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Fontan procedure

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Fontan or Fontan–Kreutzer procedure
Fontan procedure for tricuspid atresia
ICD-10-PCSZ98.890
ICD-9-CM35.94
MeSHD018729

The Fontan procedure or Fontan–Kreutzer procedure is a palliative surgical procedure used in children with univentricular hearts. It involves diverting the venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries without passing through the morphologic right ventricle; i.e., the systemic and pulmonary circulations are placed in series with the functional single ventricle. The procedure was initially performed in 1968 by Francis Fontan and Eugene Baudet from Bordeaux, France, published in 1971, simultaneously described in 1971 by Guillermo Kreutzer from Buenos Aires, Argentina, and finally published in 1973.[1][2]

Indications

The Fontan procedure is used in pediatric patients who possess only a single functional ventricle, either due to lack of a heart valve (e.g. tricuspid or mitral atresia), an abnormality of the pumping ability of the heart (e.g. hypoplastic left heart syndrome or hypoplastic right heart syndrome), or a complex congenital heart disease where a bi-ventricular repair is impossible or inadvisable. The surgery allows blood to be delivered to the lungs via central venous pressure rather than via the right ventricle.[3] Patients typically present as neonates with cyanosis or congestive heart failure.[4] Fontan completion is usually carried out when the patient is 2–5 years of age, but is also performed before 2 years of age.[5][6]

Contraindications

After Fontan completion, blood must flow through the lungs without being pumped by the heart. Therefore, children with high pulmonary vascular resistance may not tolerate a Fontan procedure. Often, cardiac catheterization is performed to check the resistance before proceeding with the surgery. This is also the reason a Fontan procedure cannot be done immediately after birth; the pulmonary vascular resistance is high in utero and takes months to drop. Fontan procedure is also contraindicated in those with pulmonary artery hypoplasia, patients with left ventricular dysfunction and significant mitral insufficiency.[citation needed]

Types

There are four variations of the Fontan procedure:[7]

  • Ventricularization of the Right Atrium (The original Fontan's Technique)
  • Atriopulmonary connection (the original Kreutzer's Technique)
  • Intracardiac total cavopulmonary connection (lateral tunnel) (described by Marc De Leval and Aldo Castañeda, separately)
  • Extracardiac total cavopulmonary connection (described by Carlo Marceletti)

Approach

Coronal CT image in a 19-year-old patient with tricuspid atresia treated with bidirectional Glenn shunt and Fontan.

The Fontan is usually done as a two staged repair.[8]

The first stage, also called a Bidirectional Glenn procedure or Hemi-Fontan (see also Kawashima procedure), involves redirecting oxygen-poor blood from the top of the body to the lungs. That is, the pulmonary arteries are disconnected from their existing blood supply (e.g. a Blalock Taussig or a Sano shunt created during a Norwood procedure, a patent ductus arteriosus or a pulmonary artery banding). The superior vena cava (SVC), which carries blood returning from the upper body, is disconnected from the heart and instead redirected into the pulmonary arteries. The inferior vena cava (IVC), which carries blood returning from the lower body, continues to connect to the heart.[9]

The second stage, also called Fontan Kreutzer completion, involves redirecting the blood from the IVC to the lungs. At this point, the oxygen-poor blood from upper and lower body flows through the lungs without being pumped (driven only by the pressure that builds up in the veins). This corrects the hypoxia and leaves the single ventricle responsible only for supplying blood to the body.[10]

Post-operative complications

In the short term, children can have trouble with pleural effusions (fluid building up around the lungs). This can require a longer stay in the hospital for drainage with chest tubes. To address this risk, some surgeons make a fenestration from the venous circulation into the atrium. When the pressure in the veins is high, some of the oxygen-poor blood can escape through the fenestration to relieve the pressure. However, this results in hypoxia, so the fenestration may eventually need to be closed by an interventional cardiologist.

In a 2016 review, Dr. Jack Rychik, head of the Single Ventricle Survivorship Program at Children's Hospital of Philadelphia summarized the long-term consequences of Fontan circulation as an "indolent and progressive state of heart failure" with predictable long-term consequences on several organ systems.[11] Chronic venous hypertension and lowered cardiac output are assumed to be at the root of lymphatic complications such as chylothorax, protein losing enteropathy and plastic bronchitis which may occur in the immediate post-operative period as well as in the medium term. New interventional and surgical strategies have been investigated to relieve the lymphatic complications associated with the Fontan circulation.[12] Concerns about damage to the liver have emerged more recently, as the Fontan circulation produces congestion and lymphedema in this organ which leads to progressive hepatic fibrosis and other complications of Fontan-Associated Liver Disease.[13] Screening protocols and treatment standards are emerging in the light of these discoveries.[11]

The Fontan procedure is palliative — not curative — but more than 80% of the cases can result in normal or near-normal growth, development, exercise tolerance, and good quality of life.[14] However, 10% or more of patients may eventually require heart transplantation,[15] and given the long-term consequences of chronic venous hypertension and insidious organ damage, freedom from morbidity is unlikely in the long term. New approaches to the management of failing Fontans or other clinical deterioration have included Lymphatic decompression surgical procedures & intervention, Ventricular assist devices or other mechanical support therapies as either bridge to transplantation or destination therapies.[16]

History

Francis Fontan (left) and Guillermo Kreutzer (right) in 2009

The Fontan procedure was initially described in 1971 by Dr. Francis Fontan (1929–2018) from Bordeaux, France. Prior to this, the surgical treatment for tricuspid atresia consisted of creating a shunt between a systemic artery and the pulmonary artery (Blalock-Taussig shunt) or the superior vena cava and the pulmonary artery (Glenn shunt). These procedures were associated with high mortality rates, commonly leading to death before the age of one year.[17] In an attempt to improve this, Fontan was engaged in research between 1964 and 1966 endeavouring to fully redirect flow from the superior and inferior vena cavae to the pulmonary artery.[18] His initial attempts in dogs were unsuccessful and all experimental animals died within a few hours; however, despite these failures, he successfully performed this operation in a young woman with tricuspid atresia in 1968 with Dr Eugene Baudet.[17] The operation was completed on a second patient in 1970, and after a third case the series was published in the international journal Thorax in 1971.[19] Dr. Guillermo Kreutzer from Buenos Aires, Argentina (b. 1934) without any knowledge of Fontan's experience performed a similar procedure in July, 1971 without placing a valve in the Inferior Vena Cava inlet and introducing the concept of "fenestration" leaving a small atrial septal defect to serve as a pop-off valve for the circulation.[20][17]

References

  1. ^ Fontan, F.; Baudet, E. (May 1971). "Surgical repair of tricuspid atresia". Thorax. 26 (3): 240–248. doi:10.1136/thx.26.3.240. ISSN 0040-6376. PMC 1019078. PMID 5089489.
  2. ^ Kreutzer, G.; Galíndez, E.; Bono, H.; De Palma, C.; Laura, J. P. (October 1973). "An operation for the correction of tricuspid atresia". The Journal of Thoracic and Cardiovascular Surgery. 66 (4): 613–621. ISSN 0022-5223. PMID 4518787.
  3. ^ Heaton J, Heller D (2021). "Single Ventricle". StatPearls. Treasure Island (FL): StatPearls Publishing. PMID 32491721. Retrieved 2021-08-18.
  4. ^ O'Leary PW (2002). "Prevalence, clinical presentation and natural history of patients with single ventricle". Progress in Pediatric Cardiology. 16: 31–38. doi:10.1016/s1058-9813(02)00042-5.
  5. ^ Hirsch JC, Goldberg C, Bove EL, Salehian S, Lee T, Ohye RG, Devaney EJ (September 2008). "Fontan operation in the current era: a 15-year single institution experience". Annals of Surgery. 248 (3): 402–10. doi:10.1097/SLA.0b013e3181858286. PMID 18791360. S2CID 12921302.
  6. ^ Anderson PA, Sleeper LA, Mahony L, Colan SD, Atz AM, Breitbart RE, et al. (July 2008). "Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study". Journal of the American College of Cardiology. 52 (2): 85–98. doi:10.1016/j.jacc.2008.01.074. PMC 4385517. PMID 18598886.
  7. ^ de Leval MR (April 2005). "The Fontan circulation: a challenge to William Harvey?". Nature Clinical Practice. Cardiovascular Medicine. 2 (4): 202–8. doi:10.1038/ncpcardio0157. PMID 16265484. S2CID 36129105.
  8. ^ van der Ven, JPG; van den Bosch, E; Bogers, AJCC; Helbing, WA (2018). "State of the art of the Fontan strategy for treatment of univentricular heart disease". F1000Research. 7. doi:10.12688/f1000research.13792.1. PMID 30002816.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  9. ^ van der Ven, JPG; van den Bosch, E; Bogers, AJCC; Helbing, WA (2018). "State of the art of the Fontan strategy for treatment of univentricular heart disease". F1000Research. 7. doi:10.12688/f1000research.13792.1. PMID 30002816.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  10. ^ van der Ven, JPG; van den Bosch, E; Bogers, AJCC; Helbing, WA (2018). "State of the art of the Fontan strategy for treatment of univentricular heart disease". F1000Research. 7. doi:10.12688/f1000research.13792.1. PMID 30002816.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  11. ^ a b Rychik J (2016). "The Relentless Effects of the Fontan Paradox". Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual. 19 (1): 37–43. doi:10.1053/j.pcsu.2015.11.006. PMID 27060041.
  12. ^ Hraska V, Hjortdal VE, Dori Y, Kreutzer C | title = Innominate vein turn-down procedure: Killing two birds with one stone | journal = Journal of Thoracic and cardiovascular Surgery Tech. 2021;7: | volume = 7 | issue = 1 | pages = 253-260 | date = Jun 2021 | pmid = 34318266 | doi =10.1016/j.xjtc.2021.01.045
  13. ^ Gordon-Walker TT, Bove K, Veldtman G (September 2019). "Fontan-associated liver disease: A review". Journal of Cardiology. 74 (3): 223–232. doi:10.1016/j.jjcc.2019.02.016. PMID 30928109.
  14. ^ Chan A, Aijaz A, Zaidi AN (March 2020). "Surgical outcomes in complex adult congenital heart disease: a brief review". Journal of Thoracic Disease. 12 (3): 1224–1234. doi:10.21037/jtd.2019.12.136. PMC 7139079. PMID 32274204.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  15. ^ McCormick AD, Schumacher KR (October 2019). "Transplantation of the failing Fontan". Translational Pediatrics. 8 (4): 290–301. doi:10.21037/tp.2019.06.03. PMC 6825971. PMID 31728322.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  16. ^ Clift P, Celermajer D (December 2016). "Managing adult Fontan patients: where do we stand?". European Respiratory Review. 25 (142): 438–450. doi:10.1183/16000617.0091-2016. PMID 27903666.
  17. ^ a b c Cowgill LD (June 1991). "The Fontan procedure: a historical review". The Annals of Thoracic Surgery. 51 (6): 1026–30. doi:10.1016/0003-4975(91)91044-v. PMID 2039305.
  18. ^ Anderson RH (November 1999). "Francis Fontan". Cardiology in the Young. 9 (6): 592–600. doi:10.1017/s1047951100005631. PMID 10593269.
  19. ^ Fontan F, Baudet E (May 1971). "Surgical repair of tricuspid atresia". Thorax. 26 (3): 240–8. doi:10.1136/thx.26.3.240. PMC 1019078. PMID 5089489.
  20. ^ Kreutzer G, Galíndez E, Bono H, De Palma C, Laura JP (October 1973). "An operation for the correction of tricuspid atresia". The Journal of Thoracic and Cardiovascular Surgery. 66 (4): 613–21. doi:10.1016/S0022-5223(19)40598-9. PMID 4518787.
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