Tetralogia di Fallot Sequele post correzione chirurgicaTrattamento medico e trans-catetere             G Agnoletti        ...
•  4-11% of children born with CHD•  most common cyanotic CHD•  “easy” disease…•  surgical correction•  large number of ad...
Tetralogy of FallotA single anomaly ? (when regular….)
Type of FallotRegular:     Normal ventricles and A-V valves     One VSD     No coronary artery crossing the annulus     No...
Irregular Fallot•  Obstruction at the origin of LPA•  Abnormal LAD crossing the anulus•  Multiple VSD•  Old Fallot with sm...
Palliative / complete repair                             Complete repairPalliative repair               Tailor-made….class...
Palliative / complete repairPalliative repairclassical / modified BT :• aneurysm, stenosis, distortion of theshunt…• Steno...
Palliative / complete repairPalliative repairRVOT patch:• (CEC) aneurysm ?Potts / Waterston :• dilatation / stenosis / dis...
Palliative / complete repair           Complete repair Tailor-made….Transanular Patch : PVRInfundibular patch : infundibul...
PR in adult patients•     Progressive dilatation or the RV•     Ventricular Arrhythmias (QRS >)•     Dilatation ofthe tric...
Postoperative problems         WHAT WAS KNOWN             Complete repair•  Re-stenosis of RVOT•  Dilatation of RVOT•  PV ...
New problemsvicious circles : rythm - function - dysfunctionVolume / pressure overload + myocardial  injury (independent f...
Strain !
Rythm - Function - Dysfunction• Delayed RVO deformation Heart 2011• < LV strain, related to degree of RV dilatation AmJ Ca...
Rythm!• Sudden death•  AVB•  VT•  Atrial arrhythmias•  PM, ICD, resincronization….
Dyssynchronous…           Comparison of left ventricular           contractility between before and after           use of...
Medical treatment• PREVENTION• ETIOLOGICAL TREATMENT?• TREATMENT OF SYMPTOMS? (antyarrhythmics...)• ACCURATE FOLLOW-UP !• ...
•  Restenosis of RVOT             Dilatation - stent•  Stenosis of PAs                Dilatation - stent•  Regurgitation/s...
Postoperative Fallot             What we should look at•    Pulmonary arteries•    Pulmonary valve•    Infundibular region...
Postoperative Fallot           What we should look at•  Pulmonary arteries : hypoplasia
Postoperative Fallot           What we should look at•  Embriological development of PAs stops at 3mo   postnatally - flow...
Postoperative Fallot          What we should look at•  Pulmonary arteries: coarctation of LPA
Postoperative Fallot•  Pulmonary arteries: ipo LPA (stent)
Postoperative Fallot            What we should look at                but it is too late…•  Pulmonary arteries: Single RPA…
Postoperative Fallot            What we should look at                but it is too late…•  Pulmonary arteries: Single LPA
Postoperative Fallot•  Pulmonary arteries: kissing Stent
Postoperative Fallot              What we should look at•  Pulmonary arteries: StentWhich weight? Redilatation, Fracture, ...
Postoperative Fallot              What we should look at                        StentsWhich weight? Redilatation, Fracture...
Postoperative Fallot             What we should look at•    Pulmonary arteries•    Pulmonary valve•    Infundibular region...
Postoperative Fallot                 PVR Why? How?               To avoid               •  RV failureWHY            •  Ven...
PVR WHEN ?• PVR do not normalize RV function nor solve the arrhythmogenicity Circ J 2009• Severe QRS prolongation before o...
LVEF after PVR• PVR may improve LV function Tex Heart Inst J 2011
PVR                       MRI studies17 adults after PVR Therrien J, Am J Cardiol, 2005  •  RVEDV >170 ml/m²              ...
PVR: HOW ?• Surgical (biological, mechanical, injectable…)• Percutaneous? (Melody, Sapien, new valves...)
Postoperative Fallot•  Pulmonary valve: incompetence
Postoperative Fallot•  Pulmonary valve: incompetence
Percutaneous PV• Durability ? Morbidity ?• Stent fracture, endocarditis, trombosis......    Stent fracture, valve dysfunct...
Postoperative Fallot•  Pulmonary valve: stenosis
Postoperative Fallot•  Acquired atresia of the PV                  RFA perforation
Postoperative Fallot             What we should look at•    Pulmonary arteries•    Pulmonary valve•    Infundibular region...
Balloon / Stenting of RVOT       •  Balloon: rarely effective       Stent       •  Relieves the obstruction       •  Low r...
Postoperative Fallot•  Infundibulum
Postoperative Fallot•  Infundibulum
Postoperative Fallot             What we should look at•    Pulmonary arteries•    Pulmonary valve•    Infundibular region...
•  RV/ LV !!!!!Postoperative Fallot
•  RV/ LV !!!!!Postoperative Fallot
Postoperative Fallot•  RV/ LV !!!!!
•  RV/LV !!!!!Postoperative (percutaneous) Fallot
Postoperative Fallot             What we should look at•    Pulmonary arteries•    Pulmonary valve•    Infundibular region...
Occluding devicesTo close a communication I     want to stent it….
Residual VSD
Devices            and ideas    How shall I close a hole?  Generally putting somethinginside....or simply covering it.... ...
Devicesand ideas
Postoperative Fallot             What we should look at•    Pulmonary arteries•    Pulmonary valve•    Infundibular region...
Aortic root• Progressive aneurismal dilatation and AR in 15% of patients• Normalisation of initially dilated aortic root (...
Postoperative Fallot•  Other problems: collaterals (overload, bleeding…)•  Embolisations: coils, particles, glue, devices…..
Il Fallot e’ una malattia che guarisce o          una malattia cronica ?
Per chi voglia dedicarsi ad attività piu’ redditizie               e meno rischiose…   Riconoscere una crisi anossica, con...
 Tetralogia di Fallot
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Tetralogia di Fallot

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Tetralogia di Fallot

  1. 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. 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. 3. Tetralogy of FallotA single anomaly ? (when regular….)
  4. 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. 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. 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. 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. 8. Palliative / complete repairPalliative repairRVOT patch:• (CEC) aneurysm ?Potts / Waterston :• dilatation / stenosis / distortion ofPAs, PAH
  9. 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. 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. 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. 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. 13. Strain !
  14. 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. 15. Rythm!• Sudden death•  AVB•  VT•  Atrial arrhythmias•  PM, ICD, resincronization….
  16. 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. 17. Medical treatment• PREVENTION• ETIOLOGICAL TREATMENT?• TREATMENT OF SYMPTOMS? (antyarrhythmics...)• ACCURATE FOLLOW-UP !• Clinical evaluation, ECG, Holter, ECO, (2D, 3D, TDI...), VO2, MRI
  18. 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. 19. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  20. 20. Postoperative Fallot What we should look at•  Pulmonary arteries : hypoplasia
  21. 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. 22. Postoperative Fallot What we should look at•  Pulmonary arteries: coarctation of LPA
  23. 23. Postoperative Fallot•  Pulmonary arteries: ipo LPA (stent)
  24. 24. Postoperative Fallot What we should look at but it is too late…•  Pulmonary arteries: Single RPA…
  25. 25. Postoperative Fallot What we should look at but it is too late…•  Pulmonary arteries: Single LPA
  26. 26. Postoperative Fallot•  Pulmonary arteries: kissing Stent
  27. 27. Postoperative Fallot What we should look at•  Pulmonary arteries: StentWhich weight? Redilatation, Fracture, restenosis… Stent-related diseases….
  28. 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. 29. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  30. 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. 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. 32. LVEF after PVR• PVR may improve LV function Tex Heart Inst J 2011
  33. 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. 34. PVR: HOW ?• Surgical (biological, mechanical, injectable…)• Percutaneous? (Melody, Sapien, new valves...)
  35. 35. Postoperative Fallot•  Pulmonary valve: incompetence
  36. 36. Postoperative Fallot•  Pulmonary valve: incompetence
  37. 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. 38. Postoperative Fallot•  Pulmonary valve: stenosis
  39. 39. Postoperative Fallot•  Acquired atresia of the PV RFA perforation
  40. 40. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  41. 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. 42. Postoperative Fallot•  Infundibulum
  43. 43. Postoperative Fallot•  Infundibulum
  44. 44. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  45. 45. •  RV/ LV !!!!!Postoperative Fallot
  46. 46. •  RV/ LV !!!!!Postoperative Fallot
  47. 47. Postoperative Fallot•  RV/ LV !!!!!
  48. 48. •  RV/LV !!!!!Postoperative (percutaneous) Fallot
  49. 49. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  50. 50. Occluding devicesTo close a communication I want to stent it….
  51. 51. Residual VSD
  52. 52. Devices and ideas How shall I close a hole? Generally putting somethinginside....or simply covering it.... cover occlude anchorage
  53. 53. Devicesand ideas
  54. 54. Postoperative Fallot What we should look at•  Pulmonary arteries•  Pulmonary valve•  Infundibular region•  Right ventricle•  Left ventricle•  Interventricular septum•  Aorta
  55. 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. 56. Postoperative Fallot•  Other problems: collaterals (overload, bleeding…)•  Embolisations: coils, particles, glue, devices…..
  57. 57. Il Fallot e’ una malattia che guarisce o una malattia cronica ?
  58. 58. Per chi voglia dedicarsi ad attività piu’ redditizie e meno rischiose… Riconoscere una crisi anossica, conoscere l’echo

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