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Management of Fontan failure
  1. Emanuela Concetta D'Angelo,
  2. Cristina Ciuca,
  3. Gabriele Egidy Assenza
  1. Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
  1. Correspondence to Dr Gabriele Egidy Assenza, Department of Cardio-Thoracic and Vascular Medicine, Azienda Ospedaliero-Universitaria di Bologna IRCCS, Bologna 40138, Italy; gabriele.egidyassenza{at}

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Learning objectives

  • Understanding unique features of Fontan circulation.

  • Delineate typical modalities of failure in adult patients surviving with Fontan circulation.

  • Provide a rational framework to approach diagnostics and therapeutic measures to Fontan failure.


The first surgical rerouting of systemic venous inflow to the pulmonary circulation without a pumping ventricle was pioneered (in humans) by Dr Francis Fontan in 1968, as a palliative approach in three adult patients with tricuspid atresia.1 After extensive surgical iterations, nowadays, lateral tunnel baffling or extracardiac total cavopulmonary connection (TCPC) represent the modern staged surgical treatment of congenital heart diseases (CHDs) unsuitable for biventricular circulation either due to single ventricular anatomical cluster or adverse anatomical constraints (atrial isomerism, straddling atrioventricular valves and others) (figure 1).2

Figure 1

Circulatory schematic models in biventricular and Fontan circulation (modified from Gewillig and Brown).5 Comparison between (physiological) biventricular circulation and Fontan circulation is provided. Total hydraulic circulatory power variation and flow resistors are depicted. Key findings of Fontan circulation are obligatory chronic systemic venous hypertension and preload limited ventricular chamber. Due to the abnormal pulmonary vascular development in patients with Fontan circulation, true pulmonary vascular resistance is often higher than normal. Ao, aorta; LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle; SV, single ventricle; TCPC, total cavopulmonary connection.

Long-term survival of patients with Fontan circulation (FC) outgrew the expectations and ~95% survival rate at 10 years after Fontan surgery is reported with the more modern surgical innovations.3 However, such delicate circulatory compromise comes at a cost along the way, and a constellation of cardiac and non-cardiac complications is reported in this growing patient population.2 Large registry data report 10-year freedom from death, heart transplantation (HTx), Fontan takedown or conversion, protein-losing enteropathy (PLE), plastic bronchitis (PB), or New York Heart Association functional …

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  • ECD and CC contributed equally.

  • Contributors ECD and CC—reporting, conception and design. GEA—planning, conduct, reporting, conception and design, and interpretation of data.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

  • Author note References which include a * are considered to be key references.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.