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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Methods Citations
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Invasive hemodynamic assessment of the effect of sildenafil treatment after Fontan operation
TLDR
The hemodynamic parameters related to the ventricular morphology and the type of previous palliation were found and the pharmacological modulation of the pulmonary vascular status is an important component of the treatment of patients with Fontan circulation.
Delving into the Molecular World of Single Ventricle Congenital Heart Disease
TLDR
A review highlights and summarizes recent advances in uncovering the genetic and molecular mechanisms in SV CHD etiology, indicating that sophisticated spatiotemporal regulation of gene transcription networks and functional cellular pathways govern disease progression.
Early outcomes of fenestrated intra‐extracardiac Fontan procedure: Insights, experiences, and expectations
TLDR
Early outcomes in patients with intraextracardiac fenestrated FP are presented and the advantages of lateral tunnel and extracARDiac conduit modifications of the original technique are presented.
THREE- COUNTRY STUDY FOUND BET TER SURVIVAL R ATES FOR E X TREMELY PRETERM INFANTS BORN IN SWEDEN
  • 2021

References

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Long-Term Kidney Function After the Fontan Operation: JACC Review Topic of the Week.
Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.
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.
Transplantation of the failing Fontan.
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.
Pathophysiology of thrombosis and anticoagulation post Fontan surgery.
Hypoplastic left heart syndrome: current considerations and expectations.
Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy*
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.
Hypoplastic left heart syndrome: experience with palliative surgery.
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.
Fontan-associated liver disease: A review.
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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