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Pinnacle Point from the top of St. Bartolome

Pinnacle Point from the top of St. Bartolome. Need for a Method to Deal with Patients with a Single Ventricle. Fontan Operation Suitable Lesions. Tricuspid atresia Single ventricle Unbalanced AVSD Hypoplastic left heart Hypoplastic right heart. RESTORES OXYGENATION

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Pinnacle Point from the top of St. Bartolome

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  1. Pinnacle Point from the top of St. Bartolome

  2. Need for a Method to Deal with Patients with a Single Ventricle

  3. Fontan OperationSuitable Lesions • Tricuspid atresia • Single ventricle • Unbalanced AVSD • Hypoplastic left heart • Hypoplastic right heart

  4. RESTORES OXYGENATION FOR A LARGE GROUP OF PATIENTS WITH “UNCORRECTABLE” COMPLEX DEFECTS

  5. Marine Iguana, late afternoon by the sea

  6. Aristotle and Galen—Misinterpreted the circulation but recognized the importance of both ventricles of the heart. • Colombo and Harvey—Established that blood propelled by the heart through two separate in-series circuits. • Starr (1942)– Cauterized the right ventricle in dogs and found only minimal increase in systemic venous pressures.

  7. Robard & Wagner (1948) – Anastamosed right atrium to main pulmonary artery, ligating the MPA proximal to the anastamosis, in dogs. • Carlon (1951), Padova, Italy – Anastamosed the azygos vein to the RPA in dogs. One/five survived. • Robicsek (Hungary, 1950’s) – May experiments with the anastamosis of the vena cavae to the pulmonary circuit. • Glenn (1957) – first cavopulmonary shyunt in a child with congenital heart disease.

  8. Glenn (Robicsek) Shunt

  9. Blue footed boobies

  10. Francis Fontan (1968, Bordeaux, France– In a 12 year old girl with tricuspid atresia placed a cavopulmonary shunt to the RPA(Glenn), inserted an aortic valve allograft between the right atrium and the main pulmonary artery, closed the atrial septal defect and sutured a pulmonary valve allograft into the IVC-right atrial junction.

  11. Fontan Operations • A family of related operations which are used in many types of congenital heart disease • Originally described for use in patients with tricuspid atresia by Fontan and Baudet in 1971 • Many revisions have been made, so today’s operations bear little resemblance to the original

  12. Fontan Physiology • All Fontan operations separate the pulmonary and systemic circulations • Patients with a completed Fontan have one functioning ventricle, the systemic pump • The Fontan is the last stage of the reconstruction of the univentricular heart, so the patient will be cyanotic until completion

  13. Conduits caused late morbidity and it became apparent that the “atrial contraction” was not essential confirming the earlier work of Robicsek. • Kreutzer (1973) – Anastamosis of the Right Atrium directly to the Pulmonary Artery.

  14. Fontan OperationHemi-Fontan/Classic Fontan

  15. Hemi-Fontan/FontanSurgical Technique • The right atrial appendage is sutured to the undersurface of the RPA • A patch is placed to exclude the IVC flow from the appendage/RPA connection • To complete the Fontan, the patch is removed

  16. Hemi-Fontan/FontanAdvantages • Smaller atrial suture lines • Flow patterns may be smoother, minimizing thrombus formation

  17. Fontan OperationLateral Tunnel

  18. Lateral TunnelSurgical Technique • A 4 mm circular punch is used on a dacron patch to make a hole • The flow entering the RA from the IVC is baffled to the stump of the SVC using the dacron sutured along the lateral wall of the RA • The SVC stump is connected to the undersurface of the RPA

  19. Lateral TunnelAdvantages • The use of the dacron baffle prevents the venous atrium from becoming distended • The fenestration allows for a pressure pop-off, minimizing post-op complications

  20. Fontan Fenestration • Since the early 90’s, many centers put hole is their venous baffles to allow for a pressure pop-off • Fenestrations minimize post-op complications • Patients remain mildly cyanotic • Fenestrations can be closed in the cath lab using devices

  21. Fontan OperationsExtracardiac Baffle

  22. Extracardiac BaffleSurgical Technique • A 4 mm circular punch is used on a 20 mm dacron tube to make a hole • The dacron tube is sutured to the IVC and passed lateral to the RA • The tube graft is sutured to the undersurface of the RPA • A hole is made in the wall of the RA and aligned with the hole in the tube

  23. Extracardiac BaffleAdvantages • Since the tube graft is extracardiac, the use of sutures in the RA is minimized • Fewer RA suture lines may reduce the incidence of atrial arrhythmias

  24. Criteria of Choussat • Age at operation, 4-15 yrs. • Sinus rhythm • Normal systemic venous return • Normal right atrial volume • Mean pulmonary artery pressure, < 15mm Hg. • Pulmonary arterial resistences < 4 units/m2 • Pulmonary artery/aorta diameter ratio > 0.75 • Left ventricular ejection fraction > 0.60 • Competent mitral valve • Absence of pulmonary artery distortion

  25. Although the criteria of Choussat and Fontan are guidelines to low-risk Fontan operation and the operation can be done in the absence of some of these criteria, operative mortality and morbidity increase as more and more of these criteria are violated.

  26. Fontan OperationsPostoperative Complications • Pleural and pericardial effusions • Chylothorax • Hepatic congestion and dysfunction • Arrhythmias • Atrial distension

  27. Initially major complications were pleural and pericardial effusions. • Fontan anastamoses underwent a number of revisions. deLaval et al. • Finally, most have resorted to a two staged procedure with fenestration. • Now extracardiac conduits are in vogue.

  28. Red-footed Booby

  29. Driscoll et al. 1992- Mayo Clinic • 5 – 15 yr. Follow up • 352 patients (215 survivors) • Mortality • 30 days - 84% • 1 year - 77% • 5 year - 70% • 15 year - 60%

  30. Driscoll et al. 1992- Mayo Clinic Predictors of Mortality • Fulfilled all of Choussat’s Criteria • 5 year - 87% • 10 year - 71% • Violated one or more • 5 year - 68% • 10 year - 58% Most Important were • Elevated RA pressure • Elevated PA pressure • Decrease LV function • AV valve insufficiency

  31. Driscoll et al. 1992- Mayo Clinic Symptoms • Easy fatigue 36% • Shortness of breath 30% • Palpitations 28% • Rapid heart rate 27% • Nausea or vomiting after exercise 5-10%

  32. Large land tortoise, Isla Santa Cruz Galapagos

  33. Driscoll et al. 1992- Mayo Clinic Exercise (physical education – 140 students) • 21% participate fully • 33% less than fully • 36% do not participate

  34. Driscoll et al. 1992- Mayo Clinic% Survival

  35. Masked Booby

  36. Driscoll et al. 1992- Mayo Clinic Employment • 80 patients employed • 52 full time • 28 part time (4 because of heart problems) • 50 unemployed • 11 because of heart problems

  37. Driscoll et al. 1992- Mayo Clinic Fertility after the Fontan • Three pregnancies before the Fontan • All three aborted (1 spont.; 2 therap.) • Seven pregnancies after Fontan • 3 spont. abortions • 3 therap. Abortions • 1 liveborn child

  38. Pregnancy Outcomes After Fontan RepairCanobbio, MM et al.JACC 28:763-7, 1996 • 126 female patients after Fontan (Mayo) • 33 pregnancies • 15 (45%) live births (14 mothers) • 13 spontaneous abortions • 5 elective terminations • Complications • Atrial flutter (1) • Decreased LV fx., AI, A-V valve regurg. in another

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