Management of the failing Fontan

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Adult Congenital Heart Disease

Background:

• Increasing survival of single ventricle patients leads to a growing adult Fontan population.
• This population presents unique challenges due to the absence of a sub-pulmonic ventricle.
• Variability in clinical status reflects diverse substrates and evolving surgical techniques over decades.

Purpose of the Study:

• To review the challenges and sequelae in the burgeoning Fontan population.
• To discuss the physio-pathological mechanisms of Fontan circulation.
• To explore therapeutic options for optimizing failing Fontan status and delaying irreversible deterioration.

Main Methods:

• Literature review of Fontan circulation and single ventricle physiology.
• Analysis of clinical status variability in Fontan patients.
• Discussion of evolving surgical techniques and understanding of Fontan physiology.

Main Results:

• Successful pediatric palliation results in an expanding adult single ventricle population.
• The absence of the sub-pulmonic ventricle leads to inevitable sequelae.
• Understanding of Fontan physiology has gradually improved over the last four decades.

Conclusions:

• The growing Fontan population requires specialized care due to long-term sequelae.
• Optimizing management of failing Fontan circulation is crucial to delay irreversible deterioration.
• Further research into the complex physiology and therapeutic strategies is warranted.