Management of Patients With Fontan Circulation

Rychik J, Atz AM, Celermajer DS, et al.
Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association. Circulation 2019;Jul 1:[Epub ahead of print].

This American Heart Association Scientific Statement offers a comprehensive overview of Fontan circulation and associated multiorgan system issues. This summary focuses on recommendations for follow-up monitoring and care for patients with Fontan circulation. The following are key points to remember:

  1. Although there is a paucity of evidence to support any specific screening protocol, the authors of the statement believe that surveillance testing for cardiovascular and end-organ issues is reasonable and clinically important.
  2. For patients of all ages with Fontan circulation, cardiology outpatient visits and electrocardiography are recommended every 6-12 months, with annual echocardiography. Cardiac magnetic resonance imaging (MRI) should be performed once every 3 years in children and every 2-3 years in adults.
  3. Holter monitoring is recommended every 2-3 years in children and every 1-2 years in adolescents and adults. Exercise testing should be considered every 2-3 years as tolerated in children, every 1-3 years in adolescents, and every 1-2 years in adults.
  4. Invasive assessment with cardiac catheterization should be performed as clinically indicated in children with Fontan circulation, and should be considered every 10 years for adolescents and adults.
  5. Serial assessment of exercise capacity is helpful in patients with Fontan circulation, as reduced exercise tolerance is common. Causes of exercise intolerance include lack of subpulmonary pump, chronotropic incompetence, lung restriction, reduced skeletal muscle mass, and deconditioning. Exercise training, including leg muscle resistance training, may prove beneficial in improving cardiac output and quality of life.
  6. The frequency of end-organ system surveillance testing should be individualized and tailored to a patient’s age. It is reasonable to perform surveillance testing every 3-4 years in children <12 years of age, every 1-3 years in adolescents 12-18 years of age, and every 1-2 years in adulthood.
  7. Basic assessment of the liver in children includes a comprehensive metabolic panel, platelet count, and serum gamma-glutamyl transferase (GGT). Basic assessment in adolescents includes the above laboratory studies as well as a prothrombin time/international normalized ratio (PT/INR). Basic liver assessment in adults is similar to adolescents with addition of a cholesterol panel and liver ultrasound. Additional liver studies which could be considered include alpha-fetoprotein, serum FibroSure biomarkers, advanced imaging with computed tomography or MRI, and liver elastography.
  8. Additional organ systems requiring surveillance include the kidney (renal function tests, serum cystatin C), lymphatics (serum albumin, total protein, absolute lymphocyte count), endocrine/metabolic (calcium, vitamin D, Tanner staging), and neurologic/psychologic (neurodevelopmental/cognitive testing).
  9. Patients with Fontan circulation are at increased risk for thromboembolic events. Although the optimal anticoagulation strategy is unclear, some form of thromboprophylaxis is recommended either with an antiplatelet agent or anticoagulation.

Clinical Topics: Anticoagulation Management, Arrhythmias and Clinical EP, Cardiac Surgery, Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Dyslipidemia, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, Sports and Exercise Cardiology, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, Lipid Metabolism, Nonstatins, Interventions and Imaging, Interventions and Structural Heart Disease, Echocardiography/Ultrasound, Magnetic Resonance Imaging, Exercise, Sports and Exercise and Congenital Heart Disease and Pediatric Cardiology, Sports and Exercise and ECG and Stress Testing, Sports and Exercise and Imaging

Keywords: Adolescent, Anticoagulants, Cardiac Catheterization, Cholesterol, Cognition, Cystatin C, Echocardiography, Elasticity Imaging Techniques, Electrocardiography, Electrocardiography, Ambulatory, Exercise Test, Exercise Tolerance, Fontan Procedure, Heart Defects, Congenital, Liver Function Tests, Magnetic Resonance Imaging, Pediatrics, Platelet Aggregation Inhibitors, Prothrombin Time, Quality of Life, Resistance Training, Serum Albumin, Vitamin D

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