The Fontan circulation - Echocardiography 13 F Mei… · The essence of the Fontan circulation •...
Transcript of The Fontan circulation - Echocardiography 13 F Mei… · The essence of the Fontan circulation •...
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The Fontan circulation
Folkert Meijboom
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What to expect?
• Why a Fontan-circulation• Indications• How does it work
• Types of Fontan circulation• Historical overview
• Role of echocardiography
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What to expect?
• Why a Fontan-circulation• Indications• How does it work
• Types of Fontan circulation• Historical overview
• Role of echocardiography
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Why a Fontan circulation?
• No functional RV
• Volume overload LV
• systemic venous return plus pulmonary
venous return
• Desaturation systemic circulation - cyanosis
•How van this be solved?
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Why a Fontan circulation?
• No functional RV
• Volume overload LV
• systemic venous return plus pulmonary
venous return
• Desaturation systemic circulation - cyanosis
Not by a biventricular repair
Univentricular repair
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Indication for a Fontan
situations for which a biventricular is notpossible
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Indication for a Fontan
situations for which a biventricular is notpossible
• HLHS• TA• MA• DILV• Unbalanced AVSD• Ebstein• PA – intact IVS• etc
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The essence of the Fontan circulation
• only 1 functional ventricle systemic ventricle
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The essence of the Fontan circulation
• only 1 functional ventricle systemic ventricle
• no subpulmonary ventricle
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The essence of the Fontan circulation
• only 1 functional ventricle systemic ventricle
• no subpulmonary ventricle
• Systemic venous return flows, without a pumping
ventricle, into the lungs
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The essence of the Fontan circulation
• only 1 functional ventricle systemic ventricle
• no subpulmonary ventricle
• Systemic venous return flows, without a pumping
ventricle, into the lungs
• no desaturated blood in the systemic circulation
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The essence of the Fontan circulation
• only 1 functional ventricle systemic ventricle
• no subpulmonary ventricle
• Systemic venous return flows, without a pumping
ventricle, into the lungs
• no desaturated blood in the systemic circulation
• No longer a volume-overloaded LV
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How does it work?
How can an adequate pulmonary blood flow be
achieved?
1. elevated systemic venous pressure2. low pulmonary vascular resistance
3. Low LA pressures
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How does it work?
Flow
depends on
pressure difference
between
systemic venous pressureand
LA pressure
and
pulmonary vascular resistance
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Pressure difference VCS - LA
• Push & pull circulation:
• Elevated systemic venous pressure = push
• Periodic lowering of LA pressure• During systole pull• During rapid filling of ventricle
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systole
a
c v
x
y
LA pressure curve = the “pull”
© H.Hamer, AZG, Groningen
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Anatomy (history) of the Fontan-operation
© H.Hamer, AZG, Groningen
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“the” patient with a Fontan circulation
• Does not exist
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“the” Fontan circulation
• Mixed bag
• many different types of
• Intracardiac anatomy
• Type of surgical repair
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Echocardiography & Fontan
•Know what the underlying cardiac defect is
•Know what type of Fontan repair is done
•Understand the Fontan circulation
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Echocardiography & Fontan
•Know what the underlying cardiac defect is
•Know what type of Fontan repair is done
•Understand the Fontan circulation
• Read the surgical report!!
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Echocardiography & Fontan
•Know what the underlying cardiac defect is
•Know what type of Fontan repair is done
•Understand the Fontan circulation
• If you do not know where you are looking for, you may not find it
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Echocardiography & Fontan
• in univentricular hearts – before surgery –echo is important for:
• Intracardiac anatomy
• Ventricular function
• AV valve function
• Biventricular - univentricular repair
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Echocardiography & Fontan
• Univentricular hearts after Fontan• (Intracardiac anatomy)
• Ventricular function
• AV valve function
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Echocardiography & Fontan
• Univentricular hearts after Fontan• (Intracardiac anatomy)
• Ventricular function
• AV valve function
Anatomy & function of the Fontan circulation
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Echocardiography & Fontan
• Univentricular hearts after Fontan• (Intracardiac anatomy)
• Ventricular function
• AV valve function
Anatomy & function of the Fontan circulation
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ventricular function & Fontan
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ventricular & AV valve function
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ventricular & AV valve function
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ventricular & AV valve function
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AV valve function
• Assessment of AV valve regurgitation = very importantModerate to severe regurgitation• elevated LA pressure• decrease in transpulmonary gradient• decrease in transpulmonary flow• decrease in cardiac output• compensatory mechanism = fluid retention• elevated central venous pressure• transpulmonary gradient restored• cardiac output restored
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AV valve function
• Assessment of AV valve regurgitation = very importantModerate to severe regurgitation• elevated LA pressure• decrease in transpulmonary gradient• decrease in transpulmonary flow• decrease in cardiac output• compensatory mechanism = fluid retention• elevated central venous pressure• transpulmonary gradient restored• cardiac output restored
• To a certain limit decompensation
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Fontan & AV valve function
• Assessment of AV valve regurgitation = very important
• If one sees development of AV valve regurgitation• Alarm signs• Worsening ventricular function
• How to assess AV valve regurgitation: beyond the scope of this talk
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Fontan & ventricular function
• How do we assess & measure function of the systemic ventricle?
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Fontan & ventricular function
• EAE & ASE recommendations on chamber quantification• LV = systemic ventricle
• Standardized cross sections• 4Ch view• Parasternal long axis
• Normal values for sex and body size
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Fontan & ventricular function
• EAE & ASE recommendations on chamber quantification• Standardized cross sections???
• 4Ch view• Parasternal long axis
• Normal values for sex and body size
• But embrace the idea behind it:• Use reproducible cross section• Anatomic landmarks
• DILV ≠ HLFS ≠ unbalanced AVSD
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Fontan &ventricular function
• Eyeballing
• Quantify what you can quantify, in a reproducible way
• Essential for longitudinal follow-up• M-mode• 2D • 3D • Doppler (incl TDI)
not for comparsion withnormal values; patient as his/her own control
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2D & color Doppler
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Inflow
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Tissue Doppler
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Ejection time, Tei-index
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Relative duration systole in cardiac cycle(good functioning adult Fontan)
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duration systole, incl IVCT & IVRT(poorly functioning adult Fontan)
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duration systole – very short diastole(3 yr old with HLHS)
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SD ratio in the RV
Friedberg, J Am Soc Echocardiogr 2007;20:749
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duration systole – short diastole(21 yr old criss-cross, TCPC with PLE)
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CW dP/dt
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TAPSE
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PV flow pattern: very low S in absence MR
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systole
a
c v
x
y
LA pressure curve
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Echo & Doppler measurements
• Absolute values difficult to interpret
• Change over time/ in different conditions mayguide us:
• in clinical decision making
• When to send a patient to the cathlab
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Echo & Doppler measurements
• Absolute values difficult to interpret
• Change over time/ in different conditions mayguide us:
• in clinical decision making
• When to send a patient to the cathlab
• The essence of the Fontan circulation: V = P/R we cannot measure this with echo
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Visualisation of Fontananatomy
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Visualisation of Fontananatomy• Segmental approach
• Hepatic veins• VCI• Tunnel
• Intracardiac• Extracardiac
• RA• Connection with PA• VCS• Connection with PA• Pulmonary artery branches• Pulmonary veins
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Visualisation of Fontananatomy & function• Segmental approach
• Hepatic veins• VCI• Tunnel
• Intracardiac• Extracardiac
• RA• Connection with PA• VCS• Connection with PA• Pulmonary artery branches• Pulmonary veins
•Color Doppler patterns
•Pulsed Doppler flow patterns
•Shunts/leaks in the tunnel
•thrombi
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VCI & hepatic veins
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VCI pulsed Doppler: Scale! Low velocity filter! Respiration!
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VCI pulsed Doppler: Scale! Low velocity filter! Respiration!
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TCPC – driving force = inspirationno influence of cardiac cycle
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Connection VCI – extracardiac lateral tunnel
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Lateral, extracardiac tunnellook for thrombi
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Apical 4 Ch view intracardiac tunnel
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Intra-atrial tunnel
•Apical scan; upside down
•Intra-atrial tunnel
•initially a small diameter
•No PV obstruction
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Intra-atrial tunnel; adult age
•can dilate in time,
•become very wide
•can give PV obstruction
•Looks like RA – PA
•(different flow patterns)
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VCI Doppler in RA – PA conduiteffect of atrial contraction
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Tricuspid atresia; RA – AP connectie
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RA –PA conduit
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Atrio – ventricular connection
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Tricuspid atresia & RA – RV connectie
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RA – RV conduit with stenosis
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PA flow in RA – RV conduit:
Pulsatile systolic flow in PA!
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Doppler patterns & type of Fontan
• Doppler patterns in different types of connection are different• Doppler patterns in different patients with the same
type of connections are different
• registration of ECG – p-waves – and respiration curve
• Off-line studying on tracings: • What explains flow patterns?• What explains phasic changes?
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intracardiac tunnel VCI - PA
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Look for shunts/abnormal flows
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Baffle leakage with R – L shunt
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Baffle leakage with R – L shunt
Courtesy Jan Marek
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What to do with a baffle leak?
• Beyond the scope of this talk
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Bidirectional Glenn anastomosis
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VCS – Glenn from R supraclavicular
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VCS – Glenn from R supraclavicular
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Glenn anastomose
•VCS
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APL from SSN; almost always obtainable
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APL bij TCPC
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From SSN: LPA
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From SSN: LPA
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APL in LA – AP connection
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R upper pulmonary vein
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PV Doppler
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RUPV obstruction (RA – RV connection)
•AP4K
•PW
•Always look for the R pulmonary veins, especially if the IAS is bulging
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Dilated RA in RA-AP connectionbeware of R PV obstruction
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RUPV obstruction
Pulsed wave Doppler (“pull-back”)
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Conclusion
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Conclusion
• Why a Fontan-circulation• Indications• How does it work
• Types of Fontan circulation• Historical overview essential for the
understanding of echo& Doppler images
• Role of echocardiography in the follow-up• Measuring function in a reproducible way
for longitudinal follow up
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Conclusion
• Fontan & echo = difficult & fascinating
• You need to understand Fontan physiology, especially when you consider intervention• (elective redo of “old” RA- PA to TCPC)
• Echo is very helpful, but do not hesitate to send a patient to the cathlab when in doubt
V= P/R