Circulations de Fontan: un equilibre précaire?
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1 Martinique 2015 Session Fontan Circulations de Fontan: un equilibre précaire? Marc Gewillig, MD, PhD Louvain, Belgique
2 Normal biventricular circulation Ao 0utput % RA pressure low PA pulsatility LV S LV Ao sat > 95% PA RV P RA LA Gewillig TCS 2000
3 Fontan circulation: new portal system V Ao V 0utput (50) % CV pressure high PA pulsatility Ao sat (80-)93% CV PA LA Portal system : dam congestion output continuous decreased Gewillig TCS 2000
4 Effect of a dam Upstream : en amont congestion Dam use: continuous flow inertion, non-pulsatile Downstream en aval controlled & decreased flow
5 Exercise & CO: Nl vs Fontan circulation N EF 70% Cardiac Output % of nl baseline Better Fontan Poor Fontan Exercise level Gewillig ICVTS 2010
6 Fontan : progressive undoing Glenn Fontan Systemic Output % of nl saturation % Good Fontan Bad Fontan 40 CVP mmhg Fenestration size % of conduit Gewillig Goldberg 2014
7 The Fontan dilemma Normal biventricular No compromise Fontan circulation Compromise And full saturation And full output And no congestion Or good saturation Or good output & only moderate congestion f Ω of neo-portal Fontan
8 Management of Fontan failure low output Fontan portal connection congestion Fontan portal connection Neutralise? Ao V suction < -10 mmhg V V Heart? Keep LAP low, but Deprived for BSA Deprived for size : overgrown ² CV PA Neutralise : no Attenuate : very little?!? LA Aggravate : yes Acute little - no suction Chronic compliance
9 Fontan CO : EF - PVR 100 normal LV Circulatory Output % of nl at rest F PVR 1.5 F PVR 2.5 F PVR Theoretical concept - Experience based - To be refined EF of UVH % Gewillig ICVTS 2010
10 Fontan CO : EF - PVR 100 normal LV Circulatory Output % of nl at rest F UVH 70% F UVH 50% F UVH 30% Theoretical concept - Experience based - To be refined PVR Gewillig ICVTS 2010
11 Fontan : evolution of PVR 5? Medication? 4 PVR 3 normal age Gewillig ICVTS 2010
12 Fontan: effect of damming by portal system Primary effects Upstream: congestion Lung: low flow with inertia (continuous non-pulsatile) Downstream: decreased flow Secundary effects Congestion Oedema, ascites Lymphatic system: PLE, bronchial cast Liver fibrosis, cirrosis, CA PVR (low flow, no pulsatility, absence high flow high pressure, vasoactors) Ventricle Systole: contraction Diastole: suction compliance filling pressure SVR ventriculo-arterial coupling Collaterals : veno-venous, arterio-arterial Humoral : homeostatic reactions
13 UVH : a ventricle with a history Dilated? Decrease preload Decrease afterload Increase contractility Collapsed? Increase preload Afterload required Contractility: Frank-Starling failing pressure/volume loop Normal LV UVH fetal UVH shunted UVH Fontan
14 Ventricular compliance: effect chronic preload 20 15? Medication? normal acute overload V EP mmhg chronic overload chronic excessive overload acute underload chronic underload overgrown Preload % of nl Gewillig ICVTS 2010
15 UVH: pulmonary flow < growth PA normal PA Growth f flow : 4 phases shunt PA in UVH Fetus Initial palliation After PCPC - Glenn After TCPC - Fontan 100% Glenn Fontan Flow after Fontan birth Chronic low flow No pulsatility, very steady Absence episodes high flow pressure Inhomogenous distribution hepatic factor X Collateral flow PVR
16 Fontan circulation : after 45 years 1980 s Heart failure Congestion Low output Fontan ventricle Black box : Big cavity, thick wall Dysfunctional S & D No control on Output Congestion Circulation failure Fontan portal system Congestion Low output Ventricle can aggravate Ventricle: 2 key issues previously overloaded Overgrown Overdilation < damage Now chronicly deprived No preload reserve No control
17 Neo-portal Fontan system Connection : TCPC No gradient Minimal turbulence & flow collision; hepatic X-factor to both lungs Lungs PVR as low as possible; capacitance; recruitment Reasons increased PVR: Hypoplasia, stenosis, kink, loss of segments PVD (micro)thrombi PV stenosis Collateral flow Vasoconstriction : primary vs secundary
18 Neo-portal Fontan system Increase transpulmonary flow Optimise Fontan connection : reconvert, streamline Dilate stent PA stenosis, occlude collaterals Dilate stent PV «stenosis» compression Pulmonary vasodilators : Mitchell 2004 at HTX: PVR WU/m 2!!! Oxygen NO Ca blocker Prostacyclin Endothelin A antagonist altitude Sildenafil Treprostinil, inhaled Iloprost Limited effect!! Bosentan, Sitaxsentan, Ambrisentan Anticoagulation Ventricular suction enhance?? Very limited effect!!
19 Fontan failure : management Prevention : avoid pulmonary artery hypoplasia Prevention : avoid PA distortion, kink, loss Prevention : avoid PVD Prevention : avoid excessive ventricular overload Prevention :.. Prevention : avoid energy loss in CPC Symptomatic : decrease congestion Curative : decrease PVR: goal % ; current 0-10% Curative : decrease LAP: goal - 10 mmhg ; current 0 (- 2) Bypass Fontan : fenestration Assist - Replace
20 The search for excellence Haute cuisine kitchen Choose products Timing Prepare: filer, ripening, fermentation, marinating, mellow, paner, glacer, larder, mariner, degorger ; cuire, blancher, fondu, friter, flamber, pocher, papillotte, poeller, rotir, griller, barder, gratiner, braiser, sauter, fumer, deglacer, sous vapeur Measure t Adapt temperature, time of cooking Taste Salt, pepper, herbs, spice Avoid failure; failure no option No need for sauce mayonaise ketchup Congenital heart disease Born ; no choice 1st palliation Accept wide variety of result No little control Glenn at standard time No mortality, only excessive cyanosis Fontan at standard time Await result ; failure is an option Search for medication
21 UVH : Pulmonary flow Volume load shunt Medication?? normal PA PA in UVH PA hypoplasia 100% Glenn Fontan birth time Gewillig ICVTS 2010
22 Management of UVH to Fontan Decisions, in order of importance: 1. Initial palliation: quality of procedure PA growth 2. Timing of Glenn: stops catch-up growth PA s Consider second wind, mixed Glenn
23 UVH : Pulmonary flow Volume load shunt normal PA PA in UVH PA hypoplasia PA hypoplasia Shunt 100% Glenn Fontan birth time Gewillig ICVTS 2010
24 Stretch GT stent expansion: bench test 3.5 mm GT < 4.5 stented graft 4.0 mm GT < 5.5 stented graft Brown, Gewillig ICC 2010
25 Growth of PA in PA-IVS Neonatal 1630 gr R/ 3.0 mm Laks shunt At 4m 4.000g R/ stent expansion 4.0 mm At 8m R/ Glenn
26 HLHS, 3 years pre-fontan Status PO Norwood 3.0 central shunt; PO Glenn Courtesy Y d Udekem
27 EB, HLHS, 3 years pre-fontan 6 mm GT mbt shunt, clip on central PA Courtesy Y d Udekem
28 EB 1 year post-fontan Le PA reconstructed with 12 GT Courtesy Y d Udekem
29 The Fontan circulation The Fontan circuit after completion runs on autopilot ; it allows very little modulation Management of failure: Better to stay out of trouble Then to get out of trouble Excell in getting optimal building blocks Optimal ventricle Optimal pulmonary circulation requirements volume load : divergent!!! current strategies overprotect the ventricle & neglect growth of PA s
30
31 Martinique 2015 Session Fontan Circulations de Fontan: un equilibre précaire? Marc Gewillig, MD, PhD Louvain, Belgique
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