"Giancarlo Rastelli Lecture" Surgical treatment of Malpositions of the Great Arteries Pascal Vouhé
Giancarlo Rastelli (1933 1970) Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau
Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau normal malpositions transposition
Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau niveau Ao Quatrième PA Cinquième niveau VSD RV LV
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
Cliquez pour modifier les styles du texte du masque Deuxième niveau Troisième niveau Quatrième niveau Cinquième niveau
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)
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
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?
1. is biventricular repair possible? - 5 anatomic conditions must be fulfilled - often associated - most difficult question
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)
2. VSD(s) can be closed - multiple VSDs - residual VSD is badly tolerated - complete closure is mandatory - hybrid approach may be useful
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
4. there is no outflow obstruction - coarctation (native or residual) - pulmonary vascular bed (hypoplasia, resistances)
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)
1. is biventrivular repair possible?. after evaluating the 5 previous conditions. technically not possible. technically possible but hazardous univentricular repair may be indicated
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
tricuspid to pulmonary valve distance
Intra Ventricular Repair - IVR short subaortic conus long subaortic conus
Intraventricular repair
Intraventricular repair
Intraventricular repair
Intraventricular repair : results. low early risk (2007 2011). 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
2. is "anatomic" repair feasible? 2 exceptions : IVR possible but difficult. anomalous insertions tricuspid ± mitral. asymmetry of subpulmonary conus
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
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
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)
2. is "anatomic" repair feasible? 2 exceptions : IVR possible but difficult. anomalous insertions tricuspid ± mitral. asymmetry of subpulmonary conus
asymmetry of subpulmonary conus extra-anatomic repair 1. LV-PA + switch (normal PV) 2. REV operation (pulm. stenosis) symmetric conus asymmetric conus
3. which extra-anatomic repair is indicated? - tricuspid-pulmonary distance < aortic orifice - "anatomic" repair is not possible - one question : pulmonary outflow tract? (normal / stenosed)
3. which extra-anatomic repair is indicated? - normal POT: LV-PA + switch - stenosed POT: REV (or Rastelli) Bex-Nikaïdoh (LV-Ao tunnel impossible)
extra-anatomic repair stenosed POT normal POT
extra-anatomic repair : LV PA connection
extra-anatomic repair : LV-PA + switch
extra-anatomic repair : REV conal septum excision conal septum mobilization
extra-anatomic repair : REV
REV procedure: results. early risk is now low. 1981 2003 : 12% (24/205). 2007 2011 : 0% (0/34). satisfactory late results ( ToF)
Survival (excluding hospital mortality) 1,0 0 0 0,7 5 0 85 % at 25 years 0,5 0 0 0,2 5 0 0,0 0 0 0,0 1 0 0,0 Di Carlo D. et al. 2 0 0,0 3 0 0,0 4 0 0,0 J Thorac Cardiovasc Surg 2011;142:336
MGA : surgical treatmant Adverse events (cumulative incidence method) 45% at 25 yrs Di Carlo D. et al. J Thorac Cardiovasc Surg 2011;142:336
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
extra-anatomic repair : Bex-Nikaidoh operation
Bex - Nikaidoh operation : results Nb pts mortality (early+late ) follow-up (mths) reop. LVOTO reop. RVOTO Dallas (2007) 19 5.2% 132 0 38% Boston (2007) 11 0% 59 0 45% Pittsburg (2007) 21 19% 71 0 18% Paris-NEM (2004-11) 9 11% 36 0 25% 60 10% 30%
MGA : surgical management Decision - making. echocardiography - planning of repair - surgeon must be present. 3D imaging. computer reconstruction
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
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
It is possible to make things complex it is better to keep it simple!!