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 appropiate conduit size.
• Perfect Fontan Follow up and Exercise rehabilitation.
 A technique for “Perfect” Fontan K

• Aortic, RA and SVC cannulation

– Minimal dissection of SVC, not
snared.
• CPB, 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%.

Abstract presentation, SAC Meeting 2020

 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.
»» Itani,
Itani, K,
K, Ann
Ann TSurg
TSurg 2009,
2009, Rinjberg,
Rinjberg, Eur
Eur JJ Cardiothorac
Cardiothorac Surg
Surg 2022
2022 Nov
Nov 7;
7; Gewilig,
Gewilig, Eur
Eur JJ Cardiothorac
Cardiothorac Surg
Surg 2022
2022 Nov;
Nov;

• 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
• Optimal univentricular management.
• Optimal pre-natal diagnosis.
• Optimal Management at initial Paliation
• Norwood, BT Shunt or PAB
• Optimal Management @ Glenn or Hemi Fontan
• Optimal Management @ Fontan completion.

• Preventing late failure is the new challenge

• Exercise rehabilitation & super Fontans, mental health.
• Identifying patients at risk for end organ fibrosis & lymphatic failure
 Exercise rehabilitation
• Adolescent and adult patients (≥16 years) with serial CPET
• over 5 years of f/up
• 37 patients. 13 RV- 24 LV.
• 55 % had stable, in peak exercise capacity.
• Regular participation in physical activity was common in patients
with a positive exercise capacity trajectory.
Heart, Lung and Circulation

Volume 30, Issue 9, September 2021, Front Pedriatics. The Fontan Fitness Clinical trial 2022.
 Super Fontans
• A phenotype: Pts with normal exercise capacity- (> 80%)
• Characteristics:
• LV morphology.
• Normal weight.
• Normal respiratory function.
• History of exercise.
• History of continuous exercise. Front Cardiovasc Med. 2021;
Mental Health in late survivors
• Abnormal cognition
• attention disorders
• executive functioning.
• Abnormal visual spatial reasoning and psychosocial development.
• anxiety disorders and depression.
• PTS, from multiple surgeries & catheterizations
• Prevention and psychological intervention to optimize long-term
outcomes are critical in the care of this vulnerable population with
complex CHD. Front Pediatr. 2022; 10: 826349 J Newberger
 The Achilles heel of the Fontan K circulation
Fontan K circulation operates at or above the functional limits of the lymphatic circulation
• Increased lymphatic production.
• Lymph drainage compromised
• No diastole.
• High CVP (12-15 mm Hg) Increased Afterload. Cessation of TD flow at 20-25 mmHG
Brace MA, Am J Physiol 258, 199
• Stasis in thoracic duct. Thoracic duct dilation and valve incompetence
• Lymphostasis & Lymphedema.

• “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 in 126 pts

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es t lit y
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 Restore a “normal” Lymphatic Drainage
LYMPHATIC DECOMPRESSION IN FONTAN KREUTZER

TD drainage to a Low pressured atrium with Diastole and Inspiration.

Hraska procedure Venous & Lymphatic
Catheterization

Videos courtesy of Marcelo D. Rivarola & Alejandro R Peirone

TD decompression: Indications & Technique
• Failing Fontan K with PB/PLE/Effusions.

• Concomitant to Fontan K for high-risk patients

(Prophylactic)
• Thoracic Lymphangiectasia types 3 and 4 in T2 MRI

• Early failure, Ascites and Hidrothorax.

• Technique of the Hraska Procedure.

• Direct Innominate vein w TD turn down to LAA
• Anatomy of LAA. (CT Angio if LAA not clearly seen in MRI)
• Wide patent anastomosis, low risk of thrombosis
• Long distance between Inn Vein and LA?
• Ringed PTFE graft
• Dunked in LA cavity.
• diminutive LAA?
• Ringed PTFE graft
• Dunked in RAA.
 Pre Fontan Kreutzer Completion MRI Lymphatic Screening

• Since 1/2017 to 5/2023, 38 pts were included in the Cohort.

• Dx: HLHS (n=10), Heterotaxy Synd (n=11), DILV (n=5), TA (n=5), DORV (n=5), PA-IVS(n=1) Ebstein’s Anomaly (n=1).

• MRI Analysis, CHOP Classification & Pathway:

• Group A: Types I and II (n=26) “Classic” extracardiac Fen. Fontan Kreutzer
• Group B: Types III and IV (n=12) Extracardiac Fontan K with Hraska.
• Direct Inn Vein Turn down (n=8), PTFE conduit interposition (n=4)

• 1 Early mortality in Group A.

• 1 inn Vein Turn Down Thrombosis & occlusion.

• Less volume of effusions in LD (group B). p=.03

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: (med 30 m).

• Patency of Inn Vein turn down assessed by Echo/CT/Ang in 12
surv.
• 4 complete occlusions. (All PTFE conduit)
• No PLE or PB.
• One late death in LD group.
• progressive right PV stenosis.
• No differences between groups
• Survival
• O2 sat= Group A=92%, Group B= 91.4%
• Functional status
Failing Fontan Kreutzer with PLE/PB/Effusions (n=10)
• Criteria for TD decompression surgery:
– Preserved or mildly depressed Single Ventricle Fx (crucial) & normal EDP.
– Patent Thoracic duct and patent Inn & cervical veins.
• 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 mm Hg
• 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 Fontan Kreutzer: Plastic Bronchitis

Age (yrs.) CHOP type Diagnosis Time since Ascites Pleural Procedure Outcome
Fontan K effusions

Alive. 2y
No
PA IVS Hraska +
PB after
6 IV Stenotic 2y No Yes, right SVC-PA
Cath
BDG plasty
Interventio
n.

Hraska Alive,
55 IV TA 1 No NO
MVR asymp

Alive, 2 yr.
Heterotaxy
6 IV 1,5 yrs. No No Hraska Asymptoma
syndrome
tic
 Clinical experience in Failing Fontan Kreutzer: PLE (n=4)
Age (yrs) Diagnosis CHOP Yrs since Ascites Effusions Procedure Outcome
type Fontan
Heterotaxy Improved
syndrome, Albumin
bilateral SVC Yes, Bilat R Glenn take Late death,
5 IV 2 Yes
asplenia, down, AVV R acute Pulm
common AVVR, Hemorrhage
6m f/up.
Heterotaxy Hraska + ECC
Alive 5 yr. f/up,
19 Syndrome III 9 Yes Yes change
normal albumin
Asplenia
Normal
Fontan Albumin. Late
Tricuspid
53 III 36 Yes, massive No conversion + Death, 2 yrs
Atresia II
Hraska f/up fulminant
hepatitis.
Initial Mild
Improv. PLE
relapse,
Hraska + ECC
Mitral Atresia Intervention X2,
18 III 15 Yes No change
DORV Partial Hraska
Connection
occlusion
Death
 Clinical experience in Failing Fontan Kreutzer: intractable effusions

Age (yrs) CHOP type Diagnosis Time since Plastic Ascites Pleural Procedure Outcome
Fontan K Bronc. effusions

TA 1B ECC Hraska + ECC Alive,

18 II 14y No Yes no
stenosis change asymptomatic

Heterotaxy
Hraska, TAPVR Early death,
4 IV syndrome, 2m No Yes Yes, Bilat
correction. Vent Dysfx
asplenia

Late death 2
3 IV HLHS 4m No Yes, massive Yes, Bilat Hraska yrs. f/up, viral
pneumonia
 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.

• Surveillance programs. (Mental, exercise, nutrition, etc)

• 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?