Tetralogia di Fallot
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Transcript of Tetralogia di Fallot
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Tetralogia di Fallot Sequele post correzione chirurgica
Trattamento medico e trans-catetere
G Agnoletti Citta’ della Salute
TORINO [email protected]
Cardiopatie congenite: Dalla diagnosi prenatale all’età adulta
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• 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)
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Tetralogy of Fallot A single anomaly ? (when regular….)
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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)
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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
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Palliative / complete repair
Palliative repair classical / modified BT
RVOT patch
Potts
Waterston
Complete repair Tailor-made….
Transanular Patch
Infundibular patch
Double patch
Commissurotomy
Monocusp
Injectable pulmonary valve
VSD closure
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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
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Palliative / complete repair
Palliative repair RVOT patch:
• (CEC) aneurysm ?
Potts / Waterston :
• dilatation / stenosis / distortion of PAs, PAH
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Palliative / complete repair Complete repair Tailor-made….
Transanular Patch : PVR
Infundibular patch : infundibular dilatation or obstruction
Double patch : infundibular dilatation or obstruction
Commissurotomy : PVR
Monocusp : PVR
Injectable pulmonary valve : PVR
VSD closure : residual VSD
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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
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Postoperative problems WHAT WAS KNOWN
Complete repair • Re-stenosis of RVOT • Dilatation of RVOT • PV regurgitation (native or artificial) • PA stenoses • MAPCAs • AR • VSD
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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
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Strain !
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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
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Rythm!
• Sudden death
• AVB
• VT
• Atrial arrhythmias
• PM, ICD, resincronization….
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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
Dyssynchronous…
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Medical treatment
• PREVENTION • ETIOLOGICAL TREATMENT? • TREATMENT OF SYMPTOMS? (antyarrhythmics...) • ACCURATE FOLLOW-UP ! • Clinical evaluation, ECG, Holter, ECO, (2D, 3D, TDI...), VO2, MRI
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• Restenosis of RVOT Dilatation - stent
• Stenosis of PAs Dilatation - stent
• Regurgitation/stenosis of PV PVR
• MAPCAs Embolisation
• Residual VSD Occlusive devices
• AR Percutaneous valves ?
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Postoperative Fallot What we should look at
• Pulmonary arteries • Pulmonary valve • Infundibular region • Right ventricle • Left ventricle • Interventricular septum • Aorta
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Postoperative Fallot What we should look at
• Pulmonary arteries : hypoplasia
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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
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Postoperative Fallot What we should look at
• Pulmonary arteries: coarctation of LPA
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Postoperative Fallot • Pulmonary arteries: ipo LPA (stent)
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Postoperative Fallot What we should look at
but it is too late…
• Pulmonary arteries: Single RPA…
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Postoperative Fallot What we should look at
but it is too late…
• Pulmonary arteries: Single LPA
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Postoperative Fallot • Pulmonary arteries: kissing Stent
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Postoperative Fallot What we should look at
• Pulmonary arteries: Stent Which weight? Redilatation, Fracture, restenosis…
Stent-related diseases….
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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…
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Postoperative Fallot What we should look at
• Pulmonary arteries • Pulmonary valve • Infundibular region • Right ventricle • Left ventricle • Interventricular septum • Aorta
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Postoperative Fallot PVR Why? How?
To avoid • RV failure • Ventricular arrhythmia • Atrial arrhythmia
• QRS enlargement +++: too late • Refractory arrythmias : too late • RV dyssynchrony : too late • RV/LV dysfunction : too late…
WHY
WHEN
Timing ! Preventive treatment in « asymptomatic patients » based on benefit/risk ratio
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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
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LVEF after PVR
• PVR may improve LV function Tex Heart Inst J 2011
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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
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PVR: HOW ? • Surgical (biological, mechanical, injectable…) • Percutaneous? (Melody, Sapien, new valves...)
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Postoperative Fallot
• Pulmonary valve: incompetence
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Postoperative Fallot • Pulmonary valve: incompetence
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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
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Postoperative Fallot • Pulmonary valve: stenosis
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Postoperative Fallot • Acquired atresia of the PV
RFA perforation
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Postoperative Fallot What we should look at
• Pulmonary arteries • Pulmonary valve • Infundibular region • Right ventricle • Left ventricle • Interventricular septum • Aorta
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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!
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Postoperative Fallot • Infundibulum
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Postoperative Fallot • Infundibulum
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Postoperative Fallot What we should look at
• Pulmonary arteries • Pulmonary valve • Infundibular region • Right ventricle • Left ventricle • Interventricular septum • Aorta
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Postoperative Fallot
• RV/ LV !!!!!
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Postoperative Fallot
• RV/ LV !!!!!
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Postoperative Fallot • RV/ LV !!!!!
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Postoperative (percutaneous) Fallot
• RV/LV !!!!!
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Postoperative Fallot What we should look at
• Pulmonary arteries • Pulmonary valve • Infundibular region • Right ventricle • Left ventricle • Interventricular septum • Aorta
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Occluding devices To close a communication I
want to stent it….
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Residual VSD
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Devices and ideas
How shall I close a hole? Generally putting something
inside....or simply covering it.... cover
occlude anchorage
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Devices and ideas
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Postoperative Fallot What we should look at
• Pulmonary arteries • Pulmonary valve • Infundibular region • Right ventricle • Left ventricle • Interventricular septum • Aorta
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Aortic root
• Progressive aneurismal dilatation and AR in 15% of patients • Normalisation of initially dilated aortic root (Eur J Cardiothor Surg 2010) … • Early repair !
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Postoperative Fallot • Other problems: collaterals (overload, bleeding…) • Embolisations: coils, particles, glue, devices…..
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Il Fallot e’ una malattia che guarisce o una malattia cronica ?
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Per chi voglia dedicarsi ad attività piu’ redditizie e meno rischiose…
Riconoscere una crisi anossica, conoscere l’echo