Heart-Liver Interplay in Patients with Fontan Circulation

Area of Science:

• Cardiology
• Hepatology
• Pediatric Cardiology

Background:

• The Fontan procedure enables adults with congenital heart disease to survive into adulthood.
• Fontan circulation increasingly leads to liver complications, reduced heart function, and exercise capacity.
• Identifying risk factors for liver dysfunction in Fontan patients is critical.

Purpose of the Study:

• Identify univentricular heart malformations susceptible to liver dysfunction.
• Assess essential liver injury markers for Fontan patient follow-up.
• Determine optimal liver function evaluation methods.
• Explore effective data interpretation and organ failure response strategies for congenital cardiology teams.

Main Methods:

• Cross-sectional clinical study of 70 patients who underwent the Fontan procedure.
• Elective hospitalization for clinical assessment, laboratory tests, and instrumental examinations.
• Evaluation included NTproBNP, liver function tests (GGT), cardiac imaging, and Fibroscan for hepatic stiffness.

Main Results:

• 68% of patients had abnormal Gamma-Glutamyl Transferase (GGT) levels; 25% showed altered hepatic stiffness.
• Systemic atrioventricular valve (SAVV) dysfunction was linked to reduced VO2 max and increased hepatic stiffness.
• 56% had elevated NTproBNP, often with right-sided ventricular morphology.

Conclusions:

• SAVV dysfunction significantly contributes to poorer functional outcomes and hepatic fibrosis via increased venous congestion.
• Multidisciplinary follow-up is essential for early complication detection and improved outcomes in Fontan patients.
• Prompt treatment and management are vital for patients with Fontan circulation.