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Published on: December 30, 2025
[Prominent elevation of liver enzymes after Fontan operation]
Insights
Acute liver dysfunction after Fontan operation, though often mild, indicates poor prognosis. This case highlights significant liver enzyme elevation post-Fontan, linked to hemodynamic instability and high central venous pressure.
Area of Science:
- Pediatric Cardiology
- Hepatology
- Surgical Complications
Background:
- The Fontan operation is a complex surgical procedure for single-ventricle congenital heart defects.
- Acute liver dysfunction is a recognized, yet often underestimated, complication following Fontan procedures.
- Mild laboratory abnormalities can mask the severity and prognostic significance of hepatic dysfunction.
Observation:
- A 1.5-year-old boy with hypoplastic left heart syndrome underwent a Fontan operation.
- Hemodynamic monitoring included arterial pressure, central venous pressure, and regional cerebral oxygenation (rSO2).
- Postoperatively, the patient exhibited marked elevations in liver enzymes (GOT, GPT).
Findings:
- Elevated central venous pressure (22-25 mmHg) and unstable systemic arterial pressure were noted.
- Fenestration expansion and nitric oxide (NO) administration were used to manage hemodynamics.
- Significant increases in GOT and GPT levels were observed, peaking on postoperative day 7.
Implications:
- Hepatic dysfunction post-Fontan is primarily associated with hemodynamic disturbances.
- Abdominal regional cerebral oxygenation (rSO2) and high central venous pressure are key contributing factors.
- Close monitoring of liver function and hemodynamics is crucial for managing Fontan patients.
Abstract:
It is known that acute liver dysfunction is one of the complications after Fontan operation. We tend to overlook it because their laboratory abnormalities are typically mild and hepatic dysfunction is an uncommon complication in children after cardiac surgery. However, this complication is likely to be an important indicator of poor prognosis. We report a patient who showed a prominent elevation of liver enzymes after Fontan operation. A year and 5 month old boy was scheduled for Fontan operation due to hypoplastic left heart syndrome. We used arterial pressure, central venous pressure and rSO2 probes (INVOS 5100, Somanetics Corp., USA) attaching on his head, abdomen and leg for circulatory management. The operation was performed with the heart beating. The blood removal tubes were inserted to the superior vena cava and inferior vena cava and the blood sending tube was inserted to the innominate artery when Norwood stage 1 was performed. After making an extracardiac conduit and a fenestration, we tried to take off the oxygenator with dopamine 5 microg x kg(-1) x min(-1), dobutamine 3 microg x kg(-1) x min(-1), isosorbide 2.5 microg x kg(-1) x min(-1). The central venous pressure was increased to 22-25 mmHg and systematic arterial pressure was unstable around 50 mmHg. We suggested the surgeons to expand the fenestration because the low flow through it was found on TEE examination, and introduced 15 ppm of nitric monoxide (NO) to decrease pulmonary vascular resistance and to control the central venous pressure at the same time. rSO2 was decreased to 50 temporarily when the oxygenator was taken off, however it was returned to 70 just after expanding the fenestration. On the first postoperative day, the patient showed marked elevations in GOT 17,305 U x l(-1), GPT 8,110 U x l(-1), gradually peaking out to GOT 105 U x l(-1), GPT 1,348 U x l(-1) by the seventh postoperative day. Hepatic dysfunction is related mainly to hemodynamic disturbances and is also related to the abdominal rSO2 and the high central venous pressure.
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