tetralogia di fallot

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

•  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)

Tetralogy of Fallot A single anomaly ? (when regular….)

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)

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

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

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

Palliative / complete repair

Palliative repair RVOT patch:

• (CEC) aneurysm ?

Potts / Waterston :

• dilatation / stenosis / distortion of PAs, PAH

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

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

Postoperative problems WHAT WAS KNOWN

Complete repair •  Re-stenosis of RVOT •  Dilatation of RVOT •  PV regurgitation (native or artificial) •  PA stenoses •  MAPCAs •  AR •  VSD

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

Strain !

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

Rythm!

• Sudden death

•  AVB

•  VT

•  Atrial arrhythmias

•  PM, ICD, resincronization….

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…

Medical treatment

• PREVENTION • ETIOLOGICAL TREATMENT? • TREATMENT OF SYMPTOMS? (antyarrhythmics...) • ACCURATE FOLLOW-UP ! • Clinical evaluation, ECG, Holter, ECO, (2D, 3D, TDI...), VO2, MRI

•  Restenosis of RVOT Dilatation - stent

•  Stenosis of PAs Dilatation - stent

•  Regurgitation/stenosis of PV PVR

•  MAPCAs Embolisation

•  Residual VSD Occlusive devices

•  AR Percutaneous valves ?

Postoperative Fallot What we should look at

•  Pulmonary arteries •  Pulmonary valve •  Infundibular region •  Right ventricle •  Left ventricle •  Interventricular septum •  Aorta

Postoperative Fallot What we should look at

•  Pulmonary arteries : hypoplasia

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

Postoperative Fallot What we should look at

•  Pulmonary arteries: coarctation of LPA

Postoperative Fallot •  Pulmonary arteries: ipo LPA (stent)

Postoperative Fallot What we should look at

but it is too late…

•  Pulmonary arteries: Single RPA…

Postoperative Fallot What we should look at

but it is too late…

•  Pulmonary arteries: Single LPA

Postoperative Fallot •  Pulmonary arteries: kissing Stent

Postoperative Fallot What we should look at

•  Pulmonary arteries: Stent Which weight? Redilatation, Fracture, restenosis…

Stent-related diseases….

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…

Postoperative Fallot What we should look at

•  Pulmonary arteries •  Pulmonary valve •  Infundibular region •  Right ventricle •  Left ventricle •  Interventricular septum •  Aorta

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

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

LVEF after PVR

• PVR may improve LV function Tex Heart Inst J 2011

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

PVR: HOW ? • Surgical (biological, mechanical, injectable…) • Percutaneous? (Melody, Sapien, new valves...)

Postoperative Fallot

•  Pulmonary valve: incompetence

Postoperative Fallot •  Pulmonary valve: incompetence

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

Postoperative Fallot •  Pulmonary valve: stenosis

Postoperative Fallot •  Acquired atresia of the PV

RFA perforation

Postoperative Fallot What we should look at

•  Pulmonary arteries •  Pulmonary valve •  Infundibular region •  Right ventricle •  Left ventricle •  Interventricular septum •  Aorta

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!

Postoperative Fallot •  Infundibulum

Postoperative Fallot •  Infundibulum

Postoperative Fallot What we should look at

•  Pulmonary arteries •  Pulmonary valve •  Infundibular region •  Right ventricle •  Left ventricle •  Interventricular septum •  Aorta

Postoperative Fallot

•  RV/ LV !!!!!

Postoperative Fallot

•  RV/ LV !!!!!

Postoperative Fallot •  RV/ LV !!!!!

Postoperative (percutaneous) Fallot

•  RV/LV !!!!!

Postoperative Fallot What we should look at

•  Pulmonary arteries •  Pulmonary valve •  Infundibular region •  Right ventricle •  Left ventricle •  Interventricular septum •  Aorta

Occluding devices To close a communication I

want to stent it….

Residual VSD

Devices and ideas

How shall I close a hole? Generally putting something

inside....or simply covering it.... cover

occlude anchorage

Devices and ideas

Postoperative Fallot What we should look at

•  Pulmonary arteries •  Pulmonary valve •  Infundibular region •  Right ventricle •  Left ventricle •  Interventricular septum •  Aorta

Aortic root

• Progressive aneurismal dilatation and AR in 15% of patients • Normalisation of initially dilated aortic root (Eur J Cardiothor Surg 2010) … • Early repair !

Postoperative Fallot •  Other problems: collaterals (overload, bleeding…) •  Embolisations: coils, particles, glue, devices…..

Il Fallot e’ una malattia che guarisce o una malattia cronica ?

Per chi voglia dedicarsi ad attività piu’ redditizie e meno rischiose…

Riconoscere una crisi anossica, conoscere l’echo