(Observational Study)

Study Summary

The Fontan Study looked at the health status, exercise capacity, laboratory results and heart performance in children after the Fontan Procedure.

546 Participants

Were included in the study

81% of Fontan Patients

Scored in the normal range for physical functioning

87% of Fontan Patients

Were in the normal range for mental functioning

Who was in the study?

People in the study:
  • Were 6-18 years of age
  • Had the Fontan surgery at least 6 months before entry into the study
  • Were not pregnant

What happened during the study?

Each qualified person completed the following study procedures:
  • Health status questionnaire (parents and children 10 years old and older)
  • Echocardiogram
  • Blood sample
  • Cardiac MRI (magnetic resonance imaging)
  • Exercise Testing

What were the results of the study?

Here are some things that we learned about Fontan patients as a group:
  • The health surveys showed that, although the average score for the Fontan patient was lower than the average score in children without congenital heart disease, 81% of Fontan patients scored in the normal range for physical functioning and 87% were in the normal range for mental functioning.
  • We found that children in the study did not perform as well on the bicycle exercise test as children without congenital heart disease and that younger children in the study did better than older children.

The things we learned may not apply to every child. The findings reflect those for all 546 people who participated. Individual results varied quite a bit.

The PHN is grateful to all of the families who participated in this study. The results from this study will help us to design future studies to learn more about helping children who had Fontan surgery live healthier lives.

Study Publications

  • Contemporary Outcomes After the Fontan Procedure: A Pediatric Heart Network Multicenter Study

    P. Anderson, J Am Coll Cardiol 2008; 52:85-98.

    The Fontan study was designed to study children born with a single ventricle or heart pumping chamber who underwent a type of surgery called the Fontan procedure Data was collected from 546 children between 6 and 18 years of age about their heart structure and function after surgery, ability to exercise, heart rhythm and certain lab tests. Questions were asked to assess physical, mental, and emotional well being. The study showed that most Fontan survivors had a normal sense of well being. Most had normal ability for the heart to squeeze, but the majority had decreased relaxation of the heart muscle. Ability to exercise was also decreased in most. Children with a single right ventricle (pumping chamber) had decreased function of the heart and its valves compared with children with a single left ventricle. If the Fontan procedure was done at an older age, heart valves did not work as well and children were less likely to have a normal heart rhythm.

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  • Design of a Large Cross-Sectional Study to Facilitate Future Clinical Trials in Children with the Fontan Palliation

    L. Sleeper, Am Heart J 2006; 152:427-433.

    This article describes how the Fontan study was designed and implemented in the Pediatric Heart Network. The purpose of the study was to determine whether clinical measures of how well the heart is working (such as lab tests, exercise testing, and echocardiogram) compare with health status (quality of life) in patients who have had a Fontan procedure for congenital heart disease. The data will help researchers design future clinical trial.

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  • Protein-losing enteropathy after fontan operation: investigations into possible pathophysiologic mechanisms

    A. Ostrow, Ann Thorac Surg 2006; 82:695-700.

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  • Impaired Power Output and Cardiac Index with Hypoplastic Left Heart Syndrome: A Magnetic Resonance Imaging Study

    K. Sundareswaran, Ann Thorac Surg 2006; 82(4):1267-75; discussion 1275-77.

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  • Flow Study of an Extra-Cardiac Connection with Persistent Left Superior Vena Cava

    D. de Zélicourt, J Thorac Cardiovasc Surg 2006; 131:785-91.

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  • Relationship of Patient and Medical Characteristics to Health Status in Children and Adolescents after the Fontan Procedure

    B. McCrindle, Circulation 2006; 113:1123-1129.

    Children with a single ventricle (heart pumping chamber) who have undergone the Fontan procedure are at risk of having poor health because of frequent hospitalizations and surgeries, decreased ability to exercise, and other medical problems. Parents of children in the Fontan study answered questions about their child’s physical activity, behavior, emotions and self-esteem. The results showed that as a group, children who have had the Fontan procedure have more problems with vision, speech, hearing, attention, learning, behavior, anxiety and depression compared to children in the general population. The impact of these other problems was made worse by having a lower family income. Future research is needed to identify ways to diagnose, prevent and treat these problems and to recognize the impact they have on children who have had the Fontan procedure.

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  • Physical Activity Levels in Children and Adolescents are Reduced After the Fontan Procedure, Independent of Exercise Capacity, and are Associated with Lower Perceived General Health

    B. McCrindle, Arch Dis Child 2007; 92:509-514.

    The Pediatric Heart Network studied 147 of the children in the Fontan study to see how physically active they were on a daily basis. The researchers found that these children participated in physical activity far less often than healthy children, even if their exercise tests showed that they are able to exercise. Children who spent less time being physically active believed they were less healthy overall, whereas children who were not able to exercise as well not only believed they were less healthy, but also had lower level of physical function and self-esteem.

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  • Functional State of Patients with Heterotaxy Syndrome Following the Fontan Operation

    A. Atz, Cardiol Young 2007; 17 Suppl 2:44-53.

    Children with heterotaxy syndrome have been found to have poorer outcomes compared to children with other complex heart problems, even if those children undergo the same surgeries. Eight percent of children in the Fontan study were found to have heterotaxy syndrome. Those children with heterotaxy were compared to children in the Fontan study without heterotaxy, in regards to their medical and surgical history, heart structure and function, ability to exercise, and health status. The study showed that there were no real differences between the two groups in the children’s sense of physical and psychosocial well-being or ability to exercise.

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  • The Fontan Procedure Our Odyssey Continues

    C. Backer, J Am Coll Cardiol 2008; 52:114-116.

    This article describes the results of the Fontan study and how those results may change the way we care for children who will need to have the Fontan procedure.

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  • A Cross-Sectional Study of Exercise Performance During the First 2 Decades of Life After the Fontan Operation

    S. Paridon, J Am Coll Cardiol 2008; 52:99-107.

    This study discusses the exercise performance of a large group of children in the Fontan study. Of the 546 children in the study, 411 had exercise testing performed, and most (60%) were not able to exercise to peak levels. Those who were able to reach peak exercise were more likely to be older. Many reported fatigue as a reason for not reaching peak exercise. The wide range of performance was found to be related to the whether the heart is able to increase the amount of blood squeezed out of the pumping chamber (stroke volume). The study also showed that as males go through puberty and gain more muscle, the heart after a Fontan procedure may not be able to keep up with increased demands, making exercise more difficult.

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  • Functional Status, Heart Rate, and Rhythm Abnormalities in 521 Fontan Patients 6-18 Years of Age

    A. Blaufox, J Thorac Cardiovasc Surg 2008; 136:100-107.

    Problems with heart rate and rhythm are common after the Fontan procedure. The purpose of this study was to see if daily health status in children enrolled in the Fontan study was affected by heart rate or rhythm disorders. Researchers found that a lower resting heart rate and a higher heart rate during exercise were only weakly associated with better physical health. Therefore, other factors may have more impact on how children function after then Fontan procedure.

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  • Functional State Following the Fontan Procedure

    I. Williams, Cardiol Young 2009; 15:1-11.

    Despite improvements in outcomes after surgery, the functional state varies in children who have had the Fontan procedure. This study developed a scoring system for overall function, looking at how the pumping chamber squeezes, the ability to exercise, physical well-being, and certain lab tests. Lower overall function after surgery was found in children who have a right heart pumping chamber, heart pressures that are higher than normal, lower oxygen levels before surgery, abnormal heart rhythms after surgery, and lower income of their caregiver.

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  • Comparison of Echocardiographic and Cardiac Magnetic Resonance Imaging Measurements of Functional Single Ventricular Volumes, Mass, and Ejection Fraction (From the Pediatric Heart Network Multicenter Fontan Cross-Sectional Study)

    R. Margossian, Am J Cardiol 2009; 104:419-428.

    The size and function of a single heart pumping chamber (ventricle) are key elements when managing patients after the Fontan procedure. Children in the Fontan study had echocardiograms and MRIs performed as part of the study. This article describes how closely the measurements compare between different doctors who read the studies.

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  • Parent Versus Child-Reported Functional Health Status After the Fontan Procedure

    L. Lambert, Pediatrics 2009; 124:e942-949.

    Children ages 10-18 enrolled in the Fontan study and their parents completed forms to assess the child’s health status. Parents’ perceptions of the functional health status of their children after the Fontan procedure were worse than the children’s perceptions of their own well being in several areas.

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  • Blood Flow Distribution in a Large Series of Fontan Patients: A Cardiac Magnetic Resonance Velocity Mapping Study

    K. K. Whitehead, J Thorac Cardiovasc Surg. 2009 Jul; 138(1): 96–102.

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  • Laboratory Measures of Exercise Capacity and Ventricular Characteristics and Function are Weakly Associated with Functional Health Status After Fontan Procedure

    B. McCrindle, Circulation 2010; 121:34-42.

    This study looked at how the well being of children who were in the Fontan study related to the test results from echocardiography, MRI, blood and exercise tests. The results showed that the results of these tests were only weakly associated with health status. This suggests that these tests may not be good markers for functional health status.

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  • Anthropometric measures after the Fontan procedure: Implications for suboptimal functional outcome

    M. Cohen, Am Heart J 2010; 160:1092-1098.e1.

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  • The Fontan patient: inconsistencies in medication therapy across seven pediatric heart network centers

    P. Anderson, Pediatr Cardiol 2010; 31(8): 1219–1228.

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  • Relation of the size of secondary ventricles to exercise performance in children after a Fontan operation

    A. Prakash, Am J Cardiol 2010; 106(11):1652-6.

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  • Arrhythmias in a contemporary Fontan cohort: prevalence and clinical associations in a multi-center cross-sectional study

    E. Stephenson, J Am Coll Cardiol 2010; 56:890-6.

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  • Living at an altitude adversely affects exercise capacity in Fontan patients

    J. R. Darst, Cardiol Young. 2010 Dec; 20(6): 593–601.

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  • Non-Geometric Echocardiographic Indices of Ventricular Function in Patients with a Fontan Circulation

    J. Rhodes, J Am Soc Echocardiogr 2011; 24:1213-9.

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  • Practice variability and outcomes of coil embolization of aortopulmonary collaterals before Fontan completion: A report from the Pediatric Heart Network Fontan Cross-Sectional Study

    P. Banka, Am Heart J 2011; 162:125-130.

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  • Late Status of Fontan patients with Persistent Surgical Fenestration

    A. Atz, J Am Coll Cardiol 2011; 57:2437-43.

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  • Factors associated with serum brain natriuretic peptide levels after the Fontan procedure

    A. Atz, Congenit Heart Dis 2011; 6:313-321.

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  • Factors Impacting Echocardiographic Imaging after the Fontan Procedure: A Report from the Pediatric Heart Network Fontan Cross-Sectional Study

    R. Williams, Echocardiography 2013; 30:1098-1106.

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  • Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection

    A. Atz, Cardiol Young 2013; 23(3): 335–343.

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  • Comparison of Fontan Survivors with and without Pacemakers: A Report from the Pediatric Heart Network Fontan Cross-sectional Study

    R. Williams, Congenit Heart Dis 2013;8(1): 32–39.

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  • Age at Fontan procedure impacts exercise performance in adolescents: results from the Pediatric Heart Network Multicenter study

    P. Madan, Am Heart J. 2013 Aug;166(2):365-372.e1.

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  • Functional health status in children and adolescents after Fontan: Comparison of generic and disease-specific assessment

    B. McCrindle, Cardiol Young 2014; 24(3): 469–477.

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  • Abnormal spirometry after the Fontan procedure is common and associated with impaired aerobic capacity

    A. R. Opotowsky, Am J Physiol Heart Circ Physiol. 2014 Jul 1;307(1):H110-7.

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  • Assessment of Diastolic Function in Single-Ventricle Patients After the Fontan Procedure

    R. Margossian, J Am Soc Echocardiogr 2016 Nov;29(11):1066-1073.

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  • Impact of Cardiac Rehabilitation on the Exercise Function of Children with Serious Congenital Heart Disease

    J. Rhodes, Pediatrics 2005; 116(6):1339-1345.

    Children with congenital heart disease (CHD) often can’t exercise as well as their peers. Part of this may be due to their heart defect and part may be that they may not be very physically active. A 12-week cardiac rehabilitation program was designed to see if children could improve their ability to exercise safely. Sixteen patients completed the program. Improvements were found in 15 of 16 patients and no patient had any heart problems from the exercise.

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  • Sustained Effects of Cardiac Rehabilitation in Children with Serious Congenital Heart Disease

    J. Rhodes, Pediatrics 2006; 118(3):e586-593.

    Previous studies have shown immediate benefits when children with congenital heart disease are enrolled in a cardiac rehabilitation (exercise) program. This study aimed to see if the benefits lasted after the exercise program ended. The results showed, compared with children who did not undergo cardiac rehabilitation after the Fontan procedure, children who did participate had significant improvements in exercise function, behavior, self-esteem and emotional state 6 months after their program ended.

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