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Tetralogia di Fallot
1. Tetralogia di Fallot
Sequele post correzione chirurgica
Trattamento 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)
4. Type of Fallot
Regular:
Normal ventricles and A-V valves
One VSD
No coronary artery crossing the annulus
No PA branch stenosis
Favorable:
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
7. Palliative / complete repair
Palliative repair
classical / modified BT :
• aneurysm, stenosis, distortion of the
shunt…
• Stenosis / distortion of PAs (from regular
to “irregular”)
• development of MAPCAs
• LV overload
• acquired atresia of the PV
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 problems
vicious circles : rythm - function - dysfunction
Volume / pressure overload + myocardial
injury (independent from type of correction)
Progressive increase of PR
Progressive enlargement of QRS
"
Arrhythmias / dysfunction
Ann Noninvasive Elettrocardiol 2011
14. Rythm - Function - Dysfunction
• Delayed RVO deformation Heart 2011
• < LV strain, related to degree of RV dilatation Am
J Cardiol 2011
• < regional deformation of RV and IVS related to
PR J Am Soc Echocardiogr 2011
• RV-septal dyssynchrony related to degree of PR
Eur J Cardiothor Surg 200
• QRS duration related to LV longitudinal strain
Circulation 2012
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
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
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
27. Postoperative Fallot
What we should look at
• Pulmonary arteries: Stent
Which weight? Redilatation, Fracture, restenosis…
Stent-related diseases….
28. Postoperative Fallot
What we should look at
Stents
Which 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 failure
WHY • Ventricular arrhythmia
• Atrial arrhythmia
• QRS enlargement +++: too late
WHEN • 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
33. PVR
MRI studies
17 adults after PVR Therrien J, Am J Cardiol, 2005
• RVEDV >170 ml/m² no improvement
• RVEDV > 85 ml/m² normalization of volumes
20 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.
35. PVR: HOW ?
• Surgical (biological, mechanical, injectable…)
• Percutaneous? (Melody, Sapien, new
valves...)
41. Postoperative Fallot
What we should look at
• Pulmonary arteries
• Pulmonary valve
• Infundibular region
• Right ventricle
• Left ventricle
• Interventricular septum
• Aorta
42. 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!
45. Postoperative Fallot
What we should look at
• Pulmonary arteries
• Pulmonary valve
• Infundibular region
• Right ventricle
• Left ventricle
• Interventricular septum
• Aorta
50. Postoperative Fallot
What we should look at
• Pulmonary arteries
• Pulmonary valve
• Infundibular region
• Right ventricle
• Left ventricle
• Interventricular septum
• Aorta
55. Postoperative Fallot
What we should look at
• Pulmonary arteries
• Pulmonary valve
• Infundibular region
• Right ventricle
• Left ventricle
• Interventricular septum
• Aorta
56. Aortic root
• Progressive aneurismal dilatation and AR
in 15% of patients
• Normalisation of initially dilated aortic
root (Eur J Cardiothor Surg 2010) …
• Early repair !
