Tetralogia di Fallot
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Tetralogia di Fallot Presentation Transcript

  • 1. Tetralogia di Fallot Sequele post correzione chirurgicaTrattamento medico e trans-catetere G Agnoletti Citta’ della Salute TORINO gabriella.agnoletti@oirmsantanna.piemonte.it Cardiopatie congenite: Dalla diagnosi prenatale all’età adulta
  • 2. •  4-11% of children born with CHD•  most common cyanotic CHD•  “easy” disease…•  surgical correction•  large number of adults with repaired Fallot (10000 in Italy)
  • 3. Tetralogy of FallotA single anomaly ? (when regular….)
  • 4. Type of FallotRegular: Normal ventricles and A-V valves One VSD No coronary artery crossing the annulus No PA branch stenosisFavorable: Size and harmony of PA branches (Nakata)
  • 5. Irregular Fallot•  Obstruction at the origin of LPA•  Abnormal LAD crossing the anulus•  Multiple VSD•  Old Fallot with small LV, myocardial hypertrophy or dysfunction•  A-V canal with PS
  • 6. Palliative / complete repair Complete repairPalliative repair Tailor-made….classical / modified BT Transanular PatchRVOT patch Infundibular patchPotts Double patchWaterston Commissurotomy Monocusp Injectable pulmonary valve VSD closure
  • 7. Palliative / complete repairPalliative repairclassical / modified BT :• aneurysm, stenosis, distortion of theshunt…• Stenosis / distortion of PAs (from regular to “irregular”)• development of MAPCAs• LV overload• acquired atresia of the PV
  • 8. Palliative / complete repairPalliative repairRVOT patch:• (CEC) aneurysm ?Potts / Waterston :• dilatation / stenosis / distortion ofPAs, PAH
  • 9. Palliative / complete repair Complete repair Tailor-made….Transanular Patch : PVRInfundibular patch : infundibular dilatation or obstructionDouble patch : infundibular dilatation or obstructionCommissurotomy : PVRMonocusp : PVRInjectable pulmonary valve : PVRVSD closure : residual VSD
  • 10. PR in adult patients•  Progressive dilatation or the RV•  Ventricular Arrhythmias (QRS >)•  Dilatation ofthe tricuspid annulus TR•  PR + TR RV dysfunction•  In patients with restrictive physiology decreased duration and volume of PR (antegrade flow in end -diastole)Redington Card Young
  • 11. Postoperative problems WHAT WAS KNOWN Complete repair•  Re-stenosis of RVOT•  Dilatation of RVOT•  PV regurgitation (native or artificial)•  PA stenoses•  MAPCAs•  AR•  VSD
  • 12. New problemsvicious circles : rythm - function - dysfunctionVolume / pressure overload + myocardial injury (independent from type of correction) Progressive increase of PRProgressive enlargement of QRS   "  Arrhythmias / dysfunction Ann Noninvasive Elettrocardiol 2011
  • 13. Strain !
  • 14. Rythm - Function - Dysfunction• Delayed RVO deformation Heart 2011• < LV strain, related to degree of RV dilatation AmJ Cardiol 2011• < regional deformation of RV and IVS related to PR J Am Soc Echocardiogr 2011• RV-septal dyssynchrony related to degree of PREur J Cardiothor Surg 200• QRS duration related to LV longitudinal strainCirculation 2012
  • 15. Rythm!• Sudden death•  AVB•  VT•  Atrial arrhythmias•  PM, ICD, resincronization….
  • 16. Dyssynchronous… Comparison of left ventricular contractility between before and after use of the cardiac resynchronization therapy with a defibrillation system (CRT-D). Saito Y et al. Interact CardioVasc Thorac Surg 2011;12:64-66
  • 17. Medical treatment• PREVENTION• ETIOLOGICAL TREATMENT?• TREATMENT OF SYMPTOMS? (antyarrhythmics...)• ACCURATE FOLLOW-UP !• Clinical evaluation, ECG, Holter, ECO, (2D, 3D, TDI...), VO2, MRI
  • 18. •  Restenosis of RVOT Dilatation - stent•  Stenosis of PAs Dilatation - stent•  Regurgitation/stenosis of PV PVR•  MAPCAs Embolisation•  Residual VSD Occlusive devices•  AR Percutaneous valves ?
  • 19. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  • 20. Postoperative Fallot What we should look at•  Pulmonary arteries : hypoplasia
  • 21. Postoperative Fallot What we should look at•  Embriological development of PAs stops at 3mo postnatally - flow-dependent effects•  Pulmonary arteries 1 year later after cutting
  • 22. Postoperative Fallot What we should look at•  Pulmonary arteries: coarctation of LPA
  • 23. Postoperative Fallot•  Pulmonary arteries: ipo LPA (stent)
  • 24. Postoperative Fallot What we should look at but it is too late…•  Pulmonary arteries: Single RPA…
  • 25. Postoperative Fallot What we should look at but it is too late…•  Pulmonary arteries: Single LPA
  • 26. Postoperative Fallot•  Pulmonary arteries: kissing Stent
  • 27. Postoperative Fallot What we should look at•  Pulmonary arteries: StentWhich weight? Redilatation, Fracture, restenosis… Stent-related diseases….
  • 28. Postoperative Fallot What we should look at StentsWhich weight? Redilatation, Fracture, Restenosis… Stents in children < 30 Kg New stents (Advanta etc) Low profile Resorbable????? Concern for MRI/CT…
  • 29. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  • 30. Postoperative Fallot PVR Why? How? To avoid •  RV failureWHY •  Ventricular arrhythmia •  Atrial arrhythmia • QRS enlargement +++: too lateWHEN • Refractory arrythmias : too late • RV dyssynchrony : too late • RV/LV dysfunction : too late… Timing ! Preventive treatment in « asymptomatic patients » based on benefit/risk ratio
  • 31. PVR WHEN ?• PVR do not normalize RV function nor solve the arrhythmogenicity Circ J 2009• Severe QRS prolongation before or AFTER PVR determinant of adverse outcome J Am Coll Cardiol 2010• PVR do not improve RV EF or QRS duration (meta-analysis) Am J Cardiol 2010• PVR may improve LV function Tex Heart Inst J 2011
  • 32. LVEF after PVR• PVR may improve LV function Tex Heart Inst J 2011
  • 33. PVR MRI studies17 adults after PVR Therrien J, Am J Cardiol, 2005 •  RVEDV >170 ml/m² no improvement •  RVEDV > 85 ml/m² normalization of volumes20 children before/after PVR Valsangiacomo Büchel. European Heart Journal. 2005•  Significant decrease of RVEDV, RVESV, RV mass RVEDV ≥200ml/m² : no normalization No change in RV EF
  • 34. PVR: HOW ?• Surgical (biological, mechanical, injectable…)• Percutaneous? (Melody, Sapien, new valves...)
  • 35. Postoperative Fallot•  Pulmonary valve: incompetence
  • 36. Postoperative Fallot•  Pulmonary valve: incompetence
  • 37. Percutaneous PV• Durability ? Morbidity ?• Stent fracture, endocarditis, trombosis...... Stent fracture, valve dysfunction, and right ventricular outflow tract reintervention after transcatheter pulmonary valve implantation: patient -related and procedural risk factors in the US Melody Valve Trial. Circ Cardiovasc Interv 2011
  • 38. Postoperative Fallot•  Pulmonary valve: stenosis
  • 39. Postoperative Fallot•  Acquired atresia of the PV RFA perforation
  • 40. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  • 41. Balloon / Stenting of RVOT •  Balloon: rarely effective Stent •  Relieves the obstruction •  Low risk/benefit ratio •  Prolongs the life of RV-PA conduits •  Transforms the obstruction in free PR Size of pulmonary branches!
  • 42. Postoperative Fallot•  Infundibulum
  • 43. Postoperative Fallot•  Infundibulum
  • 44. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  • 45. •  RV/ LV !!!!!Postoperative Fallot
  • 46. •  RV/ LV !!!!!Postoperative Fallot
  • 47. Postoperative Fallot•  RV/ LV !!!!!
  • 48. •  RV/LV !!!!!Postoperative (percutaneous) Fallot
  • 49. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  • 50. Occluding devicesTo close a communication I want to stent it….
  • 51. Residual VSD
  • 52. Devices and ideas How shall I close a hole? Generally putting somethinginside....or simply covering it.... cover occlude anchorage
  • 53. Devicesand ideas
  • 54. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  • 55. Aortic root• Progressive aneurismal dilatation and AR in 15% of patients• Normalisation of initially dilated aortic root (Eur J Cardiothor Surg 2010) … • Early repair !
  • 56. Postoperative Fallot•  Other problems: collaterals (overload, bleeding…)•  Embolisations: coils, particles, glue, devices…..
  • 57. Il Fallot e’ una malattia che guarisce o una malattia cronica ?
  • 58. Per chi voglia dedicarsi ad attività piu’ redditizie e meno rischiose… Riconoscere una crisi anossica, conoscere l’echo