Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.

@article{Rychik2019EvaluationAM,
  title={Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.},
  author={Jack Rychik and Andrew M Atz and David S. Celermajer and Barbara Deal and Michael A. Gatzoulis and Marc Gewillig and Tain-Yen Hsia and Daphne T. Hsu and Adrienne H. Kovacs and Brian W. McCrindle and Jane W. Newburger and Nancy A Pike and Mark D. Rodefeld and David N. Rosenthal and Kurt R Schumacher and Bradley Scott Marino and Karen K Stout and Gruschen R. Veldtman and Adel K. Younoszai and Yves D'udekem},
  journal={Circulation},
  year={2019},
  pages={
          CIR0000000000000696
        }
}
It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year… 
Management of Fontan circulation in pregnancy: a multidisciplinary approach to care.
Anesthetic considerations for Fontan circulation and pregnancy
TLDR
The Fontan operation has improved life expectancy markedly for patients born with single-ventricle-type congenital heart disease, and anesthesiologists need to familiarize themselves on their altered physiology and how to best treat these patients throughout their pregnancy.
Perioperative Management of the Fontan Patient for Cardiac and Noncardiac Surgery.
TLDR
The pathophysiology pertinent to the provision of anesthesia in this complex patient group is summarized and important modifications to anesthetic technique and perioperative management are described.
Fontan Circulation of the Next Generation: Why It's Necessary, What it Might Look Like
TLDR
Care in the current era is directed toward the objective of achieving successful separation of the systemic and pulmonary circulations, using the functionally single ventricle to support the systemic arterial circulation while relying on surgically created direct systemic venous-topulmonary arterial connections to deliver blood flow to the lungs.
Case report of the broad spectrum of late complications in an adult patient with univentricular physiology palliated by the Fontan circulation
TLDR
A case of an adult patient after Fontan palliation is presented, illustrative of the complex clinical course and the broad spectrum of complications that can be encountered during follow-up, highlighting the need for a multidisciplinary approach in the clinical care for patients with an univentricular physiology.
Imaging of complications following Fontan circulation in children — diagnosis and surveillance
TLDR
The author discusses how and when radiology is important in Fontan patients, as well as how new techniques enabling quantitative measures in imaging with US, MRI and CT are adapted for pediatric use, and how they contribute to urgently needed surveillance strategies.
Imaging of complications following Fontan circulation in children - diagnosis and surveillance.
TLDR
The author discusses how and when radiology is important in Fontan patients, as well as how new techniques enabling quantitative measures in imaging with US, MRI and CT are adapted for pediatric use, and how they contribute to urgently needed surveillance strategies.
Recent outcomes of the extracardiac Fontan procedure in patients with hypoplastic left heart syndrome
TLDR
Preoperative weight and IVC diameter appeared to have no influence on immediate postoperative outcomes in HLHS patients undergoing Fontan, and performing the Fontan off CPB and with a fenestration also conferred no added clinical benefit.
Ventricular assist device for Fontan: who, when and why?
TLDR
Fontan patients demonstrating signs of heart failure should be evaluated early and often for feasibility and optimal timing of VAD implantation, whereas patients with increased transpulmonary gradient may not benefit from a single-VAD solution.
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References

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TLDR
The ‘Fontan circulation' has evolved to include a variety of surgical procedures designed to overcome the absence of two distinct ventricular chambers, bringing a multiplicity of haemodynamic complications and sequelae of their abnormal circulatory status.
Fontan Conversion
TLDR
The purpose of this article is to review the extant literature on Fontan Conversion and to assess the lessons learned over 20 years.
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TLDR
In a contemporary series of Fontan operations performed largely in patients with hypoplastic left heart syndrome or variants, systemic ventricle morphology had no effect on mortality.
Fontan Operation in the Current Era: A 15-Year Single Institution Experience
TLDR
The Fontan procedure can be performed with low risk regardless of ventricular anatomy, and duration of XC time is associated with survival.
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TLDR
In the authors' experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings.
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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.
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TLDR
There is a significant, increasing arrhythmia burden in adult patients with a Fontan circulation, and arrhythmia development could be regarded as an inevitable consequence of an AP Fontan.
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TLDR
The lack of economic incentive for development of right-sided pumps may be partially ameliorated both by recognition of the size of the cohort of Fontan patients now surviving into adulthood as well as by the increasing recognition of important right- sided heart failure in adults with biventricular hearts supported only with durable left-sided ventricular assist devices.
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