Fontan Follow-Up 2 (Observational Study)

You were enrolled in the Fontan Cross-Sectional Study or Fontan 1 Study that looked at how children and adolescents with single ventricle heart defects were doing after their Fontan Surgery.

Now we would like to learn how these children and young adults, who are now between the ages of 12 and 25 years, have done since the first study finished about six years ago.

The study began in November 2009 and all children who were enrolled in the original Fontan study were invited to participate.

Who was in the study?

People in the study were:

  • Between 12-25 years of age
  • Participants in the Fontan 1 or Fontan Cross Sectional study
  • Not pregnant

What happened during the study?

Each qualified person completed the following study procedures:
  • Medical Record Review: You were contacted and your medical chart was reviewed to obtain information about surgeries, tests or times that you were in the hospital. You were asked if you are taking medicine or getting services like physical or speech therapy. You may have been asked to sign a form allowing us to get information from other places where you have received or are receiving care.
  • Questionnaires: Depending on your age, you or both you and one of your parents were asked to complete questionnaires about yourself. Some of these questionnaires will be completed in a telephone interview. Others will be mailed to your home for you or one of your parents to fill out. The questionnaires asked about physical, emotional, and social aspects of your health and about your quality of life. The questionnaires asked about your living situation, education, work outside the home, and other factors, such as health insurance coverage, that may affect your medical care.

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-up on average 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 at 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 for the CHQ Physical and Psychosocial Summary score, and 97% and 91% for the SF-36 Physical and Mental Aggregate Score, respectively.
  • 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.