"Giancarlo Rastelli Lecture"

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1 "Giancarlo Rastelli Lecture" Surgical treatment of Malpositions of the Great Arteries Pascal Vouhé

2 Giancarlo Rastelli ( ) Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau

3 Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau normal malpositions transposition

4 Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau niveau Ao Quatrième PA Cinquième niveau VSD RV LV

5 1. ventricular septal defect - nearly always conoventricular - potential extension (inlet, trabecular) - very rarely: muscular 2. subarterial conal muscles - usually: double conus - asymmetrical development of each conus - makes the variability in anatomy

6 Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau

7 Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau Robert Replogle : top-ten wine cellars in the world (Wine Spectator)

8 There are few more perplexing areas than the continuum of complete transposition of the great arteries, DORV and tetralogy of Fallot. In the overall scheme of things, the definition of congenital heart disease is less important than how well the malformation can be repaired. Robert Replogle 1985

9 for the surgeon : 3 (simple) questions 1. is biventricular repair possible? if "YES" 2. is "anatomic" repair feasible? if "NO" 3. which extra-anatomic repair is indicated?

10 1. is biventricular repair possible? - 5 anatomic conditions must be fulfilled - often associated - most difficult question

11 1. there is no ventricular hypoplasia - RV (coarctation) or LV (LVOTO) - acceptable limit? no clear-cut rule (AV orifice size, volume MRI..) - RV : cavopulmonary anastomosis - LV :. shunt, ASD closure (to promote growth). atrial repair (leaving RV as systemic)

12 2. VSD(s) can be closed - multiple VSDs - residual VSD is badly tolerated - complete closure is mandatory - hybrid approach may be useful

13 MGA: surgical treatment 3. AV valves do not preclude repair - tricuspid and/or mitral valves - overiding / straddling - abnormal insertions on conal septum - malformation (cleft.) - associated valve replacement may be an option

14 4. there is no outflow obstruction - coarctation (native or residual) - pulmonary vascular bed (hypoplasia, resistances)

15 5. it is possible to connect the LV to one of the great arteries - position of VSD / conduction tissue - in most cases: conoventricular VSD. resection of conal septum is possible. even if it must be extensive (difficult?) - rarely: muscular VSD (conal septum resection is not possible)

16 1. is biventrivular repair possible?. after evaluating the 5 previous conditions. technically not possible. technically possible but hazardous univentricular repair may be indicated

17 2. is "anatomic" repair feasible? - "anatomic" repair = Intra Ventricular Repair - LV to aorta - RV to PA (left in native position) - one question: tricuspid-pulmonary distance? - subpulmonary conus lenght - or < aortic orifice diameter

18 tricuspid to pulmonary valve distance

19 Intra Ventricular Repair - IVR short subaortic conus long subaortic conus

20 Intraventricular repair

21 Intraventricular repair

22 Intraventricular repair

23 Intraventricular repair : results. low early risk ( ). NEM : 0% (0/43). EACTS : 6.9% (40/581). satisfactory late results ( VSD or ToF). subaortic stenosis ( < 5%). residual/recurrent pulmonary stenosis. normal quality of life

24 2. is "anatomic" repair feasible? 2 exceptions : IVR possible but difficult. anomalous insertions tricuspid ± mitral. asymmetry of subpulmonary conus

25 anomalous insertions of tricuspid valve : IVR possible but difficult Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau

26 anomalous insertions of tricuspid valve : IVR possible but difficult uez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau

27 anomalous insertions of tricuspid valve : quez pour modifier les styles du texte du masque extra-anatomic repair may be easier Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau 1. LV-PA + switch (normal pulmonary valve) 2. Bex-Nikaïdoh operation (pulmonary stenosis)

28 2. is "anatomic" repair feasible? 2 exceptions : IVR possible but difficult. anomalous insertions tricuspid ± mitral. asymmetry of subpulmonary conus

29 asymmetry of subpulmonary conus extra-anatomic repair 1. LV-PA + switch (normal PV) 2. REV operation (pulm. stenosis) symmetric conus asymmetric conus

30 3. which extra-anatomic repair is indicated? - tricuspid-pulmonary distance < aortic orifice - "anatomic" repair is not possible - one question : pulmonary outflow tract? (normal / stenosed)

31 3. which extra-anatomic repair is indicated? - normal POT: LV-PA + switch - stenosed POT: REV (or Rastelli) Bex-Nikaïdoh (LV-Ao tunnel impossible)

32 extra-anatomic repair stenosed POT normal POT

33 extra-anatomic repair : LV PA connection

34 extra-anatomic repair : LV-PA + switch

35 extra-anatomic repair : REV conal septum excision conal septum mobilization

36 extra-anatomic repair : REV

37 REV procedure: results. early risk is now low : 12% (24/205) : 0% (0/34). satisfactory late results ( ToF)

38 Survival (excluding hospital mortality) 1, , % at 25 years 0, , , , ,0 Di Carlo D. et al , , ,0 J Thorac Cardiovasc Surg 2011;142:336

39 MGA : surgical treatmant Adverse events (cumulative incidence method) 45% at 25 yrs Di Carlo D. et al. J Thorac Cardiovasc Surg 2011;142:336

40 MGA: surgical treatment Rastelli vs REV : late results Mayo pts 160 Boston pts 101 Munich pts 39 REV 171 pts survival freedom from RVOT reoperation 59% 27% 52% 25% 84% 58% 32% 93% 85% 66% 95% Di Carlo D. et al. freedom from LVOTO reoperation J Thorac Cardiovasc Surg 2011;142:336

41 extra-anatomic repair : Bex-Nikaidoh operation

42 Bex - Nikaidoh operation : results Nb pts mortality (early+late ) follow-up (mths) reop. LVOTO reop. RVOTO Dallas (2007) % % Boston (2007) 11 0% % Pittsburg (2007) 21 19% % Paris-NEM ( ) 9 11% % 60 10% 30%

43 MGA : surgical management Decision - making. echocardiography - planning of repair - surgeon must be present. 3D imaging. computer reconstruction

44 Biventricular repair? : 5 conditions? NO FONTAN "Anatomic" repair? : tricuspid-pulmonary distance? > Ao IVR < Ao Extra-anatomic repair? : pulmonary stenosis? NO LV-PA + switch YES Bex-Nikaïdoh REV

45 Conclusions. surgical indication : usually not difficult - careful preoperative evaluation - close collaboration cardiologist / surgeon. biventricular repair - is usually possible - carries a low early risk - provides satisfactory late results. univentricular repair may be an option in selected patients

46 It is possible to make things complex it is better to keep it simple!!

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