Abnormalities in liver function and coagulation profile following the Fontan procedure

Area of Science:

• Cardiovascular Surgery
• Pediatric Cardiology
• Hepatology

Background:

• The Fontan procedure is a palliative surgery for complex congenital heart defects.
• Long-term outcomes and potential complications, including liver dysfunction and coagulopathy, require ongoing investigation.

Purpose of the Study:

• To assess liver function and coagulation status in patients with Fontan circulation.
• To correlate these parameters with the time elapsed since the modified Fontan procedure.

Main Methods:

• Retrospective analysis of clinical data from 28 surviving patients post-modified Fontan procedure.
• Cross-sectional study examining liver function tests and coagulation profiles in relation to time since surgery.

Main Results:

• Abnormal liver function, mainly cholestasis, was observed in 21 patients, particularly those with longer follow-up periods (p < 0.01).
• Coagulation abnormalities were present in 22 patients, including decreased plasminogen and protein C activity, and deficiencies in factors V and VII.
• A prethrombotic state was identified in five patients, significantly associated with longer time intervals since surgery (p = 0.05).

Conclusions:

• Fontan patients with long-term follow-up frequently exhibit mild cholestasis and a prethrombotic state.
• Coagulation profiles are variable and can present both procoagulant and anticoagulant abnormalities.
• Regular, detailed evaluations and consideration of anticoagulant therapy are crucial for managing these patients.