50 Years of Fontan-Kreutzer Operation
[PRESENTATION TITLE]
Christian Kreutzer MD Pediatric and congenital Heart Surgery
Hospital Universitario Austral.
History of the Fontan Kreutzer operation
Fontan’s “Ventricularization” of RA Kreutzer’s Anterior AP connection w fenest Kreutzer’s Posterior AP connection.
Fontan F, Thorax 1971. Kreutzer GO (1971), JTCVS, Kreutzer GO (1978), Arq Bras Cardiol
The evolution
• 70’s & early 80’ APC Widely adopted
• Giant Right Atrium
• Arrhythmias
• Thrombus formation.
• Compression of the Right Pulmonary Veins.
• Vicious Circle
• “Total Cavo Pulmonary Connection”
• De Leval 1986./ Castañeda 1987 (Fenestration)
• Marcelleti’s Extracardiac Conduit. 1988.
• Extracardiac PTFE Conduit from IVC to RPA.
• All Chambers in normal pressure.
• “No Touch” RA.
• Most Common Fontan K worldwide
Fontan Kreutzer in 2022
• Modern techniques (ECC/LT) > 20 year follow up
• Not a “failed strategy”.
• Excellent Survival 94 % at 20 years. (1) (better than many Bivent Repairs)
• QOL is reasonable
• Fontan K Failure 10/20 %
• Fontan K failure w preserved V. Fx.
• Fontan K failure w S Ventricle Failure (Really a Fontan failure??)
• “You can live without one ventricle but you can’t with none” Billy
Kreutzer, since 1971.
(1) EJCTS 2014 Sep;46(3):465-73;
Aus/NZ Fontan Registry
Fontan Kreutzer in 2022
• What is a Fontan K failure? Let’s be honest.
• A suboptimal management is our failure not Fontan’s
• Most SV CHD have normal pulmonary arteries at birth.
• Sub aortic stenosis can and should be avoided since birth.
• Chronic volume overload (long standing loose bands) should be avoided.
• Phrenic nerve palsy is always iatrogenic.
• Perfect CPB management & Organ protection at Fontan K &
• Technical perfection of the Fontan Pathway and appropoate conduit size.
• Perfect Fontan Follow up and Exercise rehabilitation.
How to get a PERFECT Fontan Kreutzer
procedure
• Avoid residual lesions or obstructions.
– A gradient of 1 mm HG is a DISASTER!!! (James Lock)
– Technical perfection.
• It's got to look good! Wide anastomosis.
• Perfect venous return during CPB.
• Specially the Liver!!
• The phrenic nerve should never be bothered in a FK.
• Inspiration is the only way to load your ventricle
Paralysis of the phrenic nerve as a risk factor for suboptimal Fontan hemodynamics. Ovroutski S EJCTS 2005
Surgical Technique
• Aortic, RA and SVC cannulation
– Minimal dissection of SVC, not
snared.
• Cooling to core T of 18°
–– (20
(20 min)
min)
• Distal Conduit to RPA Anastomosis
during Cooling
–– Inn
Inn Vein
Vein Turn
Turn down
down (selected
(selected cases)
cases)
• Aortic Cross Clamping & DHCA.
– 10/15 min for Inferior anastomosis
– 5/10 min for Fenestration.
Why DHCA
• Hospital Austral y Hospital privado de Cordoba
– 2011/2020. N= 60 pts, (CPBt: 104. 5min, Ao x Ct: 38. 67min, DHCAt: 24.12min)
• Mortality= 0%.
• Take down= 0 %
• Phrenic Nerve Palsy= 0 %
• Early Extubation in 94.3 % of cases
• Morbidity= 20,5 %.
–– Effusions
Effusions (longer
(longer than
than 77 days):
days): 7,5%
7,5%
–– Sepsis=
Sepsis= 5,6%
5,6%
–– Chylothorax=
Chylothorax= 1,88%,
1,88%,
–– Miscelaneous:
Miscelaneous: 11,6%.
11,6%.
Why DHCA
• In summary, The benefit of avoiding DHCA does not
outweigh the harm of conventional CPB in Fontans.
– No evidence against its limited use (<20`).
– Reduces LOS. SVR Trial
– Allows consistent Technical Perfection
• Perfect inferior Anastomosis.
–– Avoids
Avoids distortion
distortion of
of the
the conduit
conduit
–– Larger
Larger Conduits
Conduits inin smaller
smaller patients.
patients.
• Prevents damage to the right phrenic nerve.
• Ensures perfect drainage of caval veins.
• Avoids acute hepatic & Renal Failure from poor drainage during CPB
Fontan Kreutzer in 2022
• Preventing late failure is the new challenge
• Optimal pre Fontan K Management.
• Optimal pre-natal diagnosis.
• Optimal Management at initial Paliation
• Norwood, BT Shunt or PAB
• Optimal Management @ Glenn or Hemi Fontan
• Optimal conduit size. (controversial)
• Itani, K, Ann TSurg 2009, Rinjberg, Eur J Cardiothorac Surg 2022 Nov 7; Gewilig, Eur J Cardiothorac Surg 2022 Nov;
• Identifying patients at risk for late M/M
• Identifying patients at risk for lymphatic failure and end organ fibrosis.
Lymphatics and Fontan K circulation
Fontan K circulation operates at or above the functional limits of the lymphatic circulation
• Increased lymphatic production.
• Lymph drainage compromised Brace MA, Am J Physiol 258, 1990
• No diastole.
• High CVP (12-15 mm Hg) Increased Afterload. Cessation of TD flow at 20-25 mmHG
• Stasis in thoracic duct. Thoracic duct dilation and valve incompetence
• “Lymph will find the way”.
• Early Lymph Complications
• Pleural effusions, Chylothorax, Pulmonary lymphatic edema, Ascites.
• Late Complications
• Effusions, Ascites, PLE, plastic bronchitis.
• Chronic lymphedema= Liver fibrosis, Lung Fibrosis, Renal fibrosis, Myocardial Fibrosis
Fontan end organ fibrosis
Pre Fontan T2 MRI
e in
ag ow n
r ain d
e d take
tu b &
es t lit y
C h or ta
,
S ed m
L O s
s ed r e a
r ea I nc
c
In E III ,
T Y P I V
y pe
in t
LYMPHATIC DECOMPRESSION IN FONTAN
Low pressured TD drainage with Diastole and Inspiration.
TD decompression: Indications & Techique
• Failing Fontan with PB/PLE/Effusions.
• Concomitant to Fontan procedure for high risk
patients
• Thoracic Lymphangiectasia types 3 and 4.
• Early failure, Ascites and Hidrothorax.
• Inn Vein Turn Down: Hraska Procedure.
• Direct Innominate vein w TD turn down
• Wide patent anastomosis, low risk of thrombosis
• Long distance between Inn Vein and LA?
• Ringed PTFE graft
• Dunked in LA cavity. (NOT IDEAL, AC)
• Absent or diminutive LAA?
• Ringed PTFE graft
• Dunked in RAA. (NOT IDEAL, AC)
Hraska procedure Venous & Lymph Catheterization
Pre Fontan Completion MRI Lymphatic Screening
• Since 1/2017 to 06/2022, 35 pts were included.
• Dx: HLHS (n=10), Heterotaxy Synd (n=10), DILV (n=5), TA (n=4), DORV (n=4), PA-IVS(n=1) Ebstein’s
Anomaly (n=1).
• MRI Analysis, CHOP Classification & Pathway:
• Group A: Types I and II (n=24): “Classic” extracardiac Fenestrated
Fontan
• Group B: Types III and IV (n=11): Extracardiac Fontan with
Lymphatic Decompression
• 1 Early mortality in Group A, 1 late mortality in group B.
• 1 Hraska connection Thrombosis & occlusion.
• Less volumen of effusions in LD (group B).
Early Experience with Lymphatic Decompression Concomitant to Fontan Kreutzer Procedure. World J Pediatr Congenit Heart Surg
. 2020 May;11(3):284-292
Lymphatic Decompression Concomitant to Fontan Kreutzer Procedure
• Follow up:@last f-up. (med 26 m).
• Patency of Hraska connection assessed by Echo in 10 surv.
• No PLE or PB.
• One late death in LD group.
• progressive right PV stenosis.
• No differences:
• In survival
• O2 sat= Group A=92%, Group B= 91.4%
• Functional status
Failing Fontan with PLE/PB.
• Criteria for TD decompression surgery:
– Preserved or mildly depressed Single Ventricle Fx (crucial)
– Patent Thoracic duct.
– Ideally to the Left.
• Right TD with a Right Glenn requires conduit interposition.
• Rationale: restore a normal Lymphatic drainage.
– But… Once the leak is present, probably it wont stop after decompression.
• For PLE: Duodenal pressure= 8/10 mmHg
• For PB: Airway pressure is negative.
• Lymphatic Intervention:
– Secondary to TD decompression.
• Embolization of Lung Lymphatic Collaterals for PB
• Embolization of Lymphatic collaterals for PLE.
Clinical experience in Failing Fontans: PLE
Age (yrs) Weight (kg) Diagnosis Time since Ascites Effusions Procedure Outcome
Fontan
Heterotaxy
Late death, acute
syndrome, Yes, Bilat R Glenn take
5 15 2y Yes, massive Pulm Hemorrhage
asplenia, common down, AVVr.
6mf /up .
AVVR,
Heterotaxy
Hraska, RJSC–RA Alive 4 yr f/up,
19 55 Syndrome 8y Yes Yes
PTFE conduit normal albumin
Asplenia
Normal albumin,
Late Death, 2 yrs
Tricuspid Fontan conversion
53 56 35 y Yes, massive No f/up
Atresia II + Hraska
Acute hepatic
faiulre.
Initial Mild
Hraska + ECC
Improv. PLE
28 55 Mitral Atresia 14 y Yes No change
relapse, Alive
after ontervention
Clinical experience in Failing Fontans: PB & intractable effussions
Age (yrs) Weight (kg) Diagnosis Time since Plastic Bronc. Ascites Pleural Procedure Outcome
Fontan effusions
Alive. 1y No
PA IVS Stenotic Hraska + PA
6 21 2y Yes No Yes, right PB after Cath
BDG plasty
Intervention.
Heterotaxy Alive, 2 yr
6 16 1,5 yrs Yes No No Hraska
síndrome Asymptomatic
TA 1B ECC Hraska + ECC Alive,
18 57 14 No Yes no
stenosis change asymptomatic
Late death 2 yrs
f/up, viral
3 8 HLHS 4m No Yes, massive Yes, Bilat Hraska
infection, Vent
dysf
Heterotaxy
Hraska, TAPVR Early death, Vent
4 15 syndrome, 2m No Yes Yes, Bilat
correction. Dysfx
asplenia
Summary I
• The results of FONTAN KREUTZER procedure have improved
significantly through the understanding, & refinement of the surgical
technique.
• The fate of a Fontan patient is determined since the prenatal stage.
• Optimal management is mandatory.
• It must be perfect. Residual lesions are poorly tolerated
• The strategy of deep hypothermic circulatory Arrest is an alternative to
continuously improve results.
Summary II
• Survival is no longer the problem but QOL & end organ fibrosis.
• Exercise planning and surveillance programs.
• Preventing late FALD, RF, Lung fibrosis and Myocardial fibrosis.
• Lymphatic intervention (Cath & surgical) for Failing Fontan is here
• Lymphatic decompression can be achieved @ Fontan w promising
results for patients at risk or with of Lymphatic failure.
• To all Fontans? Ameliorate End organ fibrosis?
• A randomized trial?
