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Published on: May 2, 2025
Comparison of prognostic variables in children and adults with Fontan circulation
Hideo Ohuchi1, Kenji Yasuda1, Aya Miyazaki1
1Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka 565-8565, Japan.
Insights
Adult Fontan patients face different risks than children, with non-cardiac issues like renal dysfunction and metabolic problems predicting outcomes. Cardiac factors are less predictive in adults, highlighting the need for comprehensive care in Fontan survivors.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Adult Congenital Heart Disease
Background:
- The Fontan operation creates unique hemodynamics leading to late non-cardiac complications like hepato-renal and metabolic issues.
- Clinical variable associations with outcomes can evolve over the long-term postoperative course.
Purpose of the Study:
- To compare how cardiac and non-cardiac variables impact clinical outcomes in pediatric versus adult Fontan patients.
- To identify distinct predictors of adverse events in different age groups post-Fontan surgery.
Main Methods:
- Prospective evaluation of 167 child and 116 adult Fontan patients.
- Analysis of hemodynamics, neurohumoral factors, exercise capacity, hepato-renal function, and metabolic variables.
- Assessment of associations with unscheduled hospitalization and death over 3.7 ± 2.1 years.
Main Results:
- Adults had higher medication rates, lower cardiac index, elevated natriuretic peptides, worse renal function, cholestatic liver disease, and poorer exercise response compared to children.
- High central venous pressure (CVP) and low arterial oxygen saturation predicted events in children, but not adults.
- Renal dysfunction and metabolic abnormalities predicted events in adults.
- Neurohumoral activation, low albumin, hyponatremia, and impaired exercise predicted events in both groups.
Conclusions:
- Predictive values of clinical variables differ significantly between pediatric and adult Fontan patients.
- Non-cardiac factors are crucial determinants of outcome in adult Fontan survivors, necessitating their consideration alongside cardiac issues for improved prognosis.
Background:
Non-cardiac complications, such as hepato-renal and metabolic problems, are emerging late after the Fontan operation due to its unique hemodynamics. Consequently, associations between clinical variables and postoperative outcome may change during the prolonged postoperative course.
Methods And Results:
To determine if child and adult Fontan patients differ in the impact of cardiac and non-cardiac variables on clinical outcome, we prospectively evaluated associations between hemodynamics, neurohumoral factors, exercise variables, hepato-renal function and metabolic variables and unscheduled hospitalization, including death in 167 consecutive child and 116 adult Fontan patients. When compared with child patients, the adult patients showed higher rates of medications, lower cardiac index, higher values of natriuretic peptides, greater renal dysfunction, more cholestatic livers, and more impaired responses to exercise (p<0.05-0.0001). During the follow-up of 3.7 ± 2.1 years, 64 clinical events (37 in adults), including 13 deaths, occurred. A high CVP and low arterial oxygen satutration strongly predicted the child events (p<0.001), whereas these prognostic parameters were marginal in the adults. Instead, renal dysfunction and metabolic abnormality predicted adult events (p<0.05). Neurohumoral activation, low albumin, hyponatremia, and impaired exercise variables equally predicted clinical events in child and adult Fontan patients.
Conclusions:
Distinctive differences in predictive value of clinical variables exist between child and adult Fontan patients. In addition to cardiac issues, we should consider non-cardiac determinents of clinical outcome to maximize our efforts to improve prognosis for adult Fontan survivors.

