Tetralogy of Fallot. Damien Bonnet
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1 Tetralogy of Fallot Damien Bonnet Unité médico-chirurgicale de Cardiologie Congénitale et Pédiatrique Hôpital Universitaire Necker Enfants malades APHP, Université Paris Descartes, Sorbonne Paris Cité IcarP Cardiology, Institut Hospitalo-Universitaire IMAGINE Centre de Référence Maladies Rares Malformations Cardiaques Congénitales Complexes-M3C Centre de Référence Maladies Rares Maladies Cardiaques Héréditaires- CARDIOGEN
2 Migration of neural crest cells into the outflow tract Hutson & Kirby 2007
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4 Subpulmonary'' conus' PA# Septal'' band' Postero'inferior+limb+ of+the+septal+band+ Anterior+limb+ +of+the+septal+band+ Ventriculo1' infundibular'' fold' VIF# TV# Anterior'papillary'muscle'of'the'tricuspid'valve' Moderator(band(
5 Subpulmonary'' conus' PA# Septal'' band' Ventriculo1' infundibular'' fold' VIF# TV# Anterior'papillary'muscle'of'the'tricuspid'valve' Moderator(band(
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7 Main topics in tetralogy of Fallot Prenatal diagnosis Perinatal management Strategy for repair Late outcomes
8 Prenatal diagnosis of tetralogy of Fallot What are the main issues? 1. Associated cytogenetic and extra-cardiac anomalies
9 Trisomie 21 Délétion 22q11 Alagille CHARGE Waardenburg 10 % karyotypic anomalies on standard analysis 18% 22q11 deletion : 15% in ToF-PS, 26% in ToF-PA
10 Phenotypic heterogeneity One genotype-different cardiac phenotypes ToF IAA CAT 1 gene X CHD FISH 22q11 deletion
11 ToF IAA PA-VSD CAT ToF-Abs PV DORV
12 One third of fetuses with ToF had extra cardiac anomalies 15% had intra-uterine growth retardation Kaguelidou F, Bonnet D. Eur Heart J Jun;29(11):
13 Prenatal diagnosis of tetralogy of Fallot What are the main issues? 1. Associated cytogenetic and extra-cardiac anomalies 2. Accuracy of diagnosis and decision for in utero transfer
14 Accuracy of diagnosis ToF-PS can be ToF-PA ToF-PA can be Common arterial trunk Left pulmonary artery branch can arise from the arterial duct Kaguelidou F, Bonnet D. Eur Heart J Jun;29(11):
15 Prenatal diagnosis of tetralogy of Fallot What are the main issues? 1. Associated cytogenetic and extra-cardiac anomalies 2. Accuracy of diagnosis and decision for in utero transfer 3. Prediction of repair
16 Kaguelidou F, Bonnet D. Eur Heart J Jun;29(11):
17 Kaguelidou F, Bonnet D. Eur Heart J Jun;29(11):
18 Perinatal management 1. Ducto-dependent defect?
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21 Perinatal management 1. Ducto-dependent defect? 2. Associated cardiac anomalies
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24 Pulmonary branches stenosis Multiple VSDs
25 AVSD Fallot
26 Outflow VSD without conal septum
27 Patent arterial duct from brachiocephalic trunk
28 Perinatal management 1. Ducto-dependent defect? 2. Associated cardiac anomalies 3. Palliative surgery
29 Palliative surgery Ducto-dependent? Yes No ToF-PS ToF-PA 1 ToF-PA 2 ToF-PS ToF-PA + MAPCAs Staged rehabilitation of pulmonary arteries RV-PA opening BT shunt RV-PA restrictive conduit Complete repair at > 6 kgs
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31 Helen Taussig Alfred Blalock and Eileen Saxon Vivien Thomas 31
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39 MAPCAs with or without communication with the pulmonary arteries
40 MAPCAs with or without communication with the pulmonary arteries
41 Unifocalisation
42 Stategy for repair What are the main issues? Neonatal repair could be performed - closure of VSD is difficult - pulmonary arteries size and PVR do not always allow repair without conduit Repair with closure of the VSD in children > 6 kgs - If closure of VSD is possible : multiple VSD - Without conduit : coronary artery anatomy - Without valve : size/stenosis of pulmonary artery branches
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46 Late outcome What are the issues? Right outflow tract obstructions Pulmonary branches stenoses Chronic pulmonary regurgitation Ventricular tachycardia, other arrhythmias and sudden death
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50 Conduits VD-AP
51 Traitement chirurgical de la tétralogie de Fallot Réparation Voix atriale Patch pulmonaire Voix infundibulaire Patch infundibulo-pulmonaire
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53 Traitement chirurgical des tétralogies de Fallot Fallot + Agénésie des valves ou petites branches : «Monocusp»
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55 Homogreffe pulmonaire
56 Tube Contegra - Venpro
57 Labcor VenPro
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62 Valves pulmonaires Valve pulmonaire «Melody» Valve injectable «Shelhigh» Schreiber. J Thorac Cardiovasc Surg 2006
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68 3D Knowledge system
69 Capelli et al. 2011
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72 Voltage and pace mapping of ventricular tachycardia in tetralogy of Fallot Khairy P, Stevenson WG. Catheter ablation in tetralogy of Fallot. Heart Rhythm 2009;6(7):
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74 Tetralogy of Fallot with absent pulmonary valve
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80 Tetralogy of Fallot-Absent pulmonary valve: repair
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82 Tetralogy of Fallot-Absent pulmonary valve: repair With Lecompte manoeuver
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84 «Tetralogy of Fallot» Thank you
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