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Fontan Circulation

Fontan Circulation. Nick Collins July 12 2011. Why?. Congenital Heart Disease Too much is never enough Champagne EP Echo for the discerning Beyond EF…. High end structural interventions Haematology Respiratory medicine Phil Roberts Context Jeff Pretto ASD Fenestrations….

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Fontan Circulation

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  1. Fontan Circulation Nick Collins July 12 2011

  2. Why? • Congenital Heart Disease • Too much is never enough • Champagne EP • Echo for the discerning • Beyond EF…. • High end structural interventions • Haematology • Respiratory medicine • Phil Roberts • Context • Jeff Pretto • ASD • Fenestrations….

  3. Unfamiliar • Outline • Questions • Not too many slides…. • Garry…. • Fontan in principle • Rationale • Late issues • Management • Imaging • Cross sectional

  4. If Nothing Else…. • Palliative operation for functionally single/uni ventricular heart • Not perfect repair • 70% survival at 25 years • Prone to late complications • Case to illustrate

  5. Fontan • Single ventricle • Underlying congenital heart disease varies • Typically hypoplastic right ventricle • Morphologic LV supporting circulation • HLHS • End result of two/three operations • Initial description tricuspid atresia • Others • No subpulmonary ventricle • Creates a circulation in series, rather than parallel • Passive blood flow into pulmonary circulation • Illustrate

  6. Passive Filling • Requires elevated venous pressure • Certain requirements • Requires pressure gradient for filling (ie cannot rely on RV systolic function) • Low systemic ventricle EDP • No AV valve regurgitation • No outflow tract obstruction • Low PA pressure/PVR • Fenestration • Any condition affecting PVR • Chest infection • PE • Sleep disordered breathing

  7. Pulmonary Vascular Bed • Low flow • Not pulsatile • Thrombogenic • Need for aspirin/warfarin • Contraception • Pregnancy

  8. Passive Filling • Requires elevated venous pressure • Certain limitations • Hypovolaemia • Diuretic therapy • Elevated JVP is essential! • Laparoscopic surgery • Diminished venous return

  9. Early Iterations • Dilated right atrium • Prone to atrialarrythmia • Universal problem • Warfarin • More physiological versions • Bypass the right heart • Two methods • Lateral tunnel • Incorporates right atrial tissue • Extra cardiac

  10. ASD vs Fenestration • Single ventricle • Functionally single atria • Surgically created ASD • Fenestration • Communication between atria and Fontan circuit • Concern regarding PVR • Cyanosis vs venous congestion • Concern regarding systolic function • Prevent elevated EDP

  11. Any Problems….. • Universal to ACHD • Highlight specific • Arrhythmia • 50% after 20 years • Poorly tolerated – ventricular dysfunction • Atrial tachycardia • IART • AF/atrial flutter • Medical therapy • Young… • Ablation • Expand…. • Fontan conversion • Early iterations • Lateral tunnel/extra-cardiac • Arrhythmia surgery

  12. Arrhythmia • Bradycardia • Medication side effects • Implications “newer” generations • Unable to access atrial tissue • Unable to position leads into right/subpulmonary ventricle • Epicardial systems • Anticoagulation

  13. Other Late Problems… • Introduce case • 17 year old male • Previously followed CHW • “Adult” • Functional single ventricle • Double inlet left ventricle • TGA • VSD • Pulmonary stenosis

  14. Blalock Taussig shunt • Glenn shunts (Bilateral SVC) • Aim to preserve pulmonary blood flow • Fontan completion age 4 • Since well • No therapy • Asymptomatic

  15. Baseline Assessment • ECG • Torment registrars • Echocardiography • Exercise stress test • Pathology • Hb 185-200 • Normal albumin

  16. Echocardiography and the Fontan • Difficult • Ability to identify • IVC/SVC • Phasic flow • Outflow tract • Anastamosis PA and Aorta (DKS) • Pulmonary vessels • Critical – cross sectional imaging • Serial assessment • EF • Tissue Doppler • Dp/Dt • No reference ranges/nomograms

  17. Stage 1 CP Test

  18. Summarise • Stable effort tolerance • Reduced compared to peers • Hypoxaemia at rest/exercise • Secondary erythrocytosis

  19. Exercise capacity in adults with CHD- Impact of underlying diagnosis Mean ± SD Aortic coarction 28.7 ± 10.4 Tetralogy of Fallot 25.5 ± 9.1 VSD 23.4 ± 8.9 Mustard-operation 23.3 ± 7.4 Valvular disease 22.7 ± 7.6 Ebsteins anomaly 20.8 ± 4.2 Pulmonary atresia 20.1 ± 6.5 Fontan-operation 19.8 ± 5.8 ASD (late closure) 19.2 ± 6.2 ccTGA 18.6 ± 6.9 Complex anatomy 14.6 ± 4.7 Eisenmenger 11.5 ± 3.6 ANOVA p<0.0001 5 10 15 20 25 30 35 40 Peak VO2 (ml/kg/min) Diller GP, et al.Circulation 2005; 112:828-35.

  20. Late Complications • Hypoxaemia • Residual fenestration • Importance operation report • TOE/Cath/MRI • Collateral formation • Porto-systemic collaterals • High filling pressures • Pulmonary AV fistulae • Initial Fontan • Classic Glenn shunt • RA to right PA anastamosis

  21. All Downhill…. • Left sided pleuritic chest pain • No previous episodes • No infective symptoms • No cough/haemoptysis • Unchanged effort capacity • Unchanged ECG

  22. Fontan and PE • Varma et al JACC 2003 Jun 18; 41(12):2252-8 • TCCCA • 17% PE on V/Q – CTPA • None on warfarin • Potentially critical in “attrition” • Increased pulmonary resistance

  23. Fontan and PE • Warfarin • Protective in context atrial arrhythmia • Predisposes to anaemia • Warfarin/Fontan/Renal impairment • Patients with univentricular/systemic right ventricles • Difficult to evaluate • Elevated baseline Hb

  24. Additional Late Complications • Arrhythmia • Ventricular dysfunction • Multiple contributing mechanisms • Strong association with: • Symptoms • Clinical heart failure in 40% Fontan • Piran et al Circulation 2002: 105:1189-1194 • Objective exercise capacity • Arrhythmia risk • Mortality • Protein Losing Enteropathy (PLE)

  25. PLE • Most problematic • Progressive hypoproteinaemia • Aetiology unclear • Possible venous congestion • Incidence 1-15% • High mortality rate • No successful treatment options • Medical • Surgical • Transplant • Higher mortality compared to other CHD

  26. Back to business….. • Progressively fatigued • Difficulty in attending University • Headache • Role of increased Hb • Underwent venesection • Transient improvement • Cyanosis/effort tolerance • Cardiac Catheter

  27. Cardiac Catheterisation

  28. Closure right to left shunt • Fenestration alone • Amplatzer type device • More diffuse • Covered stent • Booked August….

  29. Summarise • Fontan • Final common surgical pathway for single ventricle physiology • Imperfect repair • Life long follow up • Life expectancy • Transplant • Late complications • Arrhythmia • Ventricular dysfunction

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