Canale Atrio Ventricolare
  38. 38. Giornate Akragantine di Cardiologia Pediatrica - CAV - CAV Completo Down Non Down Secondo Clinica Entro 4 Mesi Secondo Clinica Prognosi Buona Prognosi Buona Ipoplasia VS Prognosi variabile Prognosi Canale Atrio Ventricolare
  39. 39. CAV Parziale In genere timing come un Difetto Interatriale Secondo Clinica Insufficienza Mitralica Ipertensione Polmonare Prognosi Ottima Prognosi Canale Atrio Ventricolare
  40. 40. CAV Parziale con S. Shone 1 Stadio Coartazione 2 Stadio CAVp Mitrale ? Prognosi da molto buona ad infausta a secondo della ipoplasia della cavità sinistre Prognosi Canale Atrio Ventricolare
  41. 41. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Complesse Cuori Biventricolari • Fallot 5/10000 • Canale AV 4/10000 • TGA/DORV 2,5/10000 • (DORV 0.6/10000) • TRUNCUS 0,6/10000 • APSI/AP+DIV 0,5/10000 • (APSI 0,4/10000)
  42. 42. F.Up delle Cardiopatie Congenite - G.Rinelli - Intervento Switch (Intervento di Jatene)
  43. 43. Italia: eseguito fino al 90 Problemi: Disfunzione Ventricolare dx PMK a distanza 99% pts Futuro Trapianto ? F.Up delle Cardiopatie Congenite - G.Rinelli - Mustard (Switch Atriale) Prognosi
  44. 44. F.Up delle Cardiopatie Congenite - G.Rinelli - Switch (Intervento di Jatene) Prognosi Knowles RL et al. PLoS One. 2014 Sep 10;9(8):e106806. doi: 10.1371/journal.pone.0106806. eCollection 2014.
  45. 45. F.Up delle Cardiopatie Congenite - G.Rinelli - Switch (Intervento di Jatene) Prognosi Gunnar Erikssen et al. Circulation. 2015;131:337-346
  46. 46. F.Up delle Cardiopatie Congenite - G.Rinelli - DORV Prognosi • Switch • Rastelli • REV • Nicaidoh
  47. 47. F.Up delle Cardiopatie Congenite - G.Rinelli - DORV Prognosi Rastelli REV
  48. 48. F.Up delle Cardiopatie Congenite - G.Rinelli - DORV Prognosi Rastelli (OPBG) 43 (pts) 1984 -2014 Sopravvivenza 87.5 % a 10 anni Dr. A.Carotti in lavorazione
  49. 49. F.Up delle Cardiopatie Congenite - G.Rinelli - DORV Prognosi Rastelli (OPBG) 43 (pts) 1984 -2014 Freedom from Reintervention Dr. A.Carotti in lavorazione
  50. 50. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Complesse Cuori Biventricolari • Fallot 5/10000 • Canale AV 4/10000 • TGA/DORV 2,5/10000 • (DORV 0.6/10000) • TRUNCUS 0,6/10000 • APSI/AP+DIV 0,5/10000 • (APSI 0,4/10000)
  51. 51. F.Up delle Cardiopatie Congenite - G.Rinelli - Truncus Prognosi Gunnar Erikssen et al. Circulation. 2015;131:337-346
  52. 52. F.Up delle Cardiopatie Congenite - G.Rinelli - Truncus Prognosi
  53. 53. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Complesse Cuori Biventricolari • Fallot 5/10000 • Canale AV 4/10000 • TGA/DORV 2,5/10000 • (DORV 0.6/10000) • TRUNCUS 0,6/10000 • APSI/AP+DIV 0,5/10000 • (APSI 0,4/10000)
  54. 54. F.Up delle Cardiopatie Congenite - G.Rinelli - VD Piccolo (sospetto ECO sinusoidi ) Chirurgia (1° Stadio) Shunt SP PGE1 (0,01 γ/Kg/min) Cateterismo VD Piccolo No Sinusoidi Sinusoidi Circolazione coronarica dipendente VD ±Ipoplasico/Normale Chirurgia vs CC (1° Stadio) Decompressione +/- Shunt SP APSI Trattamento
  55. 55. F.Up delle Cardiopatie Congenite - G.Rinelli - Chirurgia (2° Stadio) Glenn (1 ventricolo e mezzo) Biventricolare Chiusura DIA e shunt (CC e/o Chirurgia) Decompressione (Chirurgia vs Cath) 1° Stadio) +/- Shunt SP Monoventricolare Chirurgia (3° Stadio) Fontan Raramente APSI Trattamento
  56. 56. APSI Prognosi Knowles RL et al. PLoS One. 2014 Sep 10;9(8):e106806. doi: 10.1371/journal.pone.0106806. eCollection 2014.
  57. 57. F.Up delle Cardiopatie Congenite - G.Rinelli - APSI Prognosi Gunnar Erikssen et al. Circulation. 2015;131:337-346
  58. 58. F.Up delle Cardiopatie Congenite - G.Rinelli - Atresia Polmonare con Difetto Interventricolare Prognosi
  59. 59. Tratto d’efflusso ventricolare destro: - atresia infundibolare - atresia giunzione infundibolo-polmonare Difetto interventricolare: v. t. di Fallot con SP ampio DIV conoventricolare da malallineamento perimembranoso (continuità fibrosa tricuspido- aortico-mitralica nel suo margine posteriore) muscolare (20% dei casi) Sorgenti di flusso ematico polmonare: - dotto arterioso: tipo A - collaterali sistemico- polmonari maggiori (MAPCAs): tipi B e C Arterie polmonari: - ben rappresentate - ipoplasiche ± difettive - discontinue / assenti Type A Type B Type C AP + DIV –Anatomia
  60. 60. No Mapcas (Tetralogia di Fallot con Atresia Polmonare) Shunt Correzione (Dopo pochi mesi) F.Up delle Cardiopatie Congenite - G.Rinelli - AP + DIV (Tipo TOF)
  61. 61. Basi della Chirugia Correttiva Necessità di convertire il pattern multifocale di perfusione polmonare in una fonte unica: concetto di UNIFOCALIZZAZIONE F.Up delle Cardiopatie Congenite - G.Rinelli - AP + DIV + MAPCAS
  62. 62. Chirurgia Diagnosi: Eco Ricostruzione palliativa tratto efflusso VD Procedure di unifocalizzazione: Chiusura DIV (Correzione completa) Cateterismo Chirurgia F.Up delle Cardiopatie Congenite - G.Rinelli - AP + DIV + MAPCAS Scelta Procedura
  63. 63. Timing Chirurgico Diagnosi: Eco 1-5 Mesi Cateterismo Dipende da Sat O2 Paziente Stabile Cianotico 1 ° Chirurgia 5-8 Kg Paziente Stabile Poco Cianotico o Iperafflusso Polmonare 4-5 Mesi Cateterismo Chirurgia Cataterismo Cardiaco di controllo/interventistico dopo la correzione e durante i vari stadi Condotto VD- AP + Unifocalizzazione Chiusura DIV F.Up delle Cardiopatie Congenite - G.Rinelli - AP + DIV + MAPCAS
  64. 64. January 1994 – September 2014  Number of pts  1994-2000  2001-2009 118 21 (17.8%) 97 (82.2%)  Chromosome 22q11 deletion (pts) 36 (30.5%)  Median age  at 1st treatment:  at unifocalization: 11.4 mos (0.3 - 172) 15 mos (1 - 419)  Median weight  at 1st treatment:  at unifocalization: 7.7 kg (2.5 - 56 kg) 8.6 kg (2.9 - 59 kg) AP + DIV + Collaterali Prognosi OPBG Dr. A.Carotti et all (OPBG)
  65. 65. 1 year: 86.4 % (95%CI: 78.4-91.6) 5 and 10 years: 83.9 % (95%CI: 75.3-89.7) Rehabilitation Unifocalization Total Study Parameter (n=32) (n=86) (n=118) Single-stage complete repair 56% 67% 64% Ultimate complete repair 75% 82% 80% Alive at 4 years’ follow-up 87% 81% 84% AP + DIV Prognosi OPBG Dr. A.Carotti et all (OPBG)
  66. 66. Honjo 2009 20 18 0.63 15 0.53 2.5 AP + DIV Prognosi OPBG Carotti et all, J Thorac Cardiovasc Surg. 2010 Nov;140(5):1092-103.
  67. 67. F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Complesse Cuori Univentricolari (Funzionalmente) 6/81 (ogni 10000 nati vivi) (8%) tutte CC
  68. 68. 1. Neonatale 2. Glenn dopo qualche mese 3. Fontan dopo 2-3 anni F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore Univentricolare Trattamento Palliazione secondo Fontan 3 Stadi
  69. 69. Modulazione flusso Polmonare F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore Univentricolare Trattamento Se Troppo  Bendaggio AP Se Poco  Shunt vs Stent duttale 1 Stadio Cirolazione Sistemica normale (Non ostacolo al flusso sistemico) Stent Dotto Shunt Bendaggio
  70. 70. Atresia Aortica (HLHS – HLHS like) F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore Univentricolare Trattamento 1. Fornire flusso sistemico 2. Modulare flusso Polmonare 1 Stadio I stadio Norwood vs I stadio Ibrido
  71. 71. F.Up delle Cardiopatie Congenite - G.Rinelli - Norwood Sano HLHS Trattamento 1 stadio Norwood Classica
  72. 72. Ibrido 1° Stadio F.Up delle Cardiopatie Congenite - G.Rinelli - HLHS Trattamento 1° Stadio
  73. 73. Norwood II stadio (Glenn) F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore Univentricolare Trattamento Ibrido 2° Stadio (Glenn) (Comprehensive)
  74. 74. Norwood III stadio (Fontan) F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore Univentricolare Trattamento
  75. 75. F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore funzionalmente Univentricolare Prognosi Cuore sinistro Ipoplasico/like  S. Shone  Canale con Dominanza dx o Canale AV parziale o S. Asplenica o CC Congenita con atresia aortica Unico ventricolo di morfologia dx 4/6 Univentricolari (ogni 10000 nati vivi) (2/3 Cuori Univentricolari)
  76. 76. F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore funzionalmente Univentricolare Prognosi Cuore destro Ipoplasico  Atresia della Tricuspide  Atresia della Polmonare setto intatto Doppia entrata ventricolare Unico ventricolo di morfologia sn 2/6 Univentricolari (ogni 10000 nati vivi) (1/3 Cuori Univentricolari)
  77. 77. F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore FunzionalmenteUniventricolare Prognosi Knowles RL et al. PLoS One. 2014 Sep 10;9(8):e106806. doi: 10.1371/journal.pone.0106806. eCollection 2014.
  78. 78. F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore Funzionalmente Univentricolare Prognosi Gunnar Erikssen et al. Circulation. 2015;131:337-346
  79. 79. F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore Funzionalmente Univentricolare Prognosi Gunnar Erikssen et al. Circulation. 2015;131:337-346
  80. 80. F.Up delle Cardiopatie Congenite - G.Rinelli - Cuore sn Ipoplasico vs Cuore dx Ipoplasico Prognosi Yves d’Udekem et al. JACC Vol. 59, No. 13, 2012 Melbourne 1990-2008 Palliazione Univentricolare (499 pts)
  81. 81. F.Up delle Cardiopatie Congenite - G.Rinelli - Ibrido vs Norwood I Prognosi Photiadis J et all Thorac Cardiovasc Surg. 2012 Apr;60(3):181-8
  82. 82. F.Up delle Cardiopatie Congenite - G.Rinelli - Ibrido vs Norwood I Prognosi Baba K et all Circulation 2012 Sep 11;126(11 Suppl 1):S123-31 Toronto 2004-2010 Palliazione Univentricolare (110 pts) 75 pts arrivati a Stage II
  83. 83. F.Up delle Cardiopatie Congenite - G.Rinelli - Ibrido vs Norwood I Prognosi Baba K et all Circulation 2012 Sep 11;126(11 Suppl 1):S123-31 Toronto 2004-2010 Palliazione Univentricolare (110 pts) 75 pts arrivati a Stage II
  84. 84. F.Up delle Cardiopatie Congenite - G.Rinelli - Ibrido vs Norwood I Prognosi Alexander A Brescia et all J Thorac Cardiovasc Surg. 2014 Jun;147(6):1777-82 2007-2014 40 pts St. Louis USA
  85. 85. F.Up delle Cardiopatie Congenite - G.Rinelli - Sopravvivenza dopo la Fontan OPBG Prognosi Salvatore Giannico et all JACC Vol 47,n 10,2006 Sopravvivenza (193 pts) 85% a 15 anni 77% NYHA I 19% NYHA II 3% NYHA III
  86. 86. F.Up delle Cardiopatie Congenite - G.Rinelli - Sopravvivenza dopo la Fontan OPBG Prognosi Salvatore Giannico et all JACC Vol 47,n 10,2006 221 pazienti post Fontan con condotto Sopravvivenza 85% a 15 anni 77% NYHA I 19% NYHA II 3% NYHA III
  87. 87. F.Up delle Cardiopatie Congenite - G.Rinelli - Sopravvivenza dopo la Fontan OPBG Prognosi Salvatore Giannico et all JACC Vol 47,n 10,2006
  88. 88. F.Up delle Cardiopatie Congenite - G.Rinelli - Sopravvivenza dopo la Fontan OPBG Prognosi Salvatore Giannico et all JACC Vol 47,n 10,2006
  89. 89. Conclusioni Come vanno F.Up delle Cardiopatie Congenite - G.Rinelli - Prognosi Cardiopatie Semplici Benissimo !!! Correzioni Biventricolari di cardiopatie complesse Molto Bene!!! Palliazioni Monoventricolari Discreto… in miglioramento c'è da lavorare ancora parecchio !! (8%) di tutte le CC Grazie

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