Clinical Approaches to the Patient with a Failing Fontan Procedure

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• The Fontan operation is a critical surgical procedure for patients with functionally univentricular hearts.
• Increased survival rates have led to a growing population of adult Fontan survivors.
• Long-term health complications are increasingly recognized in this unique patient group.

Purpose of the Study:

• To review the manifestations of Fontan circulation failure.
• To discuss potential therapeutic strategies for Fontan failure.
• To propose a clinical approach for managing patients with Fontan failure.

Main Methods:

• Literature review of Fontan circulation physiology and failure.
• Analysis of cardiac and extracardiac manifestations.
• Synthesis of current therapeutic options and clinical management strategies.

Main Results:

• Fontan survivors face a vulnerable physiology with broad adverse health impacts.
• Manifestations of Fontan failure are diverse, affecting both cardiac and non-cardiac systems.
• Effective management requires a multidisciplinary approach.

Conclusions:

• Fontan failure presents complex, long-term health issues for survivors.
• Improved care necessitates multi-institutional collaboration among specialists.
• Partnership between cardiologists and other organ system specialists is crucial.