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Four decades of Fontan palliation

Nature Reviews Cardiology volume 7, pages 520–527 (2010)Cite this article

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Abstract

The Fontan palliation was introduced in 1968 to treat cardiac malformations unsuitable for biventricular repair. This procedure has transformed the surgical management of congenital heart disease. In this Review, we reflect on the outcomes and clinical problems associated with this unique circulation after more than 40 years of experience. We also summarize the evolution of the Fontan procedure, highlight the long-term clinical issues and their management, and consider future expectations of a circulation driven by a single ventricle with the systemic and pulmonary blood flow in series rather than in parallel.

Key Points

  • The Fontan palliation is a surgical procedure that is used to repair congenitally malformed hearts with only one functional ventricle

  • The total cavopulmonary connection is the most widely adopted Fontan procedure

  • Despite considerable improvements in the prognosis of patients, the Fontan procedure has intrinsic limitations

  • Future advances, such as use of mechanical assist devices and transplantation technologies, could overcome or at least alleviate limitations associated with this procedure

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Figure 1: The different types of Fontan circulation.
Figure 2: The circulation of the lung.
Figure 3: Mechanisms of arterial hypoxemia in the hepatopulmonary syndrome in a two-compartment model of gas exchange in the lung.
Figure 4: Experimental studies of intestinal barrier function.

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  1. Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, WC1N 3JH, London, UK

    Marc R. de Leval & John E. Deanfield

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M. R. de Leval and J. E. Deanfield both contributed to discussion of content for the article. M. R. de Leval wrote the majority of the article and J. E. Deanfield made substantial contributions to it. M. R. de Leval and J. E. Deanfield reviewed and edited the manuscript.

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Correspondence to John E. Deanfield.

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de Leval, M., Deanfield, J. Four decades of Fontan palliation. Nat Rev Cardiol 7, 520–527 (2010). https://doi.org/10.1038/nrcardio.2010.99

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