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Top Things to Know:
Evaluation and Management of the Child and Adult With Fontan Circulation

  1. The Fontan operation is part of the current strategy of care for single ventricle type of congenital heart disease, which creates a unique circulation characterized by a state of chronically elevated central venous pressure and relatively diminished cardiac output.
  2. Individuals with a Fontan circulation manifest a host of unique complications. As more patients grow into and adulthood, it is increasingly evident that these manifestations are poorly understood, and effective treatment strategies are lacking.
  3. Complications can be generally characterized as cardiovascular or end-organ related, with overlap and interaction between the two groups. Cardiovascular complications include development of ventricular dysfunction, atrioventricular valve regurgitation or arrhythmia.
  4. Liver disease is likely present in all patients with a Fontan circulation with variable degrees of hepatomegaly, hepatic congestion and liver fibrosis common.  Elevations of liver function biomarkers are typical, however hepatic functional capacities remain satisfactory in most.
  5. Chronic venous congestion leads to lymphatic congestion in all patients with a Fontan circulation, and in some, to potentially life-threatening conditions such as protein-losing enteropathy and plastic bronchitis.
  6. Renal dysfunction is present in at least 10% of younger patients with a Fontan circulation and increases with age. Impaired renal function may often go unnoticed and should be evaluated in the older patient.
  7. Patients with Fontan circulation can manifest growth and maturational challenges, including decreased vitamin D, increased parathyroid hormone, low lean muscle mass, and decreased cortical bone mineral density.
  8. Neurodevelopmental disabilities are common in those with a Fontan circulation. Disorders of mood, anxiety and other mental health and behavioral challenges are highly prevalent, influencing quality of life.
  9. Reduced exercise tolerance is common. Nevertheless, healthcare provider restriction to exercise should be rare and exercise training, especially specific strategies such as leg muscle resistance training, may improve cardiac output as well as quality of life.
  10. Given the relatively high prevalence and possible progressive nature of end-organ dysfunction, an expert consensus opinion based “toolkit” for surveillance testing for cardiovascular and end-organ complications in the child and adult with a Fontan circulation is proposed in this AHA statement.

Citation

Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH, Hsia T-Y, Hsu DT, Kovacs AH, McCrindle BW, Newburger JW, Pike NA, Rodefeld M, Rosenthal DN, Schumacher KR, Marino BS, Stout K, Veldtman G, Younoszai AK, d’Udekem Y; on behalf of the American Heart Association Council on Cardiovascular Disease in the Young and Council on Cardiovascular and Stroke Nursing. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association [published online ahead of print July 1, 2019]. Circulation. doi: 10.1161/CIR.0000000000000696.