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16 gestione neonatologica delle cardiopatie congenite diagnosticate in utero

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16 gestione neonatologica delle cardiopatie congenite diagnosticate in utero

  1. 1. Gestione  neonatologica  delle  cardiopatie   congenite  diagnosticate  in  utero Aggiornamenti  di  Ecocardiogra4ia  Fetale   II  edizione Sapienza  Università  di  Roma                  18  -­‐  19  Aprile  2015 Gerardo  Piacentini   Unità  di  Neonatologia  e  Terapia  Intensiva  Neonatale   Ambulatorio  di  Cardiologia  Fetale  e  Pediatrica   Ospedale  “San  Giovanni  Calibita”  -­‐  Fatebenefratelli  Isola  Tiberina,  Roma  
  2. 2. Cardiopatie  congenite Pediatr  Cardiol  2003;24:195   BMJ  2009;338:a3037   Seminars  in  fetal  &  neonatal  medicine  2013;18:302 - Incidenza:  8-­‐10‰   - 6-­‐10%  cause  di  morte  in  età  pediatrica  (20-­‐40%  per  malformazioni  congenite)   - 10-­‐20%  di  tutti  i  neonati  con  CHD  (1-­‐2‰):  cardiopatia  critica Dotto-­‐dipendenza   circolo  sistemico Dotto-­‐dipendenza   circolo  polmonare Trasposizione   delle  grandi  arterie RVPAT  ostruito Aritmie   Cardiomiopatie   Tumori  cardiaci
  3. 3. Congenital  Heart  Disease  in  the  Neonatal  Period J  Pediatr  1964;65:721
  4. 4. Congenital  Heart  Disease  in  the  Neonatal  Period J  Pediatr  1964;65:721
  5. 5. Congenital  Heart  Disease  in  the  Neonatal  Period Rowe  and  Mehrizi  1968   Lancet  1975:1:140 1938  -­‐  Legatura  del  dotto  arterioso  (Gross)   1944  -­‐  Shunt  sistemico  polmonare  (Blalock-­‐Taussig)   1948  -­‐  Atriosettostomia  di  Blalock-­‐Hanlon   1952  -­‐  Bendaggio  polmonare  (Muller  e  Dammann)   1958  -­‐  Anastomosi  cavopolmonare  (Glenn)   1966  -­‐  Atriosettostomia  di  Rashkind   1968  -­‐  Intervento  di  Fontan   1975  -­‐  Switch  arterioso  (Jatene)   1975  -­‐  Introduzione  PGE1  (Elliott)   1984  -­‐  Trapianto  cardiaco  neonatale  (Bailey)
  6. 6. Cardiopatie  congenite  neonatali Prostaglandine Ecocardiogra4ia Diagnosi  prenatale Cardiochirurgia 20%   anni  ‘50 90  %   oggi Circulation  2001;103:2376   JACC  2010;56:1149
  7. 7. Prostaglandine  E1 Indicazioni   Azione           ffffff1hhffffff                                                                                                             Dosaggio   Effetto  ffffff1hhffffff                       ff   Somministrazione   - Aprire  o  mantenere  aperto  il  dotto  arterioso   - Vasodilatazione  per  azione  diretta  sulla  muscolatura  liscia   del    dotto  arterioso   - 0.01-­‐0.03  mcg/kg/min  per  mantenere  il  dotto  aperto   - 4ino  a  0.05-­‐0.1  mcg/kg/min   - Aumento  della  pO2,  incremento  pressione  arteriosa   - Miglioramento  della  perfusione       _____________________________________   - Infondere  in  una  via  separata,  possibilmente  vaso  centrale   - Compatibile  con  SG5,  SG10,  SF   - Emivita  breve  (infusione  continua)   - Dose  più  bassa  possibile  
  8. 8. Prostaglandine  E1  -­‐  Effetti  collaterali Respiratori   Cardiovascolari   SNC                       Endocrinologici   Gastrointestinali   Ematologici                                                                                                                                     - Depressione  respiratoria  o  apnea  (12%)   - Usualmente  nelle  prime  ore  dall’inizio  dell’infusione   - Incidenza  aumentata  se  peso  <  2  kg   - Dose  dipendente   - Ipotensione,  bradicardia,  tachicardia   - Irritabilità,  convulsioni,  febbre   - Ipocalcemia,  ipoglicemia   - Diarrea   - Inibizione  aggregazione  piastrina  
  9. 9. Gravidanza  con  feto  portatore  di  malformazione   Team  multidisciplinare Cardiologo) pediatra) Cardiologo) perinatale) Neonatologo) Ostetrica) Ginecologo) Cardiochirurgo) Anestesista)SP) Anestesista) TIC) Psicologo) Chirurgo) neonatale)
  10. 10. Organizzazione  sala  partoSala$parto$ Neonatologo$ Neonatologo$ Cardiologo$perinatale$ Ginecologo/ ostetrico$ Anestesista$ Infermieri$ Ostetriche$ STEN$ Isola  neonatale Box  parto Sale  operatorie
  11. 11. Fetal  Cardiovascular  Disease  Severity  Grading  Scale Rychik  J.  Prenatal    Practice  Care  Model  and  Delivery    of  the  Fetus  with  Cardiovascular  Disease.  In  :  Fetal  cardiovascular  imaging.  Elsevier.  2012  
  12. 12. Delivery  Classi4ication  Scale  for  Fetal  Cardiovascular  Disease Rychik  J.  Prenatal    Practice  Care  Model  and  Delivery    of  the  Fetus  with  Cardiovascular  Disease.  In  :  Fetal  cardiovascular  imaging.  Elsevier.  2012  
  13. 13. Codi4icazione  del  rischio  alla  nascita CODICE  BLU   CODICE  ROSSO   CODICE  GIALLO   CODICE  VERDE                                                                    
  14. 14. CODICE  BLU   CODICE  ROSSO                                                                                       - se  già  nato  (trasferimento  urgente  in  TIC)   - se  nascita  da  programmare - TGA  con  setto  interventricolare  intatto  con  forame  ovale  restrittivo   - Cuore  sinistro  ipoplasico  con  forame  ovale  restrittivo Considerare  Rushkind  in  sala  parto  o   intervento  chirurgico  urgentissimo! - Ritorno  venoso  polmonare  anomalo  totale  ostruito Urgenza  chirurgica! Codi4icazione  del  rischio  alla  nascita
  15. 15. CODICE  ROSSO - trasferimento  urgente  in  TIC  se  già  nato   - se  nascita  da  programmare - TGA  con  setto  interventricolare  intatto   - Cardiopatie  con  dotto-­‐dipendenza  del  circolo  sistemico   - Cuore  sinistro  ipoplasico  senza  forame  ovale  restrittivo   - Stenosi  aortica  critica   - Interruzione  dell’arco  artico   - Coartazione  aortica  dotto-­‐dipendente   Classi4icazione  del  rischio  alla  nascita
  16. 16. CODICE  ROSSO                                                                                          - trasferimento  urgente  in  TIC  se  già  nato   - se  nascita  da  programmare - Cardiopatie  con  dotto-­‐dipendenza  del  circolo  polmonare   - Atresia  polmonare  a  setto  interventricolare  intatto   - Atresia  polmonare  con  difetto  interventricolare   - Cardiopatia  complessa  con  atresia  polmonare  o  stenosi  polmonare  critica   - Ebstein  severo Classi4icazione  del  rischio  alla  nascita
  17. 17. CODICE  GIALLO                                                - trasferimento  in  TIC/TIN/TIP  con  posto  letto  di  appoggio - Cardiopatie  con  ipoaf4lusso  polmonare  (cianogene,  Qp/Qs  <  1)   - Tetralogia  di  Fallot   - Cardiopatia  complessa  con  stenosi  polmonare  non  severa   - Cardiopatia  con  iperaf4lusso  polmonare  (Qp/Qs  >  1)   - Truncus  arteriosus   - Cardiopatia  complessa  senza  ostruzione  polmonare  o  aortica   - Cuore  univentricolare   - Ventricolo  destro  a  doppia  uscita   - Canale  atrioventricolare  sbilanciato Flusso  polmonare  dif4icile   da  predire  a  priori! Classi4icazione  del  rischio  alla  nascita
  18. 18. CODICE  VERDE                                                                          - non  necessario  trasferimento,  controllo  ambulatoriale - Cardiopatie  con  iperaf4lusso  polmonare   - Difetto  interventricolare   - Canale  atrioventricolare  completo  o  parziale   - Valvulopatie   - Stenosi  aortica  non  critica   - Stenosi  polmonare  non  critica   - Displasia  mitralica   - Displasia  tricuspide Classi4icazione  del  rischio  alla  nascita
  19. 19. Cardiopatie  critiche  in  epoca  neonatale - Sindrome  del  cuore  sinistro  ipoplasico   - Coartazione  aortica   - Interruzione  dell’arco  aortico   - Stenosi  aortica  critica Dotto-­‐dipendenza  circolo  sistemico Dotto-­‐dipendenza  circolo  polmonare - Atresia  polmonare  a  setto  intatto   - Stenosi  polmonare  critica   - Cardiopatia  complessa  con  AP/SP
  20. 20. Cardiopatie  critiche  in  epoca  neonatale Trasposizione  delle  grandi  arterie Ritorno  polmonare  anomalo  totale
  21. 21. Neonato  con  cardiopatia  congenita 1. ABC   – eventuale  somministrazione  di  ossigeno     – posizionamento  di  catetere  venoso  ombelicale   – eventuale  inizio  infusione  di  PGE1   2. Emogasanalisi   3. Correzione  acidosi,  ipoglicemia,  diselettrolitemia   4. Valutazione  clinica  completa  (fenotipo)   5. Pressione  arteriosa  ai  4  arti   6. Saturazione  pre-­‐duttale  e  post-­‐duttale   7. RX  torace   8. Ecocardiogra4ia Valutazione  e  management  iniziale
  22. 22. Cardiopatie  con  dotto-­‐dipendenza  circolo  sistemico
  23. 23. Circolazione  fetale Dotto  arterioso Forame  ovale Dotto  venoso
  24. 24. Circolazione  transizionale Fisiologia  postnatale Alla  Nascita…   ‣ Rimozione  circolazione  placentare   ‣ Esposizione  del  letto  vascolare  polmonare  ad  alte   concentrazioni  di  O2  (vasodilatazione  polmonare)   ‣ Incremento  di  20  volte  del  4lusso  polmonare   ‣ Aumento  della  portata  cardiaca  per  compensare  le   aumentate  richieste  metaboliche O2
  25. 25. Coartazione  aortica  dotto-­‐dipendente
  26. 26. Coartazione  aortica
  27. 27. Coartazione  aortica
  28. 28. Coartazione  aortica  severa 1. Posizionamento  catetere  venoso  ombelicale   2. Inizio  infusione  di  PGE1  (Prostin  VR  0,01-­‐0,03  mcg/kg/min)   3. Trattamento  dell’eventuale  acidosi  (1-­‐2  mEq/kg,  bolo  lento)   4. Supportare  il  circolo:     • volume  (5-­‐10  ml/kg  SF  0.9%)   • inotropi:  iniziare  con  dopamina  3-­‐6  mcg/kg/min  e/o  adrenalina   0.025-­‐0.05    mcg/kg/min)   5. Ventilazione  adeguata:  PaCO2  35–45  mmHg,  pH  7.35–7.40   • SpO2    pre-­‐duttale  >90%   6. Saturazione  pre-­‐duttale  e  post-­‐duttale Management  neonatale
  29. 29. Sospetta  coartazione  aortica
  30. 30. Sospetta  coartazione  aortica 1. Osservazione  in  Terapia  Intensiva  Neonatale   2. Monitoraggio  della  pressione  arteriosa   3. Monitoraggio  SpO2  pre  e  post-­‐duttale   4. Controllo  diuresi   5. Elettrocardiogramma   6. Ecocardiogra4ia Management  neonatale
  31. 31. Sospetta  coartazione  aortica
  32. 32. Sindrome  del  cuore  sinistro  ipoplasico
  33. 33. Sindrome  del  cuore  sinistro  ipoplasico
  34. 34. Sindrome  del  cuore  sinistro  ipoplasico  
  35. 35. Sindrome  del  cuore  sinistro  ipoplasico
  36. 36. Sindrome  del  cuore  sinistro  ipoplasico
  37. 37. Sindrome  del  cuore  sinistro  ipoplasico   Management  neonatale 1. Posizionamento  catetere  venoso  ombelicale   2. Inizio  infusione  di  PGE1     3. Trattamento  dell’eventuale  acidosi     4. Supportare  il  circolo   • Evitare  eccessiva  somministrazione  di  O2     • Preferire  respirazione  in  aria  ambiente   • Evitare  dolore,  stress  e  raffreddamento   • Respiro  spontaneo  se  possibile   • Attenta  somministrazione  di  vasodilatatori  sistemici  (Milrinone)
  38. 38. 1. Apnea  o  grave  distress  respiratorio   2. Acidosi  metabolica  importante   3. Iperaf4lusso  polmonare   4. Disfunzione  miocardica  severa Sindrome  del  cuore  sinistro  ipoplasico   Indicazioni  alla  ventilazione  meccanica 1. PaCO2  35–45  mm  Hg   2. pH  7.35–7.40   3. PaO2  30–45  mm  Hg   4. SaO2  70–85%   5. Inizialmente  FiO2  0.21,  PEEP  4–5  cmH2O Obiettivi  alla  ventilazione  meccanica
  39. 39. TGA  a  setto  intatto
  40. 40. Trasposizione  delle  grandi  arterie
  41. 41. Trasposizione  delle  grandi  arterie
  42. 42. TGA  a  setto  intatto 1. Posizionamento  catetere  venoso  ombelicale   2. Inizio  infusione  di  PGE1  (Prostin  VR  0,01-­‐0,03  mcg/kg/min)   3. Trattamento  dell’eventuale  acidosi  (1-­‐2  mEq/kg,  bolo  lento)   4. Supportare  il  circolo:     • volume  (5-­‐10  ml/kg  SF  0.9%,  Ht  >40%)   • inotropi:  iniziare  con  dopamina  3-­‐6  mcg/kg/min  e/o  adrenalina   0.025-­‐0.05    mcg/kg/min)   5. Ventilazione  adeguata:  PaCO2  40-­‐45  mmHg,  pH  7.35–7.40   • SpO2    pre-­‐duttale  >  80-­‐85%   6. Saturazione  pre  e  post  duttale Management  neonatale
  43. 43. Rashkind  -­‐  Atriosettostomia - Ampliare  la  comunicazione  interatriale   - Aumentare  mixing
  44. 44. Atresia  polmonare  a  setto  integro
  45. 45. Atresia  polmonare  a  setto  integro
  46. 46. Atresia  polmonare  a  setto  integro
  47. 47. Atresia  polmonare  a  setto  integro Management  neonatale 1. Posizionamento  catetere  venoso  ombelicale   2. Inizio  infusione  di  PGE1  (Prostin  VR  0,01-­‐0,03  mcg/kg/min)   3. Trattamento  dell’eventuale  acidosi  (1-­‐2  mEq/kg,  bolo  lento)   4. Supportare  il  circolo:     • volume  (5-­‐10  ml/kg  SF  0.9%,  Ht  >40%)   • inotropi:  iniziare  con  dopamina  3-­‐6  mcg/kg/min  e/o  adrenalina   0.025-­‐0.05    mcg/kg/min) Ischemia!
  48. 48. Ritorno  venoso  polmonare  anomalo  totale
  49. 49. 1. Intubazione  e  ventilazione  meccanica  adeguata     • PaCO2  40–50  mmHg,  pH  7.35–7.40,     • SpO2    pre-­‐duttale  o  post-­‐duttale  >  85%   2. Supportare  il  circolo:     • volume  (5-­‐10  ml/kg  SF  0.9%,  Ht  >40%)   • inotropi:  iniziare  con  dopamina  3-­‐6  mcg/kg/min  e/o  adrenalina   0.025-­‐0.05    mcg/kg/min)   3. Trasferire  il  prima  possibile.  E’  una  urgenza  cardiochirurgica Urgenza  chirurgica! Management  neonatale Ritorno  venoso  polmonare  anomalo  totale
  50. 50. 50

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