Fontan circulation has improved life expectancy for infants born with complex heart disease over the last 50 years but has also resulted in significant morbidity

@article{Hedlund2021FontanCH,
  title={Fontan circulation has improved life expectancy for infants born with complex heart disease over the last 50 years but has also resulted in significant morbidity},
  author={Eva R. Hedlund and B. P. W. Lundell},
  journal={Acta Paediatrica},
  year={2021},
  volume={111},
  pages={11 - 16}
}
The prognosis for infants born with complex heart disease improved dramatically with the introduction of the Fontan circulation 50 years ago. With today's carefully designed and staged operations to a Fontan circulation, life expectancy has increased and most children will survive into adult life. The Fontan circulation entails an unphysiological circulation with high risk for multiple organ system dysfunction. Neurodevelopmental disabilities with adverse psychosocial effects are prevalent. The… 
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References

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TLDR
There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications, and a proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care.
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TLDR
Mechanisms of Fontan failure, risk factors and special considerations for Fontan patients in both medical and surgical management around heart transplantation are discussed and an updated review of survival forFontan patients afterheart transplantation is provided.
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TLDR
This review will discuss the results of different pulmonary vasodilator trials and the use of pulmonary vasODilators as a treatment strategy for Fontan patients, and the role of pulmonary vascular resistance in total cavopulmonary circulation.
The Fontan circulation after 45 years: update in physiology
TLDR
A functional decline of the Fontan circuit is expected and observed as pulmonary vascular resistance and ventricular filling pressure increase with time, but currently chronic preload deprivation with increasing filling pressures.
Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation
TLDR
Patients palliated for complex univentricular heart malformations nowadays benefit from the experience and technical developments of the past decades and have a significantly improved long-term prognosis, however, important issues concerning postoperativelong-term morbidity and mortality are still unsolved and clear intrinsic limitations of the Fontan circulation are becoming evident as the population of Fontan patients ages.
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