Managing adult Fontan patients: Where do we stand?

Paul Clift; David Celermajer

doi:10.1183/16000617.0091-2016

Managing adult Fontan patients: Where do we stand?

Paul Clift, David Celermajer

Research output: Contribution to journal › Review article › peer-review

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Abstract

The Fontan operation is performed as a palliative procedure to improve survival in infants born with a functionally univentricular circulation. The success of the operation is demonstrated by a growing adult Fontan population that exists with this unique physiology. Late follow-up has demonstrated expected and unexpected sequelae, and has shown multisystem effects of this circulation. This review discusses the challenges of managing the late complications in terms of understanding this unique physiology and the innovative therapeutic interventions that are being investigated. The challenge remains to maintain quality of life for adult survivors, as well as extending life expectancy.

Original language	English
Pages (from-to)	438-450
Number of pages	13
Journal	European Respiratory Review
Volume	25
Issue number	142
DOIs	https://doi.org/10.1183/16000617.0091-2016
Publication status	Published - 1 Dec 2016
Externally published	Yes

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10.1183/16000617.0091-2016Licence: CC BY-NC

10198273-oaFinal published version, 553 KBLicence: CC BY-NC

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AB - The Fontan operation is performed as a palliative procedure to improve survival in infants born with a functionally univentricular circulation. The success of the operation is demonstrated by a growing adult Fontan population that exists with this unique physiology. Late follow-up has demonstrated expected and unexpected sequelae, and has shown multisystem effects of this circulation. This review discusses the challenges of managing the late complications in terms of understanding this unique physiology and the innovative therapeutic interventions that are being investigated. The challenge remains to maintain quality of life for adult survivors, as well as extending life expectancy.

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