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FONTAN PROCEDURE
DR JULIETH NACHONE KABIRIGI : PEDIATRIC CARDIOLOGIST 20TH FEBRUARY 2024
CATHOLIC UNIVERSITY OF HEALTH AND ALLIED SCIENCES,MWANZA, TANZANIA
BUGANDO MEDICAL CENTRE, MWANZA, TANZANIA
HIGHLIGHTS
• OVERVIEW OF FONTAN PROCEDURE
• CLINICAL SIGNIFICANCY
• INDICATIONS
• PRINCIPLES OF MANAGEMENT
• DRIVING FORCES
• FONTAN CIRCULATION
• COMPLICATIONS AND ITS MANAGEMENT
FONTAN
• Is a palliative 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
CLINICAL SIGNIFICANCE
• Previously around 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
INDICATIONS OF THE SINGLE VENTRICLE
MANAGEMENT
EXAMPLES OF SV PHYSIOLOGY
SINGLE VEMTRICLE PHYSIOLOGY LESIONS
MORE EXAMPLES
PRINCIPLES OF MANAGEMENT OF SV
GLENN SHUNT
• Direct blood 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
CAUSES OF CYANOSIS AFTER 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
DRIVING FORCES
• Normal individual 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
PRINCIPLES OF FONTAN CIRCULATION
• 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
1. PROTECT PULMONARY CIRCULATION
• Pulmonary stenosis vulvular and subvulvular
• Restrictive VSD (PA from Hyperplastic ventricle)
• PA Banding
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?
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
HYPOPLASTIC LEFT HEART SYNDROME
• Severe MS/Mitral atresia
• Restrictive VSD
• Dominant RV
• PDA supply Ascending and
descending Aorta
• WHAT are factors are aligned with
our principles of management?
NORWOOD PROCEDURE
FONTAN PHYSIOLOGY
• No mixing except for fenestration
• Circulation in series
• Decrease volume overload
• Increase afterload
• Systemic venous congestion
FONTAN CIRCULATION
ESSENCE OF FONTAN CIRCULATION
• 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
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)
COMPONENTS FAVORING FONTAN
TEN COMMANDMENTS CURRENT ONE
• Low Pulmonary vascular resistance
(PVR)
• Normal systolic function
• Lack of AV Valve insufficiency
FACTORS ASSOCIATED WITH POOR OUTCOME
• Increase Meam PAP >15mmHg
• Increase PVR >2
• Systemic ventricular dysfunction
• AV valve regurgitation
HOW TO IMAGE FONTAN 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
FENETRATION (TOTAL CAVAL PULMONARY
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
FOLLOW UP COMPLICATIONS
1.CARDIAC TYPE
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
LYMPHATIC ABNOMALITIES
• Due to 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
FONTAN AND LIVER PROBLEM
• 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
• THANKS

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JULIETH KABIRIGI,PAED.CARDIOLOGIST-CUHAS

  • 1. FONTAN PROCEDURE DR JULIETH NACHONE KABIRIGI : PEDIATRIC CARDIOLOGIST 20TH FEBRUARY 2024 CATHOLIC UNIVERSITY OF HEALTH AND ALLIED SCIENCES,MWANZA, TANZANIA BUGANDO MEDICAL CENTRE, MWANZA, TANZANIA
  • 2. HIGHLIGHTS • OVERVIEW OF FONTAN PROCEDURE • CLINICAL SIGNIFICANCY • INDICATIONS • PRINCIPLES OF MANAGEMENT • DRIVING FORCES • FONTAN CIRCULATION • COMPLICATIONS AND ITS MANAGEMENT
  • 3. FONTAN • Is a palliative 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 • Previously around 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 THE SINGLE VENTRICLE MANAGEMENT
  • 6. EXAMPLES OF SV PHYSIOLOGY
  • 10. GLENN SHUNT • Direct blood 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 CYANOSIS AFTER 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 • Normal individual 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 FONTAN CIRCULATION • 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 PULMONARY CIRCULATION • 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 HEART SYNDROME • Severe MS/Mitral atresia • Restrictive VSD • Dominant RV • PDA supply Ascending and descending Aorta • WHAT are factors are aligned with our principles of management?
  • 19. FONTAN PHYSIOLOGY • No mixing except for fenestration • Circulation in series • Decrease volume overload • Increase afterload • Systemic venous congestion
  • 21. ESSENCE OF FONTAN CIRCULATION • 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 TEN COMMANDMENTS CURRENT ONE • Low Pulmonary vascular resistance (PVR) • Normal systolic function • Lack of AV Valve insufficiency
  • 24. FACTORS ASSOCIATED WITH POOR OUTCOME • Increase Meam PAP >15mmHg • Increase PVR >2 • Systemic ventricular dysfunction • AV valve regurgitation
  • 25. HOW TO IMAGE FONTAN 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 CAVAL PULMONARY 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.CARDIAC TYPE 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 • Due to 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 LIVER PROBLEM • 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

Editor's Notes

  1. 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
  2. QN? Systemic outflow obstruction, LOCS, PAP,Pulmonar venous HTN,Restrictive VSD,Circulation is not align with Systemic circulation
  3. Bacause all desaturated blood goes to the lungs, QP:QS is 100%
  4. Thrombosis us MRI, use WARFARIN, HOWEVER LARGE THROMBUS IS FATAL REGARDLESS OF INTERVENTION, may nee EMBOLOCTOMY