While the Fontan circulation has dramatically improved survival for infants with complex congenital heart disease, it is associated with significant long-term morbidity and potential for eventual failure.
Alerts clinicians to rising Fontan morbidity; leaves open optimal strategies to mitigate failure in this growing population.
The prognosis for infants born with complex heart disease improved dramatically with the introduction of the Fontan circulation 50 years ago. With today's carefully designed and staged operations to a Fontan circulation, life expectancy has increased and most children will survive into adult life. The Fontan circulation entails an unphysiological circulation with high risk for multiple organ system dysfunction. Neurodevelopmental disabilities with adverse psychosocial effects are prevalent. The Fontan circulation may eventually fail and necessitate heart transplantation. CONCLUSION: Fifty years development of the Fontan circulation to today's staged surgical procedures has improved survival but also revealed the burden of a high morbidity for a growing number of patients.
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Hedlund et al. (2021) studied this question.
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