Quality of Life After Fontan Operation in Young Adults

Study Questions:

How do adolescents and young adults fare, many years after the Fontan operation?


The original Pediatric Heart Network cohort of 546 Fontan patients (ages 6-18 years at first enrollment prior to 2008) from seven institutions who survived between 2009 and 2011 were asked to complete the generic 23-item Pediatric Quality of Life Inventory (PedsQL) and the six-scale PedsQL cardiac module. Teens <19 years old also completed an 87-item child health questionnaire (CHQ87), which had also been administered to the original cohort prior to 2008. Young adults >19 years completed the 36-item short form questionnaire (SF-36). Fontan PedsQL scores were compared to age, sex, and ethnicity-matched healthy subjects.


There were 255 teens and 155 young adults, for a total of 408 participants, with a mean of 15 years after Fontan surgery. Mean PedsQL scores were lower in Fontan subjects compared to normal controls, with 45% having physical functioning scores below the cut-off point for significant clinical impairment; 30% reported psychosocial impairment, with emotional functioning least likely to be impaired (20%). Physical and emotional functioning predictably declined with age, but was better for males. Psychosocial scores were positively correlated with household income in young adults. CHQ87 and SF-36 correlated with PedsQL scores.


Fontan survivors are at risk for quality of life (QOL) impairment, which worsens with age.


This is an important large sample, multisite study that highlights the issues survivors of palliated congenital heart disease face. QOL impairment with increasing age in Fontan survivors is in keeping with findings in other chronic diseases. Emotional functioning being least significantly impaired in young adults may reflect survivor bias, but may also be attributable to yet unidentified coping mechanisms. Gender inequity in PedsQL scores is concerning, and the reasons for this must be studied further. Of note is that physical or psychosocial QOL is not necessarily associated with disease severity as determined by imaging studies and other objective clinical parameters. Perceived social support, exercise rehabilitation, or the potential use of “prophylactic” pulmonary vasodilator therapy or other pharmacologic or psychosocial interventions may have the potential to improve QOL outcomes across the lifespan and underscore the need for further studies.

Keywords: Adolescent, Cardiac Surgical Procedures, Chronic Disease, Emotions, Fontan Procedure, Heart Defects, Congenital, Physical Fitness, Quality of Life, Secondary Prevention, Survivors, Vasodilator Agents, Young Adult

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