Fontan Follow-Up 2

(Observational Study)

Study Summary

This first follow-up to the Fontan study looked at how children and adolescents with single ventricle heart defects who participated in the Fontan Study were doing six years later.

This follow-up aimed to learn how these children and young adults, who were now between the ages of 12 and 25 years, had done since the first study. The study began in November 2009 and all children who were enrolled in the original Fontan study were invited to participate.

7 Years of Follow-Up

Participants were followed for over 7 years, on average, after the Fontan Cross-Sectional study.

5% Risk

The overall risk of death/transplant was 5% for those in this study.

Little Evidence of Decline in Functioning

Over the follow-up period, a statistically significant but small and clinically unimportant decline was noted in Physical Functioning.

Who was in the study?

People in the study were:
  • Between 12-25 years of age
  • Participants in the Fontan 1 (also called the Fontan Cross Sectional study)
  • Not pregnant

What happened during the study?

Each qualified person completed the following study procedures:
  • Medical Record Review: Medical charts were reviewed to obtain information about surgeries, tests or times in the hospital. Subjects were asked if they were taking medicine or getting services like physical or speech therapy.
  • Questionnaires: Depending on age, parents and participants were asked to complete questionnaires about physical, emotional, and social aspects of health and about quality of life. The questionnaires asked about living situation, education, work outside the home, and other factors, such as health insurance coverage.

What were the results of the study?

Here are some things that we learned about Fontan patients as a group:
  • Mean age of the patients was 18 years and mean time after Fontan surgery was 15 years.
  • Patients were followed for an average of over 7 years after the Fontan Cross-Sectional study.
  • An overall risk of death/transplant was at 5%; the risk was higher for patients with both an elevated brain natriuretic peptide and a low Child Health Questionnaire (CHQ) physical summary score measured during the Fontan Cross-Sectional study.
  • 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, 78% of Fontan patients scored in the normal range for physical functioning and 88% were in the normal range for mental functioning.
  • Over the follow-up period, a statistically significant but small and clinically unimportant decline was noted in CHQ Physical Functioning Summary Score, while change in CHQ Psychosocial Functioning Summary Score was not significant.

The things we learned may not apply to every child. The findings reflect those for all 427 people who participated; parents of only 245 children and adolescents completed the CHQ at two time points. 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

  • Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: The Pediatric Heart Network Fontan cohort

    A. Atz, Congenit Heart Dis 2015; 10(1):E30-42.

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  • The relationship of patient, medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure

    B. McCrindle, Pediatr Cardiol 2014; 35(4):632-640.

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  • Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation

    K. Uzark, J Pediatr 2016; 170:166-172 Epub 2015 Dec 10.

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