Cardiovascular adaptation to the Fontan circulation

Area of Science:

• Cardiovascular Physiology
• Pediatric Cardiology
• Surgical Innovation

Background:

• Medium-term survival after Fontan operations has significantly improved.
• Late cardiovascular and extracardiac morbidity remain significant challenges.
• Impaired quality of life and premature mortality are common late complications.

Purpose of the Study:

• To review the key features and principles governing the Fontan circulation.
• To provide insight into the integrative pathophysiology of the Fontan circulation.
• To stimulate research into advancing Fontan circulation management.

Main Methods:

• Review of existing literature on Fontan circulation physiology.
• Analysis of interactions between cardiac and circulatory compartments.
• Exploration of microcirculation and lymphatic system roles.

Main Results:

• The Fontan circulation requires extraordinary cardiovascular adaptations.
• Interactions between the ventricle, systemic, and pulmonary circulations are complex.
• Microcirculation and lymphatic systems play crucial roles in Fontan physiology.

Conclusions:

• Understanding Fontan circulation pathophysiology is key to improving long-term outcomes.
• Further research is needed to address late morbidity and mortality.
• Integrative approaches are essential for advancing Fontan circulation management.