What is normal Fontan circuit pressure?

Ideal Fontan mean pressure is 10-15 mmHg. Higher pressures (>15-18 mmHg) cause increased systemic venous pressure, protein-losing enteropathy, plastic bronchitis, and accelerated liver fibrosis (FALD).

What is Fontan-associated liver disease?

FALD is near-universal in Fontan patients >10 years. It ranges from nodular regenerative hyperplasia to cirrhosis and hepatocellular carcinoma (HCC). Annual ultrasound and AFP screening from age 10-15 years is recommended.

How does hepatic venous pressure guide management?

Elevated hepatic venous pressure (>15 mmHg) warrants catheterization to identify obstructions, assess Fontan fenestration function, and consider Fontan takedown or heart transplant evaluation.

Fontan Circulation Hepatic Venous Pressure

Monitor hepatic venous pressure and Fontan circuit pressure in single-ventricle patients to detect Fontan-associated liver disease (FALD) and optimize hemodynamics.

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