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JULIETH KABIRIGI,PAED.CARDIOLOGIST-CUHAS
- 1. FONTAN PROCEDURE DR JULIETHNACHONE KABIRIGI : PEDIATRIC CARDIOLOGIST 20TH FEBRUARY 2024 CATHOLIC UNIVERSITY OF HEALTH AND ALLIED SCIENCES,MWANZA, TANZANIA BUGANDO MEDICAL CENTRE, MWANZA, TANZANIA
- 2. HIGHLIGHTS • OVERVIEW OFFONTAN PROCEDURE • CLINICAL SIGNIFICANCY • INDICATIONS • PRINCIPLES OF MANAGEMENT • DRIVING FORCES • FONTAN CIRCULATION • COMPLICATIONS AND ITS MANAGEMENT
- 3. FONTAN • Is apalliative treatment option for the functional or morphologic univentricular Congenital heart disease • Is a total cavopulmonary connection as the systemic venous blood by pass the heart and goes direct to the lungs • There are multiple procedures before fontan • Pulmonary artery banding or BT- shunt • Glenn shunt • Then Fontan
- 4. CLINICAL SIGNIFICANCE • Previouslyaround 1940s , single ventricle were considered fatal • But currently there about 50,000- 70,000 people are living with Fontan worldwide1 • Thirty year survive is > 80% at 30years • 95% of free transplant survival at 5- 10years • 1. AHA 2019
- 5. INDICATIONS OF THESINGLE VENTRICLE MANAGEMENT
- 6. EXAMPLES OF SVPHYSIOLOGY
- 7. SINGLE VEMTRICLE PHYSIOLOGYLESIONS
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- 10. GLENN SHUNT • Directblood from Superior vena cava to Right Pulmonary artery • Overall survival is 90% post Glenn • Driving forces • 1.Gravitation force • 2. 6-9mo as the baby can be sit without support • After some years if the baby has desaturation continue to Fontan • Because the lower body grows fast than the upper body
- 11. CAUSES OF CYANOSISAFTER GLENN • Development of veno-venous circulation • Is the most important factor by development of collaterals on IVC and SVC • RA is 5cmHg where SVC and SVC connected with the same low pressure • When SVC is connected to RPA after quiet some time few years they dev cyanosis • The collaterals develop from IVC to SVC following the development of High pressure in the connected PAs • After 7years of Age ,the lower body develop faster than the upper body hence more blood from IVC than SVC as a result of more deoxygenated blood to the Systemic compare to SVC blood to PA • Solution to severe cyanosis is to connect IVC to PA and then is FONTAN, • Sepateted circulation no mixing SO2 100% • QP:QS=1
- 12. DRIVING FORCES • Normalindividual venous return to the heart is depend on • Negative intrathoracic pressure • RV contractility and relaxation, such blood from systemic veins • Gravity • Muscle pump • That’s why we do Glenn at least from 6-9mo when the baby is able to sit, to allow flow from SVC to RPA by gravitation drive • With time after 7years of age, these children need to perform Fontan procedure because of increase cyanosis after prolong exposure of SVC to PAP which is 3x the SVC • So all blood bypass the lungs and and go to IVC, at this point we need FONTAN to direct all of the systemic venous blood to PAs
- 13. PRINCIPLES OF FONTANCIRCULATION • Low PVR (is single and most important factor is to avoid PHTN • Example Tricuspid atresia type IC,IIC, Unrestrictive VSD, Large PDA, Obstructed TAPVR, Severe Aortic regurgitation, • Single ventricle to systemic circulation • Make sure that no obstruction to systemic outflow obstruction • Good size PAs branches no obstruction
- 14. 1. PROTECT PULMONARYCIRCULATION • Pulmonary stenosis vulvular and subvulvular • Restrictive VSD (PA from Hyperplastic ventricle) • PA Banding
- 15. 2.SYSTEMIC OUTFLOW OBSTRUCTION •Aortic stenosis (valvular or subvulvular) • Restrictive VSD (Aorta from hypoplastic chamber) • Coarctation/Hypoplastic arch • QN? Do you wish to do PAB in this patient? • Management: DAMUS KAYE PROCEDURE+BT-Shunt • WHY Can we connect ascending aorta to the dominant Ventricle?
- 16. DAMUS KAYE STANSEL(DKS) • For the favor of Coronaries, you cant cut Ascending Aorta to the dominant Ventricle because you want to maintain coronary supply to the ventricles • DKS You connect the pulmonary root to the aorta to maintain Aortic circulation PLUS BTS • Later on proceed to GLENN
- 17. HYPOPLASTIC LEFT HEARTSYNDROME • Severe MS/Mitral atresia • Restrictive VSD • Dominant RV • PDA supply Ascending and descending Aorta • WHAT are factors are aligned with our principles of management?
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- 19. FONTAN PHYSIOLOGY • Nomixing except for fenestration • Circulation in series • Decrease volume overload • Increase afterload • Systemic venous congestion
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- 21. ESSENCE OF FONTANCIRCULATION • One functional single ventricle to systemic circulation • Systemic venous return flow without pumping ventricle to the lungs • No desaturated blood to the systemic circulation(SO2 is 100%) • Qp;Qs=1 • Is a PALLIATIVE PROCEDURE
- 22. FONTAN CIRCULATION PARADOXICAL •In normal circulation, Systemic venous pressure is 5-7mmHg • Mean Pulmonary arterial pressure of at least 15mmHg in order the pulmonary vascular network to be patent • PARADOXICAL IN FONTAN is the existence of 3x normal of systemic venous hypertension (15mmHg)
- 23. COMPONENTS FAVORING FONTAN TENCOMMANDMENTS CURRENT ONE • Low Pulmonary vascular resistance (PVR) • Normal systolic function • Lack of AV Valve insufficiency
- 24. FACTORS ASSOCIATED WITHPOOR OUTCOME • Increase Meam PAP >15mmHg • Increase PVR >2 • Systemic ventricular dysfunction • AV valve regurgitation
- 25. HOW TO IMAGEFONTAN PATIENTS • Know what type of Fontan repair done • Read the surgical reports • Anatomy of Fontan circulation • IVC and SVC driving force is inspiration • Tunnel (intra or extracardiac) • Connection with PA • PA Branches • Pulmonary veins
- 26. FENETRATION (TOTAL CAVALPULMONARY CIRCULATION (TCPC) • ADVANTAGE • Is beneficial in high risk fontan • Disadvantage is causing R-L shunt • More desaturation • Qp;Qs<1 • Reduces hospital stay, less re do
- 27. FOLLOW UP COMPLICATIONS 1.CARDIACTYPE Arrhythmia 2. EXTRACARDIAC TYPE Thromboembolic Lymphatic system PLE (13%) Plastic bronchitis LIVER FAILURE • 1,ATRIAL ARRHYTHMIA 40% • Is a life threatening complication from sustained arrhythmia • Tachycardia increase Pulmonary venous atrial pressure, reducing Pulmonary blood flow, reduce Cardiac output • Pre load reduction, failure of fontan • Rx Beta blocker Bisoprol, Amiodarone if is recurrent • EPS is under research
- 28. LYMPHATIC ABNOMALITIES • Dueto increase venous pressure, decrease and impaired thoracic duct drainage • PLE 13% Loss of protein in vascular bed • Massive edema, • Fat malabsorption • Hypocalcemia • Hypomagnesemia • Immunodeficiency • TREATMENT • Low fat diet • Low salt • High calories • Diuretics • Fenestration and HEART TRANSPLANT
- 29. FONTAN AND LIVERPROBLEM • Hepatocellular carcinoma • Is from chronic elevation of hepatic venous pressure lead into chronic liver pathology • The Guideline recommend the annual screening for Hepatic adenoma • Liver imaging • Alfa fetal protein
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Editor's Notes
- #5 This is basically depends on advances in surgical techniques Pre operative care Better patient selection But regardless of all these 50% of survivals have adverse events before reaching adulthood
- #18 QN? Systemic outflow obstruction, LOCS, PAP,Pulmonar venous HTN,Restrictive VSD,Circulation is not align with Systemic circulation
- #22 Bacause all desaturated blood goes to the lungs, QP:QS is 100%
- #28 Thrombosis us MRI, use WARFARIN, HOWEVER LARGE THROMBUS IS FATAL REGARDLESS OF INTERVENTION, may nee EMBOLOCTOMY